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A 59-year-old white man presented with 2 large erythematous lesions on the right side of the chest wall that had gradually progressed over the last 1.5 years. The patient denied any fever, night sweats, fatigue, unintentional weight loss, or loss of appetite. Physical examination revealed 2 large, well-circumscribed, nearly contiguous, firm, erythematous tumors. One tumor measured 7.5×4.5 cm and the other measured 4×3.5 cm.

The Diagnosis: Cutaneous B-cell Lymphoma

Biopsies from the right side of the chest wall (Figure 1) revealed an atypical dense and diffuse lymphocytic infiltrate throughout the dermis. There was extensive crush artifact throughout the specimen. However, the findings were consistent with cutaneous B-cell lymphoma (CBCL), and the diffuse large B-cell type was favored (Figure 2). Atypical lymphocytes stained positively for antibodies against CD20 (Figure 3), CD79a, and BCL-6, and stained negatively for antibodies against MUM-1 and BCL-2. Although flow cytometry revealed no definitive immunophenotypic lymphoma population, polymerase chain reaction analysis revealed a monoclonal immunoglobulin heavy chain gene rearrangement. Computed tomography (CT) scans of the chest, abdomen, and pelvis were unremarkable. A preliminary diagnosis of primary CBCL (PCBCL) was formulated. Diffuse large B-cell lymphoma (DLBCL) and follicle center lymphoma subtypes were each considered, which triggered further workup to rule out systemic involvement.

Figure 1. Erythematous firm tumors of the right side of the chest wall (A and B).

Figure 2. Atypical lymphocytic infiltrate with background crush artifact (A and B)(both H&E, original magnifications ×20 and ×40).

Figure 3. Atypical lymphocytes stained positively for antibodies against CD20 (original magnification ×40).

Figure 4. A focus of increased radiotracer deposition is seen in the left proximal humerus on positron emission tomography–computed tomography scanning.

A bone marrow biopsy from the posterior iliac crest revealed normocellular bone marrow with normal trilineage hematopoiesis. However, whole-body staging with positron emission tomography (PET)–CT scanning revealed osseous disease in the left proximal humerus (Figure 4) as well as a slightly hypermetabolic right axillary lymph node. Magnetic resonance imaging of the brain showed no evidence of intracranial disease. Because of the apparent systemic involvement, stage IV non-Hodgkin lymphoma (DLBCL) became the new suspected diagnosis. The patient was started on the first of 6 cycles of chemotherapy with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), and the skin lesions quickly dissipated and flattened. A faint pink discoloration remained over a slightly indented area. A repeat PET-CT scan following 4 cycles of R-CHOP chemotherapy also confirmed a complete response to therapy.

In general, CBCL tends to affect adults and presents as relatively firm and plum-colored papules, nodules, tumors, or plaques, which can be either fast or slow growing. Cutaneous B-cell lymphoma may be primary or secondary to systemic involvement. Primary CBCL refers to a group of non-Hodgkin lymphomas that initially present in the skin with no evidence of extracutaneous involvement at the time of diagnosis.^1,2 Secondary CBCL (SCBCL) refers to cutaneous disease that occurs secondary to systemic B-cell lymphoma. Detecting systemic involvement and distinguishing between PCBCL and SCBCL is valuable in determining prognosis and therapeutic options, as subtypes of PCBCL often have an improved prognosis and may be treated with local irradiation.

The initial staging techniques that are preferred for cutaneous lymphomas have been debated.^3-5 For cutaneous lymphomas, except mycosis fungoides and Sézary syndrome, the International Society for Cutaneous Lymphomas and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer recommends obtaining a complete blood cell count with differential; complete metabolic studies including lactate dehydrogenase; and imaging studies of the chest, abdomen, and pelvis. Bone marrow biopsies and imaging studies of the neck or whole-body PET-CT scanning also may be useful depending on the clinical scenario.³ Although a more limited workup may be sufficient for PCBCLs such as primary cutaneous marginal zone lymphoma,⁵ a bone marrow biopsy is recommended for cases of primary cutaneous DLBCL (leg type).³ Senff et al⁵ supported the use of a bone marrow biopsy in the evaluation of follicle center lymphomas first presenting in the skin, though this method is controversial. In our patient, the laboratory results; bone marrow biopsy; and CT scan of the chest, abdomen, and pelvis failed to suggest extracutaneous disease, while the PET-CT scan revealed systemic involvement.

The differential diagnosis of CBCL includes cutaneous lymphoid hyperplasia (pseudolymphoma), which may be the result of insults such as arthropod bites, stings, vaccinations, or trauma. The clinical presentation, histology, and results of molecular studies and immunohistochemistry are essential in differentiating benign versus malignant processes.⁶ Lymphomas are expected to be larger and more persistent than benign processes, demonstrating an atypical lymphocytic infiltrate and monoclonality; immunohistochemistry will aid in the distinction between B-cell and T-cell processes and can delineate the type of B-cell lymphoma. Histology for CBCL typically reveals an atypical lymphocytic infiltrate showing a CD20⁺ and CD79a⁺ immunophenotype. Staining for antibodies against BCL-2, BCL-6, CD10, and MUM-1 also plays an important role in the diagnosis of cutaneous lymphoma and determining where the lesion(s) falls within the classification schemes. For example, to differentiate between primary cutaneous lymphoma subtypes, BCL-2 negativity and BCL-6 positivity in the context of a CD20⁺ and CD79a⁺ immunophenotype supports a follicle center lymphoma or a DLBCL (non–leg type). By contrast, CD20, CD79a, BCL-2, and MUM-1 positivity would favor a DLBCL (leg type).⁷

The natural history and therapeutic options differ greatly between subtypes of CBCL. For example, the prognosis of primary cutaneous follicle center lymphoma is generally favorable with a 5-year disease-specific survival rate of roughly 95%, and radiation therapy is recommended as a first-line therapy for localized disease.^2,8 Conversely, primary cutaneous DLBCL (leg type) frequently spreads to extracutaneous sites⁸ and carries a much lower estimated 5-year disease-specific survival rate of 55%.² Chemotherapy with R-CHOP is typically included in initial therapy for primary cutaneous DLBCL (leg type).⁸ The prognosis of systemic B-cell lymphomas also is highly variable and may depend on the type of B-cell lymphoma, the stage of disease at diagnosis, histologic and immunologic characteristics, and the therapy received. Wright et al⁹ reported that patients with systemic germinal center B cell–like DLBCL had a 5-year survival rate of 62%, whereas patients with activated B cell–like variants of DLBCL had a 5-year survival rate of 26%. Expression of CD40 may be a favorable prognostic factor following treatment with systemic chemotherapy in patients with DLBCL,¹⁰ whereas FOXP1 protein overexpression is correlated with poor disease-specific survival in certain DLBCL phenotypes.¹¹

Although it is uncertain whether the cutaneous lesions preceded systemic disease in our patient, the cutaneous lesions could be arbitrarily classified as secondary because extracutaneous disease was discovered within 6 months of the initial diagnosis.¹ However, classifying the CBCL as primary or secondary did not alter the course of treatment in our patient, as the presumed systemic disease necessitated treatment with systemic chemotherapy; both PCBCLs that develop systemic involvement and SCBCLs (primary extracutaneous disease) usually are treated with systemic chemotherapy. Our case highlights the importance of whole-body staging, as PET-CT scanning changed the course of care by detecting osseous involvement, necessitating systemic therapy as opposed to local radiation therapy alone. A multidisciplinary team with a focus on the diagnosis and management of cutaneous lymphomas helped streamline our patient’s laboratory testing and imaging studies, diagnostic and therapeutic decision making, and treatment implementation. Open channels and frequent opportunities for communication among dermatologists, dermatopathologists, medical oncologists, hematopathologists, radiologists, and radiation oncologists are needed to optimize and coordinate care for patients with cutaneous lymphoma who require transdisciplinary care.

Acknowledgement—The authors would like to thank Henry Koon, MD (hematology/oncology), for his input and expertise.

A 59-year-old white man presented with 2 large erythematous lesions on the right side of the chest wall that had gradually progressed over the last 1.5 years. The patient denied any fever, night sweats, fatigue, unintentional weight loss, or loss of appetite. Physical examination revealed 2 large, well-circumscribed, nearly contiguous, firm, erythematous tumors. One tumor measured 7.5×4.5 cm and the other measured 4×3.5 cm.

The Diagnosis: Cutaneous B-cell Lymphoma

Biopsies from the right side of the chest wall (Figure 1) revealed an atypical dense and diffuse lymphocytic infiltrate throughout the dermis. There was extensive crush artifact throughout the specimen. However, the findings were consistent with cutaneous B-cell lymphoma (CBCL), and the diffuse large B-cell type was favored (Figure 2). Atypical lymphocytes stained positively for antibodies against CD20 (Figure 3), CD79a, and BCL-6, and stained negatively for antibodies against MUM-1 and BCL-2. Although flow cytometry revealed no definitive immunophenotypic lymphoma population, polymerase chain reaction analysis revealed a monoclonal immunoglobulin heavy chain gene rearrangement. Computed tomography (CT) scans of the chest, abdomen, and pelvis were unremarkable. A preliminary diagnosis of primary CBCL (PCBCL) was formulated. Diffuse large B-cell lymphoma (DLBCL) and follicle center lymphoma subtypes were each considered, which triggered further workup to rule out systemic involvement.

Figure 1. Erythematous firm tumors of the right side of the chest wall (A and B).

Figure 2. Atypical lymphocytic infiltrate with background crush artifact (A and B)(both H&E, original magnifications ×20 and ×40).

Figure 3. Atypical lymphocytes stained positively for antibodies against CD20 (original magnification ×40).

Figure 4. A focus of increased radiotracer deposition is seen in the left proximal humerus on positron emission tomography–computed tomography scanning.

A bone marrow biopsy from the posterior iliac crest revealed normocellular bone marrow with normal trilineage hematopoiesis. However, whole-body staging with positron emission tomography (PET)–CT scanning revealed osseous disease in the left proximal humerus (Figure 4) as well as a slightly hypermetabolic right axillary lymph node. Magnetic resonance imaging of the brain showed no evidence of intracranial disease. Because of the apparent systemic involvement, stage IV non-Hodgkin lymphoma (DLBCL) became the new suspected diagnosis. The patient was started on the first of 6 cycles of chemotherapy with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), and the skin lesions quickly dissipated and flattened. A faint pink discoloration remained over a slightly indented area. A repeat PET-CT scan following 4 cycles of R-CHOP chemotherapy also confirmed a complete response to therapy.

In general, CBCL tends to affect adults and presents as relatively firm and plum-colored papules, nodules, tumors, or plaques, which can be either fast or slow growing. Cutaneous B-cell lymphoma may be primary or secondary to systemic involvement. Primary CBCL refers to a group of non-Hodgkin lymphomas that initially present in the skin with no evidence of extracutaneous involvement at the time of diagnosis.^1,2 Secondary CBCL (SCBCL) refers to cutaneous disease that occurs secondary to systemic B-cell lymphoma. Detecting systemic involvement and distinguishing between PCBCL and SCBCL is valuable in determining prognosis and therapeutic options, as subtypes of PCBCL often have an improved prognosis and may be treated with local irradiation.

The initial staging techniques that are preferred for cutaneous lymphomas have been debated.^3-5 For cutaneous lymphomas, except mycosis fungoides and Sézary syndrome, the International Society for Cutaneous Lymphomas and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer recommends obtaining a complete blood cell count with differential; complete metabolic studies including lactate dehydrogenase; and imaging studies of the chest, abdomen, and pelvis. Bone marrow biopsies and imaging studies of the neck or whole-body PET-CT scanning also may be useful depending on the clinical scenario.³ Although a more limited workup may be sufficient for PCBCLs such as primary cutaneous marginal zone lymphoma,⁵ a bone marrow biopsy is recommended for cases of primary cutaneous DLBCL (leg type).³ Senff et al⁵ supported the use of a bone marrow biopsy in the evaluation of follicle center lymphomas first presenting in the skin, though this method is controversial. In our patient, the laboratory results; bone marrow biopsy; and CT scan of the chest, abdomen, and pelvis failed to suggest extracutaneous disease, while the PET-CT scan revealed systemic involvement.

The differential diagnosis of CBCL includes cutaneous lymphoid hyperplasia (pseudolymphoma), which may be the result of insults such as arthropod bites, stings, vaccinations, or trauma. The clinical presentation, histology, and results of molecular studies and immunohistochemistry are essential in differentiating benign versus malignant processes.⁶ Lymphomas are expected to be larger and more persistent than benign processes, demonstrating an atypical lymphocytic infiltrate and monoclonality; immunohistochemistry will aid in the distinction between B-cell and T-cell processes and can delineate the type of B-cell lymphoma. Histology for CBCL typically reveals an atypical lymphocytic infiltrate showing a CD20⁺ and CD79a⁺ immunophenotype. Staining for antibodies against BCL-2, BCL-6, CD10, and MUM-1 also plays an important role in the diagnosis of cutaneous lymphoma and determining where the lesion(s) falls within the classification schemes. For example, to differentiate between primary cutaneous lymphoma subtypes, BCL-2 negativity and BCL-6 positivity in the context of a CD20⁺ and CD79a⁺ immunophenotype supports a follicle center lymphoma or a DLBCL (non–leg type). By contrast, CD20, CD79a, BCL-2, and MUM-1 positivity would favor a DLBCL (leg type).⁷

The natural history and therapeutic options differ greatly between subtypes of CBCL. For example, the prognosis of primary cutaneous follicle center lymphoma is generally favorable with a 5-year disease-specific survival rate of roughly 95%, and radiation therapy is recommended as a first-line therapy for localized disease.^2,8 Conversely, primary cutaneous DLBCL (leg type) frequently spreads to extracutaneous sites⁸ and carries a much lower estimated 5-year disease-specific survival rate of 55%.² Chemotherapy with R-CHOP is typically included in initial therapy for primary cutaneous DLBCL (leg type).⁸ The prognosis of systemic B-cell lymphomas also is highly variable and may depend on the type of B-cell lymphoma, the stage of disease at diagnosis, histologic and immunologic characteristics, and the therapy received. Wright et al⁹ reported that patients with systemic germinal center B cell–like DLBCL had a 5-year survival rate of 62%, whereas patients with activated B cell–like variants of DLBCL had a 5-year survival rate of 26%. Expression of CD40 may be a favorable prognostic factor following treatment with systemic chemotherapy in patients with DLBCL,¹⁰ whereas FOXP1 protein overexpression is correlated with poor disease-specific survival in certain DLBCL phenotypes.¹¹

Although it is uncertain whether the cutaneous lesions preceded systemic disease in our patient, the cutaneous lesions could be arbitrarily classified as secondary because extracutaneous disease was discovered within 6 months of the initial diagnosis.¹ However, classifying the CBCL as primary or secondary did not alter the course of treatment in our patient, as the presumed systemic disease necessitated treatment with systemic chemotherapy; both PCBCLs that develop systemic involvement and SCBCLs (primary extracutaneous disease) usually are treated with systemic chemotherapy. Our case highlights the importance of whole-body staging, as PET-CT scanning changed the course of care by detecting osseous involvement, necessitating systemic therapy as opposed to local radiation therapy alone. A multidisciplinary team with a focus on the diagnosis and management of cutaneous lymphomas helped streamline our patient’s laboratory testing and imaging studies, diagnostic and therapeutic decision making, and treatment implementation. Open channels and frequent opportunities for communication among dermatologists, dermatopathologists, medical oncologists, hematopathologists, radiologists, and radiation oncologists are needed to optimize and coordinate care for patients with cutaneous lymphoma who require transdisciplinary care.

Acknowledgement—The authors would like to thank Henry Koon, MD (hematology/oncology), for his input and expertise.

A 59-year-old white man presented with 2 large erythematous lesions on the right side of the chest wall that had gradually progressed over the last 1.5 years. The patient denied any fever, night sweats, fatigue, unintentional weight loss, or loss of appetite. Physical examination revealed 2 large, well-circumscribed, nearly contiguous, firm, erythematous tumors. One tumor measured 7.5×4.5 cm and the other measured 4×3.5 cm.

The Diagnosis: Cutaneous B-cell Lymphoma

Biopsies from the right side of the chest wall (Figure 1) revealed an atypical dense and diffuse lymphocytic infiltrate throughout the dermis. There was extensive crush artifact throughout the specimen. However, the findings were consistent with cutaneous B-cell lymphoma (CBCL), and the diffuse large B-cell type was favored (Figure 2). Atypical lymphocytes stained positively for antibodies against CD20 (Figure 3), CD79a, and BCL-6, and stained negatively for antibodies against MUM-1 and BCL-2. Although flow cytometry revealed no definitive immunophenotypic lymphoma population, polymerase chain reaction analysis revealed a monoclonal immunoglobulin heavy chain gene rearrangement. Computed tomography (CT) scans of the chest, abdomen, and pelvis were unremarkable. A preliminary diagnosis of primary CBCL (PCBCL) was formulated. Diffuse large B-cell lymphoma (DLBCL) and follicle center lymphoma subtypes were each considered, which triggered further workup to rule out systemic involvement.

Figure 1. Erythematous firm tumors of the right side of the chest wall (A and B).

Figure 2. Atypical lymphocytic infiltrate with background crush artifact (A and B)(both H&E, original magnifications ×20 and ×40).

Figure 3. Atypical lymphocytes stained positively for antibodies against CD20 (original magnification ×40).

Figure 4. A focus of increased radiotracer deposition is seen in the left proximal humerus on positron emission tomography–computed tomography scanning.

A bone marrow biopsy from the posterior iliac crest revealed normocellular bone marrow with normal trilineage hematopoiesis. However, whole-body staging with positron emission tomography (PET)–CT scanning revealed osseous disease in the left proximal humerus (Figure 4) as well as a slightly hypermetabolic right axillary lymph node. Magnetic resonance imaging of the brain showed no evidence of intracranial disease. Because of the apparent systemic involvement, stage IV non-Hodgkin lymphoma (DLBCL) became the new suspected diagnosis. The patient was started on the first of 6 cycles of chemotherapy with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP), and the skin lesions quickly dissipated and flattened. A faint pink discoloration remained over a slightly indented area. A repeat PET-CT scan following 4 cycles of R-CHOP chemotherapy also confirmed a complete response to therapy.

In general, CBCL tends to affect adults and presents as relatively firm and plum-colored papules, nodules, tumors, or plaques, which can be either fast or slow growing. Cutaneous B-cell lymphoma may be primary or secondary to systemic involvement. Primary CBCL refers to a group of non-Hodgkin lymphomas that initially present in the skin with no evidence of extracutaneous involvement at the time of diagnosis.^1,2 Secondary CBCL (SCBCL) refers to cutaneous disease that occurs secondary to systemic B-cell lymphoma. Detecting systemic involvement and distinguishing between PCBCL and SCBCL is valuable in determining prognosis and therapeutic options, as subtypes of PCBCL often have an improved prognosis and may be treated with local irradiation.

The initial staging techniques that are preferred for cutaneous lymphomas have been debated.^3-5 For cutaneous lymphomas, except mycosis fungoides and Sézary syndrome, the International Society for Cutaneous Lymphomas and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer recommends obtaining a complete blood cell count with differential; complete metabolic studies including lactate dehydrogenase; and imaging studies of the chest, abdomen, and pelvis. Bone marrow biopsies and imaging studies of the neck or whole-body PET-CT scanning also may be useful depending on the clinical scenario.³ Although a more limited workup may be sufficient for PCBCLs such as primary cutaneous marginal zone lymphoma,⁵ a bone marrow biopsy is recommended for cases of primary cutaneous DLBCL (leg type).³ Senff et al⁵ supported the use of a bone marrow biopsy in the evaluation of follicle center lymphomas first presenting in the skin, though this method is controversial. In our patient, the laboratory results; bone marrow biopsy; and CT scan of the chest, abdomen, and pelvis failed to suggest extracutaneous disease, while the PET-CT scan revealed systemic involvement.

The differential diagnosis of CBCL includes cutaneous lymphoid hyperplasia (pseudolymphoma), which may be the result of insults such as arthropod bites, stings, vaccinations, or trauma. The clinical presentation, histology, and results of molecular studies and immunohistochemistry are essential in differentiating benign versus malignant processes.⁶ Lymphomas are expected to be larger and more persistent than benign processes, demonstrating an atypical lymphocytic infiltrate and monoclonality; immunohistochemistry will aid in the distinction between B-cell and T-cell processes and can delineate the type of B-cell lymphoma. Histology for CBCL typically reveals an atypical lymphocytic infiltrate showing a CD20⁺ and CD79a⁺ immunophenotype. Staining for antibodies against BCL-2, BCL-6, CD10, and MUM-1 also plays an important role in the diagnosis of cutaneous lymphoma and determining where the lesion(s) falls within the classification schemes. For example, to differentiate between primary cutaneous lymphoma subtypes, BCL-2 negativity and BCL-6 positivity in the context of a CD20⁺ and CD79a⁺ immunophenotype supports a follicle center lymphoma or a DLBCL (non–leg type). By contrast, CD20, CD79a, BCL-2, and MUM-1 positivity would favor a DLBCL (leg type).⁷

The natural history and therapeutic options differ greatly between subtypes of CBCL. For example, the prognosis of primary cutaneous follicle center lymphoma is generally favorable with a 5-year disease-specific survival rate of roughly 95%, and radiation therapy is recommended as a first-line therapy for localized disease.^2,8 Conversely, primary cutaneous DLBCL (leg type) frequently spreads to extracutaneous sites⁸ and carries a much lower estimated 5-year disease-specific survival rate of 55%.² Chemotherapy with R-CHOP is typically included in initial therapy for primary cutaneous DLBCL (leg type).⁸ The prognosis of systemic B-cell lymphomas also is highly variable and may depend on the type of B-cell lymphoma, the stage of disease at diagnosis, histologic and immunologic characteristics, and the therapy received. Wright et al⁹ reported that patients with systemic germinal center B cell–like DLBCL had a 5-year survival rate of 62%, whereas patients with activated B cell–like variants of DLBCL had a 5-year survival rate of 26%. Expression of CD40 may be a favorable prognostic factor following treatment with systemic chemotherapy in patients with DLBCL,¹⁰ whereas FOXP1 protein overexpression is correlated with poor disease-specific survival in certain DLBCL phenotypes.¹¹

Although it is uncertain whether the cutaneous lesions preceded systemic disease in our patient, the cutaneous lesions could be arbitrarily classified as secondary because extracutaneous disease was discovered within 6 months of the initial diagnosis.¹ However, classifying the CBCL as primary or secondary did not alter the course of treatment in our patient, as the presumed systemic disease necessitated treatment with systemic chemotherapy; both PCBCLs that develop systemic involvement and SCBCLs (primary extracutaneous disease) usually are treated with systemic chemotherapy. Our case highlights the importance of whole-body staging, as PET-CT scanning changed the course of care by detecting osseous involvement, necessitating systemic therapy as opposed to local radiation therapy alone. A multidisciplinary team with a focus on the diagnosis and management of cutaneous lymphomas helped streamline our patient’s laboratory testing and imaging studies, diagnostic and therapeutic decision making, and treatment implementation. Open channels and frequent opportunities for communication among dermatologists, dermatopathologists, medical oncologists, hematopathologists, radiologists, and radiation oncologists are needed to optimize and coordinate care for patients with cutaneous lymphoma who require transdisciplinary care.

Acknowledgement—The authors would like to thank Henry Koon, MD (hematology/oncology), for his input and expertise.

The Diagnosis: Atrophic Pityriasis Versicolor

Pityriasis versicolor lesions accompanied by skin atrophy were first reported by De Graciansky and Mery¹ in 1971. Since then, few reports have been described and it remains a rare condition.^2-5 It manifests with oval to round, ivory-colored lesions with a typically depressed and sometimes finely pleated surface.³ The pathogenesis of the skin atrophy remains controversial. In some of the cases described, the onset of atrophy was related to long-term use of topical steroids.¹ This fact as well as impaired barrier function due to fungal infection may explain the atrophy occurring only in the pityriasis versicolor lesions.^2,6,7 Some authors call this disease “pityriasis versicolor pseudoatrophicans.”⁷ However, case reports have been described without use of topical corticosteroids. Crowson and Magro⁸ maintained that skin atrophy in these cases may occur due to mechanisms of delayed-type hypersensitivity and coined the term atrophying pityriasis versicolor as a variant of this disease. Our patient did not report prior use of topical corticosteroids.

The differential diagnosis consists of other diseases that cause skin atrophy, such as collagen vascular diseases including anetoderma, morphea or atrophoderma, lupus erythematosus, dermatomyositis, and poikilodermatous T-cell dyscrasia; parapsoriasis or mycosis fungoides; sarcoidosis; cutis laxa; acrodermatitis chronica atrophicans; necrobiosis lipoidica; and atrophy due to intralesional steroid therapy.^2,3,6-8 Histologic examination helps to achieve proper diagnosis. In our patient, cutaneous biopsy showed the presence of multiple short hyphae and spores in the horny layer with hematoxylin and eosin as well as periodic acid–Schiff stains, with typical “spaghetti and meatballs” appearance. Partial atrophy of the epidermis was observed with flattening of the epidermic ridges. A comparison with the normal areas could not be made because the biopsy was taken from cutaneous lesions without areas of uninvolved skin (Figure).

Treatment of this variant does not differ from conventional therapies for pityriasis versicolor, except that a longer treatment period might be required. Atrophy usually disappears, showing that atrophic pityriasis versicolor has a relatively good prognosis compared with other diseases that cause skin atrophy.²

Our patient was treated with ketoconazole gel 2% once daily for 3 weeks with complete resolution of the lesions and no evidence of atrophy.

The Diagnosis: Atrophic Pityriasis Versicolor

Pityriasis versicolor lesions accompanied by skin atrophy were first reported by De Graciansky and Mery¹ in 1971. Since then, few reports have been described and it remains a rare condition.^2-5 It manifests with oval to round, ivory-colored lesions with a typically depressed and sometimes finely pleated surface.³ The pathogenesis of the skin atrophy remains controversial. In some of the cases described, the onset of atrophy was related to long-term use of topical steroids.¹ This fact as well as impaired barrier function due to fungal infection may explain the atrophy occurring only in the pityriasis versicolor lesions.^2,6,7 Some authors call this disease “pityriasis versicolor pseudoatrophicans.”⁷ However, case reports have been described without use of topical corticosteroids. Crowson and Magro⁸ maintained that skin atrophy in these cases may occur due to mechanisms of delayed-type hypersensitivity and coined the term atrophying pityriasis versicolor as a variant of this disease. Our patient did not report prior use of topical corticosteroids.

The differential diagnosis consists of other diseases that cause skin atrophy, such as collagen vascular diseases including anetoderma, morphea or atrophoderma, lupus erythematosus, dermatomyositis, and poikilodermatous T-cell dyscrasia; parapsoriasis or mycosis fungoides; sarcoidosis; cutis laxa; acrodermatitis chronica atrophicans; necrobiosis lipoidica; and atrophy due to intralesional steroid therapy.^2,3,6-8 Histologic examination helps to achieve proper diagnosis. In our patient, cutaneous biopsy showed the presence of multiple short hyphae and spores in the horny layer with hematoxylin and eosin as well as periodic acid–Schiff stains, with typical “spaghetti and meatballs” appearance. Partial atrophy of the epidermis was observed with flattening of the epidermic ridges. A comparison with the normal areas could not be made because the biopsy was taken from cutaneous lesions without areas of uninvolved skin (Figure).

Treatment of this variant does not differ from conventional therapies for pityriasis versicolor, except that a longer treatment period might be required. Atrophy usually disappears, showing that atrophic pityriasis versicolor has a relatively good prognosis compared with other diseases that cause skin atrophy.²

Our patient was treated with ketoconazole gel 2% once daily for 3 weeks with complete resolution of the lesions and no evidence of atrophy.

The Diagnosis: Atrophic Pityriasis Versicolor

Pityriasis versicolor lesions accompanied by skin atrophy were first reported by De Graciansky and Mery¹ in 1971. Since then, few reports have been described and it remains a rare condition.^2-5 It manifests with oval to round, ivory-colored lesions with a typically depressed and sometimes finely pleated surface.³ The pathogenesis of the skin atrophy remains controversial. In some of the cases described, the onset of atrophy was related to long-term use of topical steroids.¹ This fact as well as impaired barrier function due to fungal infection may explain the atrophy occurring only in the pityriasis versicolor lesions.^2,6,7 Some authors call this disease “pityriasis versicolor pseudoatrophicans.”⁷ However, case reports have been described without use of topical corticosteroids. Crowson and Magro⁸ maintained that skin atrophy in these cases may occur due to mechanisms of delayed-type hypersensitivity and coined the term atrophying pityriasis versicolor as a variant of this disease. Our patient did not report prior use of topical corticosteroids.

The differential diagnosis consists of other diseases that cause skin atrophy, such as collagen vascular diseases including anetoderma, morphea or atrophoderma, lupus erythematosus, dermatomyositis, and poikilodermatous T-cell dyscrasia; parapsoriasis or mycosis fungoides; sarcoidosis; cutis laxa; acrodermatitis chronica atrophicans; necrobiosis lipoidica; and atrophy due to intralesional steroid therapy.^2,3,6-8 Histologic examination helps to achieve proper diagnosis. In our patient, cutaneous biopsy showed the presence of multiple short hyphae and spores in the horny layer with hematoxylin and eosin as well as periodic acid–Schiff stains, with typical “spaghetti and meatballs” appearance. Partial atrophy of the epidermis was observed with flattening of the epidermic ridges. A comparison with the normal areas could not be made because the biopsy was taken from cutaneous lesions without areas of uninvolved skin (Figure).

Treatment of this variant does not differ from conventional therapies for pityriasis versicolor, except that a longer treatment period might be required. Atrophy usually disappears, showing that atrophic pityriasis versicolor has a relatively good prognosis compared with other diseases that cause skin atrophy.²

Our patient was treated with ketoconazole gel 2% once daily for 3 weeks with complete resolution of the lesions and no evidence of atrophy.

The Diagnosis: Cutaneous Leishmaniasis

On examination, the patient had multiple punched-out ulcers with indurated borders and surrounding erythema arranged in a sporotrichoid pattern from the left forearm to the left lateral chest (Figure 1).

Figure 1. Sporotrichoid spread of ulcers from the distal forearm to the lateral chest.

Bacterial culture of a tissue specimen was negative, and tissue fungal culture failed to grow any organisms. Serological studies included a complete blood cell count with differential, a chemistry panel, and liver function tests, which were all unremarkable. Coccidioidomycosis and human immunodefi-ciency virus antibodies were negative. A 4-mm punch biopsy was obtained and sent to the Armed Forces Institute of Pathology for review. Histopathologic examination revealed marked inflammation with ill-formed noncaseating granulomas and focal ulceration, necrosis in the deep dermis, and both intra-cellular and extracellular amastigotes within areas of necrosis (Figures 2 and 3).

Figure 2. Mixed dermal inflammation with ill-formed, noncaseating granulomas (H&E, original magnification ×100).

Figure 3. Multiple intracellular amastigotes are highlighted by the special stain, appearing as tiny “granules” in the cytoplasm of histiocytes (Giemsa, original magnification ×400).

The rise in the number of cases of cutaneous leishmaniasis in the United States, particularly in the veteran population, can be attributed to the recent conflicts in the Middle East and Afghanistan. Infection with Leishmania species can result in a variety of clinical presentations, ranging from localized, self-limited cutaneous lesions to a life-threatening infection with visceral involvement.¹ Additionally, the host immune response is variable. This variation in clinical presentation and disease progression explains why there is no single best treatment identified for leishmaniasis to date.

The clinical pattern of spread along the lymphatics in this patient is unique. The differential diagnosis of lesions with sporotrichoid spread includes Mycobacterium marinum and other atypical mycobacterial infections, Sporothrix schenckii, nocardiosis, leishmaniasis, coccidioidomycosis, tularemia, cat scratch disease, anthrax, chromoblastomycosis, pyogenic bacteria, and other fungal or bacterial infections. With such a broad differential diagnosis, histologic confirmation is paramount.

The most widely used pharmacotherapy for leishmaniasis is with pentavalent antimony compounds, which have been studied in randomized controlled trials for leishmaniasis more than any other drug.² These antimony compounds are associated with a large spectrum of clinical adverse events, and there is increasing evidence for emerging parasite resistance to the antimonies.^3-5 Historically, amphotericin B was considered a second-line treatment of leishmaniasis due to its systemic toxicity.⁶ However, this treatment has come back into favor due to its newer, more tolerable, lipid-associated formulation.

Our patient was treated with intravenous liposomal amphotericin B at a dosage of 3 mg/kg daily for days 1 to 5, then again on days 14 and 21. He tolerated the therapeutic regimen without difficulty or adverse effects. The ulcers eventually became smaller and ceased to weep, fully healing over a course of several months.

The Diagnosis: Cutaneous Leishmaniasis

On examination, the patient had multiple punched-out ulcers with indurated borders and surrounding erythema arranged in a sporotrichoid pattern from the left forearm to the left lateral chest (Figure 1).

Figure 1. Sporotrichoid spread of ulcers from the distal forearm to the lateral chest.

Bacterial culture of a tissue specimen was negative, and tissue fungal culture failed to grow any organisms. Serological studies included a complete blood cell count with differential, a chemistry panel, and liver function tests, which were all unremarkable. Coccidioidomycosis and human immunodefi-ciency virus antibodies were negative. A 4-mm punch biopsy was obtained and sent to the Armed Forces Institute of Pathology for review. Histopathologic examination revealed marked inflammation with ill-formed noncaseating granulomas and focal ulceration, necrosis in the deep dermis, and both intra-cellular and extracellular amastigotes within areas of necrosis (Figures 2 and 3).

Figure 2. Mixed dermal inflammation with ill-formed, noncaseating granulomas (H&E, original magnification ×100).

Figure 3. Multiple intracellular amastigotes are highlighted by the special stain, appearing as tiny “granules” in the cytoplasm of histiocytes (Giemsa, original magnification ×400).

The rise in the number of cases of cutaneous leishmaniasis in the United States, particularly in the veteran population, can be attributed to the recent conflicts in the Middle East and Afghanistan. Infection with Leishmania species can result in a variety of clinical presentations, ranging from localized, self-limited cutaneous lesions to a life-threatening infection with visceral involvement.¹ Additionally, the host immune response is variable. This variation in clinical presentation and disease progression explains why there is no single best treatment identified for leishmaniasis to date.

The clinical pattern of spread along the lymphatics in this patient is unique. The differential diagnosis of lesions with sporotrichoid spread includes Mycobacterium marinum and other atypical mycobacterial infections, Sporothrix schenckii, nocardiosis, leishmaniasis, coccidioidomycosis, tularemia, cat scratch disease, anthrax, chromoblastomycosis, pyogenic bacteria, and other fungal or bacterial infections. With such a broad differential diagnosis, histologic confirmation is paramount.

The most widely used pharmacotherapy for leishmaniasis is with pentavalent antimony compounds, which have been studied in randomized controlled trials for leishmaniasis more than any other drug.² These antimony compounds are associated with a large spectrum of clinical adverse events, and there is increasing evidence for emerging parasite resistance to the antimonies.^3-5 Historically, amphotericin B was considered a second-line treatment of leishmaniasis due to its systemic toxicity.⁶ However, this treatment has come back into favor due to its newer, more tolerable, lipid-associated formulation.

Our patient was treated with intravenous liposomal amphotericin B at a dosage of 3 mg/kg daily for days 1 to 5, then again on days 14 and 21. He tolerated the therapeutic regimen without difficulty or adverse effects. The ulcers eventually became smaller and ceased to weep, fully healing over a course of several months.

The Diagnosis: Cutaneous Leishmaniasis

On examination, the patient had multiple punched-out ulcers with indurated borders and surrounding erythema arranged in a sporotrichoid pattern from the left forearm to the left lateral chest (Figure 1).

Figure 1. Sporotrichoid spread of ulcers from the distal forearm to the lateral chest.

Bacterial culture of a tissue specimen was negative, and tissue fungal culture failed to grow any organisms. Serological studies included a complete blood cell count with differential, a chemistry panel, and liver function tests, which were all unremarkable. Coccidioidomycosis and human immunodefi-ciency virus antibodies were negative. A 4-mm punch biopsy was obtained and sent to the Armed Forces Institute of Pathology for review. Histopathologic examination revealed marked inflammation with ill-formed noncaseating granulomas and focal ulceration, necrosis in the deep dermis, and both intra-cellular and extracellular amastigotes within areas of necrosis (Figures 2 and 3).

Figure 2. Mixed dermal inflammation with ill-formed, noncaseating granulomas (H&E, original magnification ×100).

Figure 3. Multiple intracellular amastigotes are highlighted by the special stain, appearing as tiny “granules” in the cytoplasm of histiocytes (Giemsa, original magnification ×400).

The rise in the number of cases of cutaneous leishmaniasis in the United States, particularly in the veteran population, can be attributed to the recent conflicts in the Middle East and Afghanistan. Infection with Leishmania species can result in a variety of clinical presentations, ranging from localized, self-limited cutaneous lesions to a life-threatening infection with visceral involvement.¹ Additionally, the host immune response is variable. This variation in clinical presentation and disease progression explains why there is no single best treatment identified for leishmaniasis to date.

The clinical pattern of spread along the lymphatics in this patient is unique. The differential diagnosis of lesions with sporotrichoid spread includes Mycobacterium marinum and other atypical mycobacterial infections, Sporothrix schenckii, nocardiosis, leishmaniasis, coccidioidomycosis, tularemia, cat scratch disease, anthrax, chromoblastomycosis, pyogenic bacteria, and other fungal or bacterial infections. With such a broad differential diagnosis, histologic confirmation is paramount.

The most widely used pharmacotherapy for leishmaniasis is with pentavalent antimony compounds, which have been studied in randomized controlled trials for leishmaniasis more than any other drug.² These antimony compounds are associated with a large spectrum of clinical adverse events, and there is increasing evidence for emerging parasite resistance to the antimonies.^3-5 Historically, amphotericin B was considered a second-line treatment of leishmaniasis due to its systemic toxicity.⁶ However, this treatment has come back into favor due to its newer, more tolerable, lipid-associated formulation.

Our patient was treated with intravenous liposomal amphotericin B at a dosage of 3 mg/kg daily for days 1 to 5, then again on days 14 and 21. He tolerated the therapeutic regimen without difficulty or adverse effects. The ulcers eventually became smaller and ceased to weep, fully healing over a course of several months.

A 56-year-old woman presented with painful, erythematous to violaceous patches with necrosis of the left eye and periorbital area of 1 day’s duration. She reported headaches and periorbital pain in the 3 weeks prior to presentation. She was being treated for hypertension, type 2 diabetes mellitus, and end-stage renal disease. The patient denied prior trauma to the area.

The Diagnosis: Rhino-orbital-cerebral Mucormycosis

Cutaneous examination revealed a dusky, erythematous to violaceous patch with a necrotic center involving the left eye and periorbital area (Figure 1). The differential diagnosis included herpes zoster, cellulitis, and fungal infection. We obtained patient consent for a punch biopsy. Histopathologic examination revealed irregularly shaped, broad, nonseptate hyphae with right-angle branching (Figure 2). Magnetic resonance imaging of the orbit and head showed involvement of the periorbital soft tissues; the ethmoidal, sphenoidal, and maxillary sinuses; and the left medial temporal lobe. The patient was started on an empirical antifungal treatment of amphotericin B deoxycholate 50 mg daily but died 4 days later due to multiorgan failure.

Figure 1. A dusky, erythematous to violaceous patch with a necrotic center on the left eye and periorbital area.

Figure 2. Histopathologic examination revealed irregularly shaped, broad, nonseptate hyphae with right-angle branching (periodic acid–Schiff, original magnification ×400).

Mucormycosis is a rare but fatal infection that may rapidly progress.¹ Risk factors include defects in host defense such as malignancy, immunodeficiency from bone marrow or solid organ transplantation, diabetes mellitus, malnutrition, abnormal metabolic states, and deferoxamine use.^1,2 Rhino-orbital-cerebral mucormycosis usually starts with eye or facial pain and unilateral facial swelling.^3,4 Visual impairment, fever, and mental status changes may follow.^1,3,4 Skin findings may progress from erythema to violaceous color changes and lastly to a black necrotic eschar resulting from tissue infarction.⁵

Radiologic imaging may be helpful but rarely is diagnostic in mucormycosis, and reliable serologic tests are lacking.¹ Therefore, suspicion of mucormycosis based on clinical and histopathologic factors followed by immediate initiation of empirical antifungal treatment is critical. The key factors in treating mucormycosis include early diagnosis, correction of underlying risk factors, prompt antifungal therapy, and surgical debridement.¹ Amphotericin B deoxycholate and its lipid derivatives (eg, amphotericin B lipid complex, liposomal amphotericin B) are the standard antifungal agents used in the treatment of mucormycosis.^6,7 Posaconazole is an extended-spectrum triazole with in vitro activity against Mucorales. Posaconazole may be useful as salvage therapy; however, strong clinical evidence to support its role as a primary therapeutic agent is lacking in the literature.^6,7 Blood vessel thrombosis and tissue necrosis can result in poor penetration of antifungal agents to the infection site; therefore, surgical debridement also may be critical for complete eradication of the disease.⁶ Confirmative diagnosis of mucormycosis can be made based on histopathologic findings.

Our case highlights the importance of clinician awareness of the typical presentation of rhino-orbital-cerebral mucormycosis to ensure prompt diagnosis and initiation of immediate treatment of this possibly fatal infection.

A 56-year-old woman presented with painful, erythematous to violaceous patches with necrosis of the left eye and periorbital area of 1 day’s duration. She reported headaches and periorbital pain in the 3 weeks prior to presentation. She was being treated for hypertension, type 2 diabetes mellitus, and end-stage renal disease. The patient denied prior trauma to the area.

The Diagnosis: Rhino-orbital-cerebral Mucormycosis

Cutaneous examination revealed a dusky, erythematous to violaceous patch with a necrotic center involving the left eye and periorbital area (Figure 1). The differential diagnosis included herpes zoster, cellulitis, and fungal infection. We obtained patient consent for a punch biopsy. Histopathologic examination revealed irregularly shaped, broad, nonseptate hyphae with right-angle branching (Figure 2). Magnetic resonance imaging of the orbit and head showed involvement of the periorbital soft tissues; the ethmoidal, sphenoidal, and maxillary sinuses; and the left medial temporal lobe. The patient was started on an empirical antifungal treatment of amphotericin B deoxycholate 50 mg daily but died 4 days later due to multiorgan failure.

Figure 1. A dusky, erythematous to violaceous patch with a necrotic center on the left eye and periorbital area.

Figure 2. Histopathologic examination revealed irregularly shaped, broad, nonseptate hyphae with right-angle branching (periodic acid–Schiff, original magnification ×400).

Mucormycosis is a rare but fatal infection that may rapidly progress.¹ Risk factors include defects in host defense such as malignancy, immunodeficiency from bone marrow or solid organ transplantation, diabetes mellitus, malnutrition, abnormal metabolic states, and deferoxamine use.^1,2 Rhino-orbital-cerebral mucormycosis usually starts with eye or facial pain and unilateral facial swelling.^3,4 Visual impairment, fever, and mental status changes may follow.^1,3,4 Skin findings may progress from erythema to violaceous color changes and lastly to a black necrotic eschar resulting from tissue infarction.⁵

Radiologic imaging may be helpful but rarely is diagnostic in mucormycosis, and reliable serologic tests are lacking.¹ Therefore, suspicion of mucormycosis based on clinical and histopathologic factors followed by immediate initiation of empirical antifungal treatment is critical. The key factors in treating mucormycosis include early diagnosis, correction of underlying risk factors, prompt antifungal therapy, and surgical debridement.¹ Amphotericin B deoxycholate and its lipid derivatives (eg, amphotericin B lipid complex, liposomal amphotericin B) are the standard antifungal agents used in the treatment of mucormycosis.^6,7 Posaconazole is an extended-spectrum triazole with in vitro activity against Mucorales. Posaconazole may be useful as salvage therapy; however, strong clinical evidence to support its role as a primary therapeutic agent is lacking in the literature.^6,7 Blood vessel thrombosis and tissue necrosis can result in poor penetration of antifungal agents to the infection site; therefore, surgical debridement also may be critical for complete eradication of the disease.⁶ Confirmative diagnosis of mucormycosis can be made based on histopathologic findings.

Our case highlights the importance of clinician awareness of the typical presentation of rhino-orbital-cerebral mucormycosis to ensure prompt diagnosis and initiation of immediate treatment of this possibly fatal infection.

A 56-year-old woman presented with painful, erythematous to violaceous patches with necrosis of the left eye and periorbital area of 1 day’s duration. She reported headaches and periorbital pain in the 3 weeks prior to presentation. She was being treated for hypertension, type 2 diabetes mellitus, and end-stage renal disease. The patient denied prior trauma to the area.

The Diagnosis: Rhino-orbital-cerebral Mucormycosis

Cutaneous examination revealed a dusky, erythematous to violaceous patch with a necrotic center involving the left eye and periorbital area (Figure 1). The differential diagnosis included herpes zoster, cellulitis, and fungal infection. We obtained patient consent for a punch biopsy. Histopathologic examination revealed irregularly shaped, broad, nonseptate hyphae with right-angle branching (Figure 2). Magnetic resonance imaging of the orbit and head showed involvement of the periorbital soft tissues; the ethmoidal, sphenoidal, and maxillary sinuses; and the left medial temporal lobe. The patient was started on an empirical antifungal treatment of amphotericin B deoxycholate 50 mg daily but died 4 days later due to multiorgan failure.

Figure 1. A dusky, erythematous to violaceous patch with a necrotic center on the left eye and periorbital area.

Figure 2. Histopathologic examination revealed irregularly shaped, broad, nonseptate hyphae with right-angle branching (periodic acid–Schiff, original magnification ×400).

Mucormycosis is a rare but fatal infection that may rapidly progress.¹ Risk factors include defects in host defense such as malignancy, immunodeficiency from bone marrow or solid organ transplantation, diabetes mellitus, malnutrition, abnormal metabolic states, and deferoxamine use.^1,2 Rhino-orbital-cerebral mucormycosis usually starts with eye or facial pain and unilateral facial swelling.^3,4 Visual impairment, fever, and mental status changes may follow.^1,3,4 Skin findings may progress from erythema to violaceous color changes and lastly to a black necrotic eschar resulting from tissue infarction.⁵

Radiologic imaging may be helpful but rarely is diagnostic in mucormycosis, and reliable serologic tests are lacking.¹ Therefore, suspicion of mucormycosis based on clinical and histopathologic factors followed by immediate initiation of empirical antifungal treatment is critical. The key factors in treating mucormycosis include early diagnosis, correction of underlying risk factors, prompt antifungal therapy, and surgical debridement.¹ Amphotericin B deoxycholate and its lipid derivatives (eg, amphotericin B lipid complex, liposomal amphotericin B) are the standard antifungal agents used in the treatment of mucormycosis.^6,7 Posaconazole is an extended-spectrum triazole with in vitro activity against Mucorales. Posaconazole may be useful as salvage therapy; however, strong clinical evidence to support its role as a primary therapeutic agent is lacking in the literature.^6,7 Blood vessel thrombosis and tissue necrosis can result in poor penetration of antifungal agents to the infection site; therefore, surgical debridement also may be critical for complete eradication of the disease.⁶ Confirmative diagnosis of mucormycosis can be made based on histopathologic findings.

Our case highlights the importance of clinician awareness of the typical presentation of rhino-orbital-cerebral mucormycosis to ensure prompt diagnosis and initiation of immediate treatment of this possibly fatal infection.

The Diagnosis: D-Penicillamine–Induced Elastosis Perforans Serpiginosa

A 27-year-old woman was referred to our clinic by her rheumatologist with a 2×5-cm annular plaque on the right side of the lateral neck along with nummular plaques on the left side of 1 year’s duration (Figure 1). Her medical history was notable for systemic sclerosis that had been treated with oral D-penicillamine (750 mg daily) for the last 10 years. Histopathologic examination of the skin biopsy specimen revealed an epidermis with perforating channels of elastic fibers admixed with collagen (Figure 2). Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (Figure 3), which confirmed the diagnosis of D-penicillamine–induced elastosis perforans serpiginosa (EPS).

Figure 1. Nummular plaques on the left side of the lateral neck.

Figure 2. A punch biopsy specimen showed an epidermis with perforating channels of elastic fibers admixed with collagen (H&E, original magnification ×10).

Figure 3. Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (original magnification ×40).

Elastosis perforans serpiginosa is a rare entity that may present in many different settings. Some associated genetic conditions include Down syndrome, pseudoxanthoma elasticum, Marfan syndrome, Ehlers-Danlos syndrome, acrogeria, osteogenesis imperfecta, Rothmund-Thomson syndrome, and moya-moya disease. Elastosis perforans serpiginosa also may be inherited in rare cases in an autosomal-dominant pattern.¹ There are solitary reports of EPS in the setting of renal disease, morphea, and systemic sclerosis. Most cases of EPS are iatrogenically acquired. As first reported in 1973, long-term D-penicillamine therapy for Wilson disease has been classically associated with the rare development of EPS.² Putative mechanisms include copper chelation by D-penicillamine in the setting of altered copper homeostasis in Wilson disease and subsequent inhibition of elastic fiber cross-linking by copper-dependent lysyl oxidase. Another proposed mechanism is the direct inhibition of collagen cross-linking by D-penicillamine resulting in abnormal elastic fiber maturation.³ Outside of the context of Wilson disease, D-penicillamine–induced EPS also has developed during the treatment of juvenile rheumatoid arthritis and cystinuria.⁴ Our patient withsystemic sclerosis also exemplifies the possibility of developing EPS from long-term D-penicillamine therapy even in the absence of coexisting Wilson disease.

Elastosis perforans serpiginosa lesions classically present as asymptomatic, serpiginously arranged, hyperkeratotic papules, nodules, and annular plaques in young adults and children. Lesions usually present on the neck, though other locations have been described. Histologically, transepidermal elimination of elastic fibers, degenerated keratinocytes, and collagen is seen in the background of a foreign-body reaction with  hematoxylin and eosin stain. Elastin stains show increased thickened elastic fibers in the dermis underlying the perforation. The histology of D-penicillamine–induced EPS is distinctive in that the elastic fibers are arranged in a bramble bush pattern with lateral buds.

The clinical course of D-penicillamine–induced EPS is variable, ranging from slow to no resolution after drug discontinuation, with residual scarring, atrophy, and concern for systemic elastosis. Adjunctive therapies include oral and topical retinoids, cryotherapy, imiquimod, and CO₂ laser.⁵ For our patient, tazarotene gel 0.1% was recommended, but the patient became pregnant soon after the diagnosis was made. Despite being untreated, her lesions have remarkably improved during her pregnancy.

The Diagnosis: D-Penicillamine–Induced Elastosis Perforans Serpiginosa

A 27-year-old woman was referred to our clinic by her rheumatologist with a 2×5-cm annular plaque on the right side of the lateral neck along with nummular plaques on the left side of 1 year’s duration (Figure 1). Her medical history was notable for systemic sclerosis that had been treated with oral D-penicillamine (750 mg daily) for the last 10 years. Histopathologic examination of the skin biopsy specimen revealed an epidermis with perforating channels of elastic fibers admixed with collagen (Figure 2). Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (Figure 3), which confirmed the diagnosis of D-penicillamine–induced elastosis perforans serpiginosa (EPS).

Figure 1. Nummular plaques on the left side of the lateral neck.

Figure 2. A punch biopsy specimen showed an epidermis with perforating channels of elastic fibers admixed with collagen (H&E, original magnification ×10).

Figure 3. Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (original magnification ×40).

Elastosis perforans serpiginosa is a rare entity that may present in many different settings. Some associated genetic conditions include Down syndrome, pseudoxanthoma elasticum, Marfan syndrome, Ehlers-Danlos syndrome, acrogeria, osteogenesis imperfecta, Rothmund-Thomson syndrome, and moya-moya disease. Elastosis perforans serpiginosa also may be inherited in rare cases in an autosomal-dominant pattern.¹ There are solitary reports of EPS in the setting of renal disease, morphea, and systemic sclerosis. Most cases of EPS are iatrogenically acquired. As first reported in 1973, long-term D-penicillamine therapy for Wilson disease has been classically associated with the rare development of EPS.² Putative mechanisms include copper chelation by D-penicillamine in the setting of altered copper homeostasis in Wilson disease and subsequent inhibition of elastic fiber cross-linking by copper-dependent lysyl oxidase. Another proposed mechanism is the direct inhibition of collagen cross-linking by D-penicillamine resulting in abnormal elastic fiber maturation.³ Outside of the context of Wilson disease, D-penicillamine–induced EPS also has developed during the treatment of juvenile rheumatoid arthritis and cystinuria.⁴ Our patient withsystemic sclerosis also exemplifies the possibility of developing EPS from long-term D-penicillamine therapy even in the absence of coexisting Wilson disease.

Elastosis perforans serpiginosa lesions classically present as asymptomatic, serpiginously arranged, hyperkeratotic papules, nodules, and annular plaques in young adults and children. Lesions usually present on the neck, though other locations have been described. Histologically, transepidermal elimination of elastic fibers, degenerated keratinocytes, and collagen is seen in the background of a foreign-body reaction with  hematoxylin and eosin stain. Elastin stains show increased thickened elastic fibers in the dermis underlying the perforation. The histology of D-penicillamine–induced EPS is distinctive in that the elastic fibers are arranged in a bramble bush pattern with lateral buds.

The clinical course of D-penicillamine–induced EPS is variable, ranging from slow to no resolution after drug discontinuation, with residual scarring, atrophy, and concern for systemic elastosis. Adjunctive therapies include oral and topical retinoids, cryotherapy, imiquimod, and CO₂ laser.⁵ For our patient, tazarotene gel 0.1% was recommended, but the patient became pregnant soon after the diagnosis was made. Despite being untreated, her lesions have remarkably improved during her pregnancy.

The Diagnosis: D-Penicillamine–Induced Elastosis Perforans Serpiginosa

A 27-year-old woman was referred to our clinic by her rheumatologist with a 2×5-cm annular plaque on the right side of the lateral neck along with nummular plaques on the left side of 1 year’s duration (Figure 1). Her medical history was notable for systemic sclerosis that had been treated with oral D-penicillamine (750 mg daily) for the last 10 years. Histopathologic examination of the skin biopsy specimen revealed an epidermis with perforating channels of elastic fibers admixed with collagen (Figure 2). Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (Figure 3), which confirmed the diagnosis of D-penicillamine–induced elastosis perforans serpiginosa (EPS).

Figure 1. Nummular plaques on the left side of the lateral neck.

Figure 2. A punch biopsy specimen showed an epidermis with perforating channels of elastic fibers admixed with collagen (H&E, original magnification ×10).

Figure 3. Verhoeff–van Gieson elastin stain highlighted “bramble bush or lumpy-bumpy” appearance of elastic fibers in the dermis (original magnification ×40).

Elastosis perforans serpiginosa is a rare entity that may present in many different settings. Some associated genetic conditions include Down syndrome, pseudoxanthoma elasticum, Marfan syndrome, Ehlers-Danlos syndrome, acrogeria, osteogenesis imperfecta, Rothmund-Thomson syndrome, and moya-moya disease. Elastosis perforans serpiginosa also may be inherited in rare cases in an autosomal-dominant pattern.¹ There are solitary reports of EPS in the setting of renal disease, morphea, and systemic sclerosis. Most cases of EPS are iatrogenically acquired. As first reported in 1973, long-term D-penicillamine therapy for Wilson disease has been classically associated with the rare development of EPS.² Putative mechanisms include copper chelation by D-penicillamine in the setting of altered copper homeostasis in Wilson disease and subsequent inhibition of elastic fiber cross-linking by copper-dependent lysyl oxidase. Another proposed mechanism is the direct inhibition of collagen cross-linking by D-penicillamine resulting in abnormal elastic fiber maturation.³ Outside of the context of Wilson disease, D-penicillamine–induced EPS also has developed during the treatment of juvenile rheumatoid arthritis and cystinuria.⁴ Our patient withsystemic sclerosis also exemplifies the possibility of developing EPS from long-term D-penicillamine therapy even in the absence of coexisting Wilson disease.

Elastosis perforans serpiginosa lesions classically present as asymptomatic, serpiginously arranged, hyperkeratotic papules, nodules, and annular plaques in young adults and children. Lesions usually present on the neck, though other locations have been described. Histologically, transepidermal elimination of elastic fibers, degenerated keratinocytes, and collagen is seen in the background of a foreign-body reaction with  hematoxylin and eosin stain. Elastin stains show increased thickened elastic fibers in the dermis underlying the perforation. The histology of D-penicillamine–induced EPS is distinctive in that the elastic fibers are arranged in a bramble bush pattern with lateral buds.

The clinical course of D-penicillamine–induced EPS is variable, ranging from slow to no resolution after drug discontinuation, with residual scarring, atrophy, and concern for systemic elastosis. Adjunctive therapies include oral and topical retinoids, cryotherapy, imiquimod, and CO₂ laser.⁵ For our patient, tazarotene gel 0.1% was recommended, but the patient became pregnant soon after the diagnosis was made. Despite being untreated, her lesions have remarkably improved during her pregnancy.

The Diagnosis: Lues Maligna

Biopsy revealed dense nodular aggregates of lymphocytes, histiocytes, and abundant plasma cells in both the superficial and deep dermis (Figure 1). There were perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (Figure 2). Special stains for organisms, including Warthin-Starry silver, Giemsa, acid-fast bacilli, Gomori methenamine-silver, and Brown-Brenn stains were negative. Immunoperoxidase stain for Treponema pallidum also was negative. The patient’s rapid plasma reagin titer at the time of the fourth biopsy was 1:256, and appropriate treatment with penicillin resulted in complete clearance of the lesions in 3 to 4 weeks.

Figure 1. Superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate (H&E, original magnification ×2).

Figure 2. Perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (H&E, original magnification ×20).

Syphilis is caused by T pallidum. Three stages typically are identified in immunocompetent hosts: primary, secondary, and tertiary syphilis. Immunocompromised patients with human immunodeficiency virus (HIV) infection may have unusual presentations.

Lues maligna is used to describe a rare noduloulcerative form of secondary syphilis.¹ It was first described in 1859² and has been associated with other disorders such as diabetes mellitus³ and chronic alcoholism.⁴ Patients usually are gravely ill and develop polymorphic ulcerating lesions. Facial and scalp involvement are common, but patients typically do not have palmoplantar involvement in conventional presentations of secondary syphilis.

A scanning view of a punch biopsy from our patient revealed irregular acanthosis of the epidermis with long and thin rete pegs, a bandlike infiltrate at the dermoepidermal junction, and a dense superficial and deep perivascular and periadnexal infiltrate. The histologic differential diagnosis includes pyoderma gangrenosum, vasculitis, lymphoma, leishmaniasis, leprosy, yaws, and mycobacterial or fungal infections.

The Centers for Disease Control and Prevention recommends screening of all HIV-positive indivi-duals for syphilis, and all sexually active individuals with syphilis should be screened for HIV.⁵ If clinical examination and findings suggest syphilis in the presence of negative serologic testing, then direct fluorescence assay for T pallidum staining of lesions, exudates or biopsy, or dark-field microscopic examination should be performed. In our case, dark-field microscopy was not performed and serologic tests were negative at presentation. Silver stains can detect T pallidum in tissue specimens, though detection may not be possible late in the course of disease.⁶

The morphology and rapid response to treatment confirmed the diagnosis in our patient. The incidence of syphilis in HIV-positive patients has risen substantially in the last 2 decades. This case illustrates an uncommon presentation that is increasing in prevalence.

The Diagnosis: Lues Maligna

Biopsy revealed dense nodular aggregates of lymphocytes, histiocytes, and abundant plasma cells in both the superficial and deep dermis (Figure 1). There were perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (Figure 2). Special stains for organisms, including Warthin-Starry silver, Giemsa, acid-fast bacilli, Gomori methenamine-silver, and Brown-Brenn stains were negative. Immunoperoxidase stain for Treponema pallidum also was negative. The patient’s rapid plasma reagin titer at the time of the fourth biopsy was 1:256, and appropriate treatment with penicillin resulted in complete clearance of the lesions in 3 to 4 weeks.

Figure 1. Superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate (H&E, original magnification ×2).

Figure 2. Perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (H&E, original magnification ×20).

Syphilis is caused by T pallidum. Three stages typically are identified in immunocompetent hosts: primary, secondary, and tertiary syphilis. Immunocompromised patients with human immunodeficiency virus (HIV) infection may have unusual presentations.

Lues maligna is used to describe a rare noduloulcerative form of secondary syphilis.¹ It was first described in 1859² and has been associated with other disorders such as diabetes mellitus³ and chronic alcoholism.⁴ Patients usually are gravely ill and develop polymorphic ulcerating lesions. Facial and scalp involvement are common, but patients typically do not have palmoplantar involvement in conventional presentations of secondary syphilis.

A scanning view of a punch biopsy from our patient revealed irregular acanthosis of the epidermis with long and thin rete pegs, a bandlike infiltrate at the dermoepidermal junction, and a dense superficial and deep perivascular and periadnexal infiltrate. The histologic differential diagnosis includes pyoderma gangrenosum, vasculitis, lymphoma, leishmaniasis, leprosy, yaws, and mycobacterial or fungal infections.

The Centers for Disease Control and Prevention recommends screening of all HIV-positive indivi-duals for syphilis, and all sexually active individuals with syphilis should be screened for HIV.⁵ If clinical examination and findings suggest syphilis in the presence of negative serologic testing, then direct fluorescence assay for T pallidum staining of lesions, exudates or biopsy, or dark-field microscopic examination should be performed. In our case, dark-field microscopy was not performed and serologic tests were negative at presentation. Silver stains can detect T pallidum in tissue specimens, though detection may not be possible late in the course of disease.⁶

The morphology and rapid response to treatment confirmed the diagnosis in our patient. The incidence of syphilis in HIV-positive patients has risen substantially in the last 2 decades. This case illustrates an uncommon presentation that is increasing in prevalence.

The Diagnosis: Lues Maligna

Biopsy revealed dense nodular aggregates of lymphocytes, histiocytes, and abundant plasma cells in both the superficial and deep dermis (Figure 1). There were perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (Figure 2). Special stains for organisms, including Warthin-Starry silver, Giemsa, acid-fast bacilli, Gomori methenamine-silver, and Brown-Brenn stains were negative. Immunoperoxidase stain for Treponema pallidum also was negative. The patient’s rapid plasma reagin titer at the time of the fourth biopsy was 1:256, and appropriate treatment with penicillin resulted in complete clearance of the lesions in 3 to 4 weeks.

Figure 1. Superficial and deep perivascular and periadnexal lymphohistiocytic infiltrate (H&E, original magnification ×2).

Figure 2. Perivascular and periadnexal aggregates of lymphocytes, histiocytes, and numerous plasma cells (H&E, original magnification ×20).

Syphilis is caused by T pallidum. Three stages typically are identified in immunocompetent hosts: primary, secondary, and tertiary syphilis. Immunocompromised patients with human immunodeficiency virus (HIV) infection may have unusual presentations.

Lues maligna is used to describe a rare noduloulcerative form of secondary syphilis.¹ It was first described in 1859² and has been associated with other disorders such as diabetes mellitus³ and chronic alcoholism.⁴ Patients usually are gravely ill and develop polymorphic ulcerating lesions. Facial and scalp involvement are common, but patients typically do not have palmoplantar involvement in conventional presentations of secondary syphilis.

A scanning view of a punch biopsy from our patient revealed irregular acanthosis of the epidermis with long and thin rete pegs, a bandlike infiltrate at the dermoepidermal junction, and a dense superficial and deep perivascular and periadnexal infiltrate. The histologic differential diagnosis includes pyoderma gangrenosum, vasculitis, lymphoma, leishmaniasis, leprosy, yaws, and mycobacterial or fungal infections.

The Centers for Disease Control and Prevention recommends screening of all HIV-positive indivi-duals for syphilis, and all sexually active individuals with syphilis should be screened for HIV.⁵ If clinical examination and findings suggest syphilis in the presence of negative serologic testing, then direct fluorescence assay for T pallidum staining of lesions, exudates or biopsy, or dark-field microscopic examination should be performed. In our case, dark-field microscopy was not performed and serologic tests were negative at presentation. Silver stains can detect T pallidum in tissue specimens, though detection may not be possible late in the course of disease.⁶

The morphology and rapid response to treatment confirmed the diagnosis in our patient. The incidence of syphilis in HIV-positive patients has risen substantially in the last 2 decades. This case illustrates an uncommon presentation that is increasing in prevalence.

An 82-year-old man presented for evaluation of a solitary asymptomatic, pink, velvety, exophytic nodule on the scrotum that had been present for approximately 1 month. His medical history was notable for Parkinson disease, hypertension, and hyperlipidemia. His current medications included carbidopa-levodopa, lisinopril-hydrochlorothiazide, amlodipine, and simvastatin.

The Diagnosis: Verruciform Xanthoma

Our patient presented with a solitary asymptomatic, pink, velvety, exophytic nodule on the scrotum that had been present for approximately 1 month (Figure 1). Biopsy of the specimen showed a lesion with a verrucalike configuration. There was hyperkeratosis, focal parakeratosis, and verrucous acanthosis without atypia. The papillary dermis was filled with large histiocytes with foamy cytoplasm (xanthoma cells). A few lymphocytes, neutrophils, eosinophils, and plasma cells also were present (Figure 2).

Figure 1. A solitary pink, velvety, exophytic nodule on the scrotum.

Figure 2. Biopsy showed a lesion with a verrucalike configuration (A)(H&E, original magnification ×20). The papillary dermis was filled with xanthoma cells (B)(H&E, original magnification ×400).

Clinically, the differential diagnosis of this lesion includes verruciform xanthoma (VX), verruca vulgaris, condyloma, verrucous carcinoma, and squamous cell carcinoma; however, the histologic finding of xanthoma cells in the papillary dermis helped to confirm the diagnosis of VX. Specimens must be evaluated closely to rule out associated or concurrent disease processes.

Verruciform xanthoma is a rare lesion first described by Shafer¹ in 1971. It usually appears as a solitary lesion most commonly presenting on the oral mucosa. In 2003, a worldwide survey of the literature profiled a total of 282 oral and 46 extraoral VX lesions.² Extraoral cutaneous lesions are less common and usually present in the anogenital region, including the penis, scrotum, vulva, and anus. Isolated cases have been reported on the extremities and other sites.^3,4 The lesions often are described as pale yellow to pink verrucous papules or plaques. Typical histologic features include hyperkeratosis, focal parakeratosis, acanthosis without atypia, and xanthoma cells in the papillary dermis that usually do not extend beyond the rete ridges.^3,5 A mild inflammatory infiltrate is common with some lymphocytes, plasma cells, neutrophils, and eosinophils.³

Several reports have noted that VX does not occur in the presence of any lipid abnormalities or detectable human papillomavirus.^3,6-9 Cases may occur sporadically without any apparent associated disease but also have been seen in the setting of atypia, malignancy, and inflammatory processes. There are at least 2 reports of VX lesions occurring with squamous cell carcinoma.^5,10 Verruciform xanthoma also has presented in the setting of epidermal nevi, actinic keratosis, seborrheic keratosis, lichen planus, lichen sclerosus, pemphigus, discoid lupus erythematosus, lymphedema, CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) syndrome, and recessive dystrophic epidermolysis bullosa.^6,7 The association of VX with these other diseases has led many authors to believe that it is a reactive phenomenon to chronic inflammation or cutaneous trauma in which degenerating keratinocytes are phagocytosed by dermal macrophages that then become lipid-laden foam cells. Rapid epidermal growth may be a result of cytokines released during this process.⁴

A Japanese report of several cases of VX with lesions on the scrotum suggested that this rare finding may be caused by chronic pressure associated with the Japanese tradition of sitting on the floor.¹¹ One case occurred with lesions on the penis and perineum 4 years after an incidence of necrotizing fasciitis and skin grafting in the same area. The authors suggested that the severe cutaneous trauma predisposed the patient to forming VX lesions.⁴ There also have been at least 6 cases of VX reported in immunocompromised patients, specifically in the setting of bone marrow transplantation, human immunodeficiency virus infection, common variable immunodeficiency, and renal transplantation.⁸ The proportion of immunocompromised cases is greater than expected considering the relatively low prevalence of cutaneous
VX lesions. A possible explanation that is consistent with the proposed reactive mechanism for VX formation is that immunocompromised patients may have a lower number of epidermal Langerhans cells, which results in decreased removal of degenerated keratinocytes and increased dermal macrophage phagocytosis.⁸

Management of VX lesions usually consists of simple excision. Intraoral excisions have been well documented as curative with rare recurrence^2,9; however, there are limited reports in the literature documenting successful excision of cutaneous lesions. Despite the availability of several treatment modalities (eg, wire loop electrosection, pulsed dye laser, radiation therapy, chloroxylenol scrub, wide surgical resection), lesions may reoccur.⁹ Because VX lesions have occurred in the setting of other cutaneous conditions, patients should be assessed for other concurrent diseases. Biopsy specimens should be carefully analyzed, as a few VX lesions have been associated with epidermal atypia and invasive squamous cell carcinoma.^5,10

An 82-year-old man presented for evaluation of a solitary asymptomatic, pink, velvety, exophytic nodule on the scrotum that had been present for approximately 1 month. His medical history was notable for Parkinson disease, hypertension, and hyperlipidemia. His current medications included carbidopa-levodopa, lisinopril-hydrochlorothiazide, amlodipine, and simvastatin.

The Diagnosis: Verruciform Xanthoma

Our patient presented with a solitary asymptomatic, pink, velvety, exophytic nodule on the scrotum that had been present for approximately 1 month (Figure 1). Biopsy of the specimen showed a lesion with a verrucalike configuration. There was hyperkeratosis, focal parakeratosis, and verrucous acanthosis without atypia. The papillary dermis was filled with large histiocytes with foamy cytoplasm (xanthoma cells). A few lymphocytes, neutrophils, eosinophils, and plasma cells also were present (Figure 2).

Figure 1. A solitary pink, velvety, exophytic nodule on the scrotum.

Figure 2. Biopsy showed a lesion with a verrucalike configuration (A)(H&E, original magnification ×20). The papillary dermis was filled with xanthoma cells (B)(H&E, original magnification ×400).

Clinically, the differential diagnosis of this lesion includes verruciform xanthoma (VX), verruca vulgaris, condyloma, verrucous carcinoma, and squamous cell carcinoma; however, the histologic finding of xanthoma cells in the papillary dermis helped to confirm the diagnosis of VX. Specimens must be evaluated closely to rule out associated or concurrent disease processes.

Verruciform xanthoma is a rare lesion first described by Shafer¹ in 1971. It usually appears as a solitary lesion most commonly presenting on the oral mucosa. In 2003, a worldwide survey of the literature profiled a total of 282 oral and 46 extraoral VX lesions.² Extraoral cutaneous lesions are less common and usually present in the anogenital region, including the penis, scrotum, vulva, and anus. Isolated cases have been reported on the extremities and other sites.^3,4 The lesions often are described as pale yellow to pink verrucous papules or plaques. Typical histologic features include hyperkeratosis, focal parakeratosis, acanthosis without atypia, and xanthoma cells in the papillary dermis that usually do not extend beyond the rete ridges.^3,5 A mild inflammatory infiltrate is common with some lymphocytes, plasma cells, neutrophils, and eosinophils.³

Several reports have noted that VX does not occur in the presence of any lipid abnormalities or detectable human papillomavirus.^3,6-9 Cases may occur sporadically without any apparent associated disease but also have been seen in the setting of atypia, malignancy, and inflammatory processes. There are at least 2 reports of VX lesions occurring with squamous cell carcinoma.^5,10 Verruciform xanthoma also has presented in the setting of epidermal nevi, actinic keratosis, seborrheic keratosis, lichen planus, lichen sclerosus, pemphigus, discoid lupus erythematosus, lymphedema, CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) syndrome, and recessive dystrophic epidermolysis bullosa.^6,7 The association of VX with these other diseases has led many authors to believe that it is a reactive phenomenon to chronic inflammation or cutaneous trauma in which degenerating keratinocytes are phagocytosed by dermal macrophages that then become lipid-laden foam cells. Rapid epidermal growth may be a result of cytokines released during this process.⁴

A Japanese report of several cases of VX with lesions on the scrotum suggested that this rare finding may be caused by chronic pressure associated with the Japanese tradition of sitting on the floor.¹¹ One case occurred with lesions on the penis and perineum 4 years after an incidence of necrotizing fasciitis and skin grafting in the same area. The authors suggested that the severe cutaneous trauma predisposed the patient to forming VX lesions.⁴ There also have been at least 6 cases of VX reported in immunocompromised patients, specifically in the setting of bone marrow transplantation, human immunodeficiency virus infection, common variable immunodeficiency, and renal transplantation.⁸ The proportion of immunocompromised cases is greater than expected considering the relatively low prevalence of cutaneous
VX lesions. A possible explanation that is consistent with the proposed reactive mechanism for VX formation is that immunocompromised patients may have a lower number of epidermal Langerhans cells, which results in decreased removal of degenerated keratinocytes and increased dermal macrophage phagocytosis.⁸

Management of VX lesions usually consists of simple excision. Intraoral excisions have been well documented as curative with rare recurrence^2,9; however, there are limited reports in the literature documenting successful excision of cutaneous lesions. Despite the availability of several treatment modalities (eg, wire loop electrosection, pulsed dye laser, radiation therapy, chloroxylenol scrub, wide surgical resection), lesions may reoccur.⁹ Because VX lesions have occurred in the setting of other cutaneous conditions, patients should be assessed for other concurrent diseases. Biopsy specimens should be carefully analyzed, as a few VX lesions have been associated with epidermal atypia and invasive squamous cell carcinoma.^5,10

An 82-year-old man presented for evaluation of a solitary asymptomatic, pink, velvety, exophytic nodule on the scrotum that had been present for approximately 1 month. His medical history was notable for Parkinson disease, hypertension, and hyperlipidemia. His current medications included carbidopa-levodopa, lisinopril-hydrochlorothiazide, amlodipine, and simvastatin.

The Diagnosis: Verruciform Xanthoma

Our patient presented with a solitary asymptomatic, pink, velvety, exophytic nodule on the scrotum that had been present for approximately 1 month (Figure 1). Biopsy of the specimen showed a lesion with a verrucalike configuration. There was hyperkeratosis, focal parakeratosis, and verrucous acanthosis without atypia. The papillary dermis was filled with large histiocytes with foamy cytoplasm (xanthoma cells). A few lymphocytes, neutrophils, eosinophils, and plasma cells also were present (Figure 2).

Figure 1. A solitary pink, velvety, exophytic nodule on the scrotum.

Figure 2. Biopsy showed a lesion with a verrucalike configuration (A)(H&E, original magnification ×20). The papillary dermis was filled with xanthoma cells (B)(H&E, original magnification ×400).

Clinically, the differential diagnosis of this lesion includes verruciform xanthoma (VX), verruca vulgaris, condyloma, verrucous carcinoma, and squamous cell carcinoma; however, the histologic finding of xanthoma cells in the papillary dermis helped to confirm the diagnosis of VX. Specimens must be evaluated closely to rule out associated or concurrent disease processes.

Verruciform xanthoma is a rare lesion first described by Shafer¹ in 1971. It usually appears as a solitary lesion most commonly presenting on the oral mucosa. In 2003, a worldwide survey of the literature profiled a total of 282 oral and 46 extraoral VX lesions.² Extraoral cutaneous lesions are less common and usually present in the anogenital region, including the penis, scrotum, vulva, and anus. Isolated cases have been reported on the extremities and other sites.^3,4 The lesions often are described as pale yellow to pink verrucous papules or plaques. Typical histologic features include hyperkeratosis, focal parakeratosis, acanthosis without atypia, and xanthoma cells in the papillary dermis that usually do not extend beyond the rete ridges.^3,5 A mild inflammatory infiltrate is common with some lymphocytes, plasma cells, neutrophils, and eosinophils.³

Several reports have noted that VX does not occur in the presence of any lipid abnormalities or detectable human papillomavirus.^3,6-9 Cases may occur sporadically without any apparent associated disease but also have been seen in the setting of atypia, malignancy, and inflammatory processes. There are at least 2 reports of VX lesions occurring with squamous cell carcinoma.^5,10 Verruciform xanthoma also has presented in the setting of epidermal nevi, actinic keratosis, seborrheic keratosis, lichen planus, lichen sclerosus, pemphigus, discoid lupus erythematosus, lymphedema, CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) syndrome, and recessive dystrophic epidermolysis bullosa.^6,7 The association of VX with these other diseases has led many authors to believe that it is a reactive phenomenon to chronic inflammation or cutaneous trauma in which degenerating keratinocytes are phagocytosed by dermal macrophages that then become lipid-laden foam cells. Rapid epidermal growth may be a result of cytokines released during this process.⁴

A Japanese report of several cases of VX with lesions on the scrotum suggested that this rare finding may be caused by chronic pressure associated with the Japanese tradition of sitting on the floor.¹¹ One case occurred with lesions on the penis and perineum 4 years after an incidence of necrotizing fasciitis and skin grafting in the same area. The authors suggested that the severe cutaneous trauma predisposed the patient to forming VX lesions.⁴ There also have been at least 6 cases of VX reported in immunocompromised patients, specifically in the setting of bone marrow transplantation, human immunodeficiency virus infection, common variable immunodeficiency, and renal transplantation.⁸ The proportion of immunocompromised cases is greater than expected considering the relatively low prevalence of cutaneous
VX lesions. A possible explanation that is consistent with the proposed reactive mechanism for VX formation is that immunocompromised patients may have a lower number of epidermal Langerhans cells, which results in decreased removal of degenerated keratinocytes and increased dermal macrophage phagocytosis.⁸

Management of VX lesions usually consists of simple excision. Intraoral excisions have been well documented as curative with rare recurrence^2,9; however, there are limited reports in the literature documenting successful excision of cutaneous lesions. Despite the availability of several treatment modalities (eg, wire loop electrosection, pulsed dye laser, radiation therapy, chloroxylenol scrub, wide surgical resection), lesions may reoccur.⁹ Because VX lesions have occurred in the setting of other cutaneous conditions, patients should be assessed for other concurrent diseases. Biopsy specimens should be carefully analyzed, as a few VX lesions have been associated with epidermal atypia and invasive squamous cell carcinoma.^5,10

The Diagnosis: Adult-Onset Xanthogranuloma

Biopsies of the lesions on the neck (Figure 1) and back were performed. Histologic analyses revealed a diffuse dermatitis consisting of foamy histiocytes admixed with a few Touton-type giant cells in the dermis (Figure 2), which was associated with an inflammatory infiltrate of eosinophils and lymphocytes. Laboratory investigations revealed mild thrombocytopenia with a platelet count of 134×10⁹/L (reference range, 140–440×10⁹/L). Other investigations including biochemistry, lipid, serum electrophoresis, and chest radiogram were normal. A bone marrow trephine biopsy and flow cytometry were performed and were normal. Magnetic resonance imaging revealed periorbital soft-tissue masses that did not extend into the orbits.

Figure 1. Firm plaques with a yellowish tinge over the side of the neck

Figure 2. A dense infiltrate of foamy histiocytes in the dermis (A) associated with a Touton-type giant cell (arrow) and an inflammatory infiltrate consisting of eosinophils and lymphocytes (B)(both H&E, original magnifications ×200 and ×400).

Adult-onset xanthogranuloma (AXG) is a rare disease entity, usually presenting in the third to fourth decades of life. The condition typically presents as a red to yellow-brown nodular cutaneous lesion located on the scalp, face, neck, trunk, or limbs. The presentation typically consists of a solitary lesion, occurring in 70% to 89% of cases,¹ but more rarely, as in this case, lesions can be multiple or even disseminated.

Histologically, AXG presents as a dense, well-circumscribed, histiocytic infiltrate consisting of lipophages possessing foamy cytoplasm and giant cells. The presence of histiocytic giant cells differentiates AXG from xanthelasma, a clinical differential diagnosis in this case, and xanthoma. In AXG, there are 4 main types of histiocytes: xanthomatized, spindle shaped, vacuolated, and oncocytic.² They can be seen in variable proportions, together with different types of giant cells (eg, Touton, foreign body, ground glass, nonspecific). A mixed infiltrate of eosinophils, lymphocytes, plasma cells, and neutrophils also may be seen scattered throughout the lesion.²

Correlating with the clinical and histological features of xanthogranuloma, the firm plaques and nodules represent the dense dermal infiltration of histiocytes that may extend into the subcutis. The lesions demonstrate a time-dependent progression both clinically and histologically. Early lesions are comprised of a dense monomorphous nonlipid histiocytic inflammatory infiltrate, and they clinically appear more erythematous. In mature lesions, as in our patient, the infiltrate is predominantly composed of lipid-laden histiocytes with associated Touton giant cells. They appear more yellowish on clinical presentation.

Adult-onset xanthogranuloma is part of a rare heterogenous group of disorders termed adult orbital xanthogranulomatous disease, which includes 3 other syndromes: necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease.

Necrobiotic xanthogranuloma is clinically characterized by the presence of subcutaneous lesions that ulcerate in approximately 40% to 50% of cases and is histologically characterized by necrobiosis with palisading epithelioid histiocytes. It also is systemically associated with paraproteinemia and multiple myeloma.³

Adult-onset asthma and periocular xanthogranuloma is characterized by yellowish papules and nodules predominantly over the lower eyelids that are histologically comprised of lymphoid follicles with reactive germinal centers. It is associated with asthma, which normally is severe and often occurs almost simultaneously with the periorbital lesions.⁴

There are no definite diagnostic criteria for Erdheim-Chester disease and the diagnosis is usually based on radiologic findings of osteosclerosis and histopathologic evidence of foamy histiocytic infiltration. Systemic manifestations are common with lymphohistiocytic infiltration of the heart, lungs, pericardium, bones, and intestines. Prognosis is uniformly dismal.

Based on the clinical presentation of a nonulcerative papulonodular eruption and the absence of systemic involvement including asthma, we made the diagnosis of AXG. In view of the heterogeneity among these clinical entities as well as the time-based evolution of the lesions involved, we continued to monitor the patient for 2 years and there was no development of other systemic manifestations and hematologic abnormalities.

In contrast to the more common form of juvenile-onset xanthogranuloma, the adult type is not associated with widespread visceral lesions. Hence extensive screening investigations for systemic disease generally are not necessary. Another difference is that AXG has been associated with hematologic abnormalities, including essential thrombocytosis, chronic lymphocytic leukemia, large B-cell lymphoma, and monoclonal gammopathy.^5,6 In our patient, the presence of thrombocytopenia and older age caused us to be concerned about an associated hematologic malignancy; therefore, a bone marrow biopsy and flow cytometry were performed.

Adult-onset xanthogranuloma typically is an asymptomatic and self-healing disease and therefore treatment usually is not required. Spontaneous regression of xanthogranuloma was observed to occur in 54% of cases with a median duration of 22 months,⁷ though lesions were noted to last as long as 15 years.⁸ When treatment is necessary, a combination of local and systemic corticosteroids, cytotoxic agents, and radiotherapy have been routinely used. In particular, intralesional corticosteroid therapy has been found to be efficacious in controlling symptoms and signs of AXG affecting the eyelids and orbits while avoiding the systemic side effects of other agents.⁹

Because our patient’s lesions were longstanding and disfiguring, we opted for active intervention with intralesional triamcinolone, which resulted in only a slight reduction in size of the lesions. The lesions remain largely unchanged in 2 years of follow-up.

The Diagnosis: Adult-Onset Xanthogranuloma

Biopsies of the lesions on the neck (Figure 1) and back were performed. Histologic analyses revealed a diffuse dermatitis consisting of foamy histiocytes admixed with a few Touton-type giant cells in the dermis (Figure 2), which was associated with an inflammatory infiltrate of eosinophils and lymphocytes. Laboratory investigations revealed mild thrombocytopenia with a platelet count of 134×10⁹/L (reference range, 140–440×10⁹/L). Other investigations including biochemistry, lipid, serum electrophoresis, and chest radiogram were normal. A bone marrow trephine biopsy and flow cytometry were performed and were normal. Magnetic resonance imaging revealed periorbital soft-tissue masses that did not extend into the orbits.

Figure 1. Firm plaques with a yellowish tinge over the side of the neck

Figure 2. A dense infiltrate of foamy histiocytes in the dermis (A) associated with a Touton-type giant cell (arrow) and an inflammatory infiltrate consisting of eosinophils and lymphocytes (B)(both H&E, original magnifications ×200 and ×400).

Adult-onset xanthogranuloma (AXG) is a rare disease entity, usually presenting in the third to fourth decades of life. The condition typically presents as a red to yellow-brown nodular cutaneous lesion located on the scalp, face, neck, trunk, or limbs. The presentation typically consists of a solitary lesion, occurring in 70% to 89% of cases,¹ but more rarely, as in this case, lesions can be multiple or even disseminated.

Histologically, AXG presents as a dense, well-circumscribed, histiocytic infiltrate consisting of lipophages possessing foamy cytoplasm and giant cells. The presence of histiocytic giant cells differentiates AXG from xanthelasma, a clinical differential diagnosis in this case, and xanthoma. In AXG, there are 4 main types of histiocytes: xanthomatized, spindle shaped, vacuolated, and oncocytic.² They can be seen in variable proportions, together with different types of giant cells (eg, Touton, foreign body, ground glass, nonspecific). A mixed infiltrate of eosinophils, lymphocytes, plasma cells, and neutrophils also may be seen scattered throughout the lesion.²

Correlating with the clinical and histological features of xanthogranuloma, the firm plaques and nodules represent the dense dermal infiltration of histiocytes that may extend into the subcutis. The lesions demonstrate a time-dependent progression both clinically and histologically. Early lesions are comprised of a dense monomorphous nonlipid histiocytic inflammatory infiltrate, and they clinically appear more erythematous. In mature lesions, as in our patient, the infiltrate is predominantly composed of lipid-laden histiocytes with associated Touton giant cells. They appear more yellowish on clinical presentation.

Adult-onset xanthogranuloma is part of a rare heterogenous group of disorders termed adult orbital xanthogranulomatous disease, which includes 3 other syndromes: necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease.

Necrobiotic xanthogranuloma is clinically characterized by the presence of subcutaneous lesions that ulcerate in approximately 40% to 50% of cases and is histologically characterized by necrobiosis with palisading epithelioid histiocytes. It also is systemically associated with paraproteinemia and multiple myeloma.³

Adult-onset asthma and periocular xanthogranuloma is characterized by yellowish papules and nodules predominantly over the lower eyelids that are histologically comprised of lymphoid follicles with reactive germinal centers. It is associated with asthma, which normally is severe and often occurs almost simultaneously with the periorbital lesions.⁴

There are no definite diagnostic criteria for Erdheim-Chester disease and the diagnosis is usually based on radiologic findings of osteosclerosis and histopathologic evidence of foamy histiocytic infiltration. Systemic manifestations are common with lymphohistiocytic infiltration of the heart, lungs, pericardium, bones, and intestines. Prognosis is uniformly dismal.

Based on the clinical presentation of a nonulcerative papulonodular eruption and the absence of systemic involvement including asthma, we made the diagnosis of AXG. In view of the heterogeneity among these clinical entities as well as the time-based evolution of the lesions involved, we continued to monitor the patient for 2 years and there was no development of other systemic manifestations and hematologic abnormalities.

In contrast to the more common form of juvenile-onset xanthogranuloma, the adult type is not associated with widespread visceral lesions. Hence extensive screening investigations for systemic disease generally are not necessary. Another difference is that AXG has been associated with hematologic abnormalities, including essential thrombocytosis, chronic lymphocytic leukemia, large B-cell lymphoma, and monoclonal gammopathy.^5,6 In our patient, the presence of thrombocytopenia and older age caused us to be concerned about an associated hematologic malignancy; therefore, a bone marrow biopsy and flow cytometry were performed.

Adult-onset xanthogranuloma typically is an asymptomatic and self-healing disease and therefore treatment usually is not required. Spontaneous regression of xanthogranuloma was observed to occur in 54% of cases with a median duration of 22 months,⁷ though lesions were noted to last as long as 15 years.⁸ When treatment is necessary, a combination of local and systemic corticosteroids, cytotoxic agents, and radiotherapy have been routinely used. In particular, intralesional corticosteroid therapy has been found to be efficacious in controlling symptoms and signs of AXG affecting the eyelids and orbits while avoiding the systemic side effects of other agents.⁹

Because our patient’s lesions were longstanding and disfiguring, we opted for active intervention with intralesional triamcinolone, which resulted in only a slight reduction in size of the lesions. The lesions remain largely unchanged in 2 years of follow-up.

The Diagnosis: Adult-Onset Xanthogranuloma

Biopsies of the lesions on the neck (Figure 1) and back were performed. Histologic analyses revealed a diffuse dermatitis consisting of foamy histiocytes admixed with a few Touton-type giant cells in the dermis (Figure 2), which was associated with an inflammatory infiltrate of eosinophils and lymphocytes. Laboratory investigations revealed mild thrombocytopenia with a platelet count of 134×10⁹/L (reference range, 140–440×10⁹/L). Other investigations including biochemistry, lipid, serum electrophoresis, and chest radiogram were normal. A bone marrow trephine biopsy and flow cytometry were performed and were normal. Magnetic resonance imaging revealed periorbital soft-tissue masses that did not extend into the orbits.

Figure 1. Firm plaques with a yellowish tinge over the side of the neck

Figure 2. A dense infiltrate of foamy histiocytes in the dermis (A) associated with a Touton-type giant cell (arrow) and an inflammatory infiltrate consisting of eosinophils and lymphocytes (B)(both H&E, original magnifications ×200 and ×400).

Adult-onset xanthogranuloma (AXG) is a rare disease entity, usually presenting in the third to fourth decades of life. The condition typically presents as a red to yellow-brown nodular cutaneous lesion located on the scalp, face, neck, trunk, or limbs. The presentation typically consists of a solitary lesion, occurring in 70% to 89% of cases,¹ but more rarely, as in this case, lesions can be multiple or even disseminated.

Histologically, AXG presents as a dense, well-circumscribed, histiocytic infiltrate consisting of lipophages possessing foamy cytoplasm and giant cells. The presence of histiocytic giant cells differentiates AXG from xanthelasma, a clinical differential diagnosis in this case, and xanthoma. In AXG, there are 4 main types of histiocytes: xanthomatized, spindle shaped, vacuolated, and oncocytic.² They can be seen in variable proportions, together with different types of giant cells (eg, Touton, foreign body, ground glass, nonspecific). A mixed infiltrate of eosinophils, lymphocytes, plasma cells, and neutrophils also may be seen scattered throughout the lesion.²

Correlating with the clinical and histological features of xanthogranuloma, the firm plaques and nodules represent the dense dermal infiltration of histiocytes that may extend into the subcutis. The lesions demonstrate a time-dependent progression both clinically and histologically. Early lesions are comprised of a dense monomorphous nonlipid histiocytic inflammatory infiltrate, and they clinically appear more erythematous. In mature lesions, as in our patient, the infiltrate is predominantly composed of lipid-laden histiocytes with associated Touton giant cells. They appear more yellowish on clinical presentation.

Adult-onset xanthogranuloma is part of a rare heterogenous group of disorders termed adult orbital xanthogranulomatous disease, which includes 3 other syndromes: necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease.

Necrobiotic xanthogranuloma is clinically characterized by the presence of subcutaneous lesions that ulcerate in approximately 40% to 50% of cases and is histologically characterized by necrobiosis with palisading epithelioid histiocytes. It also is systemically associated with paraproteinemia and multiple myeloma.³

Adult-onset asthma and periocular xanthogranuloma is characterized by yellowish papules and nodules predominantly over the lower eyelids that are histologically comprised of lymphoid follicles with reactive germinal centers. It is associated with asthma, which normally is severe and often occurs almost simultaneously with the periorbital lesions.⁴

There are no definite diagnostic criteria for Erdheim-Chester disease and the diagnosis is usually based on radiologic findings of osteosclerosis and histopathologic evidence of foamy histiocytic infiltration. Systemic manifestations are common with lymphohistiocytic infiltration of the heart, lungs, pericardium, bones, and intestines. Prognosis is uniformly dismal.

Based on the clinical presentation of a nonulcerative papulonodular eruption and the absence of systemic involvement including asthma, we made the diagnosis of AXG. In view of the heterogeneity among these clinical entities as well as the time-based evolution of the lesions involved, we continued to monitor the patient for 2 years and there was no development of other systemic manifestations and hematologic abnormalities.

In contrast to the more common form of juvenile-onset xanthogranuloma, the adult type is not associated with widespread visceral lesions. Hence extensive screening investigations for systemic disease generally are not necessary. Another difference is that AXG has been associated with hematologic abnormalities, including essential thrombocytosis, chronic lymphocytic leukemia, large B-cell lymphoma, and monoclonal gammopathy.^5,6 In our patient, the presence of thrombocytopenia and older age caused us to be concerned about an associated hematologic malignancy; therefore, a bone marrow biopsy and flow cytometry were performed.

Adult-onset xanthogranuloma typically is an asymptomatic and self-healing disease and therefore treatment usually is not required. Spontaneous regression of xanthogranuloma was observed to occur in 54% of cases with a median duration of 22 months,⁷ though lesions were noted to last as long as 15 years.⁸ When treatment is necessary, a combination of local and systemic corticosteroids, cytotoxic agents, and radiotherapy have been routinely used. In particular, intralesional corticosteroid therapy has been found to be efficacious in controlling symptoms and signs of AXG affecting the eyelids and orbits while avoiding the systemic side effects of other agents.⁹

Because our patient’s lesions were longstanding and disfiguring, we opted for active intervention with intralesional triamcinolone, which resulted in only a slight reduction in size of the lesions. The lesions remain largely unchanged in 2 years of follow-up.

The Diagnosis: Disseminated Superficial Actinic Porokeratosis

Physical examination after 7 years of tanning salon use showed a tanned white man with multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the chest, back, abdomen, arms, and legs that were rough to palpate (Figure 1), with a peripheral rim of scale seen more prominently on dermoscopy. There were no lesions on the palms or soles. A subsequent 4-mm punch biopsy was done on the right abdomen and right thigh, which showed focal thinning of the epidermis, loss of the granular layer, a discrete column of parakeratosis and a characteristic feature of a coronoid lamella in the epidermis (Figure 2). The patient received 14 narrowband UVB (NB-UVB) treatments; however, he could not continue due to transportation issues. He visited the clinic sporadically for 6 months thereafter and reportedly went to tanning salons daily. The patient was subsequently lost to follow-up.

Figure 1. Multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the abdomen (A) and leg (B) that were rough to palpate.

Disseminated porokeratosis, or disseminated superficial actinic porokeratosis (DSAP), was first described in 1967 by Chernosky and Freeman.¹ It is the most common variant of porokeratosis. Other variants include Mibelli type, porokeratosis palmaris et plantaris disseminata, punctuate porokeratosis, and linear porokeratosis.^2-4 Porokeratosis is inherited in an autosomal-dominant fashion, presenting in the third or fourth decades of life; however, most cases are sporadic.⁵ Pruritus is a common symptom and can be debilitating.⁵

Disseminated superficial actinic porokeratosis can be precipitated by excessive sun exposure, with a reported increase in lesions during summer months and resolution during the winter months.⁶ The lesions of DSAP can be experimentally induced by exposure to daily use of artificial UV sunlamps.⁷ Patients with psoriasis undergoing psoralen plus UVA and NB-UVB treatments also have been reported to trigger DSAP.⁸ A study by Neumann et al⁶ suggested that a combination of both UVB and UVA wavelengths may be most effective in inducing DSAP. Exposure to UVA and UVB light may explain an increased number of DSAP lesions in patients who excessively visit tanning salons, as the bulbs emit a combination of wavelengths with UVA in much greater amounts than UVB.

Our patient developed DSAP secondary to artificial UV light exposure from excessive tanning salon use. Medications (allopurinol and lisinopril) were initially thought to be etiologic agents for the eruption and also corroborated with histologic findings of a drug eruption on the initial biopsy. However, new lesions continued to develop even after cessation of medications and NB-UVB treatments. A subsequent biopsy and further history of daily tanning salon use confirmed the diagnosis of DSAP.

Figure 2. Biopsy revealed a discrete column of parakeratosis, a characteristic feature of a coronoid lamella in the epidermis, and moderate papillary dermis lymphocytic infiltrate (H&E, original magnification ×10).

Therapies for this condition are limited with variable degrees of success. Cryotherapy, 5-fluorouracil cream, imiquimod cream 5%, Q-switched ruby laser, diclofenac gel 3%, and acitretin for more widespread or refractory lesions have been used with partial to complete resolution of DSAP.⁹

We present this case to highlight the occurrence of DSAP secondary to UV light exposure from excessive tanning salon use.

The Diagnosis: Disseminated Superficial Actinic Porokeratosis

Physical examination after 7 years of tanning salon use showed a tanned white man with multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the chest, back, abdomen, arms, and legs that were rough to palpate (Figure 1), with a peripheral rim of scale seen more prominently on dermoscopy. There were no lesions on the palms or soles. A subsequent 4-mm punch biopsy was done on the right abdomen and right thigh, which showed focal thinning of the epidermis, loss of the granular layer, a discrete column of parakeratosis and a characteristic feature of a coronoid lamella in the epidermis (Figure 2). The patient received 14 narrowband UVB (NB-UVB) treatments; however, he could not continue due to transportation issues. He visited the clinic sporadically for 6 months thereafter and reportedly went to tanning salons daily. The patient was subsequently lost to follow-up.

Figure 1. Multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the abdomen (A) and leg (B) that were rough to palpate.

Disseminated porokeratosis, or disseminated superficial actinic porokeratosis (DSAP), was first described in 1967 by Chernosky and Freeman.¹ It is the most common variant of porokeratosis. Other variants include Mibelli type, porokeratosis palmaris et plantaris disseminata, punctuate porokeratosis, and linear porokeratosis.^2-4 Porokeratosis is inherited in an autosomal-dominant fashion, presenting in the third or fourth decades of life; however, most cases are sporadic.⁵ Pruritus is a common symptom and can be debilitating.⁵

Disseminated superficial actinic porokeratosis can be precipitated by excessive sun exposure, with a reported increase in lesions during summer months and resolution during the winter months.⁶ The lesions of DSAP can be experimentally induced by exposure to daily use of artificial UV sunlamps.⁷ Patients with psoriasis undergoing psoralen plus UVA and NB-UVB treatments also have been reported to trigger DSAP.⁸ A study by Neumann et al⁶ suggested that a combination of both UVB and UVA wavelengths may be most effective in inducing DSAP. Exposure to UVA and UVB light may explain an increased number of DSAP lesions in patients who excessively visit tanning salons, as the bulbs emit a combination of wavelengths with UVA in much greater amounts than UVB.

Our patient developed DSAP secondary to artificial UV light exposure from excessive tanning salon use. Medications (allopurinol and lisinopril) were initially thought to be etiologic agents for the eruption and also corroborated with histologic findings of a drug eruption on the initial biopsy. However, new lesions continued to develop even after cessation of medications and NB-UVB treatments. A subsequent biopsy and further history of daily tanning salon use confirmed the diagnosis of DSAP.

Figure 2. Biopsy revealed a discrete column of parakeratosis, a characteristic feature of a coronoid lamella in the epidermis, and moderate papillary dermis lymphocytic infiltrate (H&E, original magnification ×10).

Therapies for this condition are limited with variable degrees of success. Cryotherapy, 5-fluorouracil cream, imiquimod cream 5%, Q-switched ruby laser, diclofenac gel 3%, and acitretin for more widespread or refractory lesions have been used with partial to complete resolution of DSAP.⁹

We present this case to highlight the occurrence of DSAP secondary to UV light exposure from excessive tanning salon use.

The Diagnosis: Disseminated Superficial Actinic Porokeratosis

Physical examination after 7 years of tanning salon use showed a tanned white man with multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the chest, back, abdomen, arms, and legs that were rough to palpate (Figure 1), with a peripheral rim of scale seen more prominently on dermoscopy. There were no lesions on the palms or soles. A subsequent 4-mm punch biopsy was done on the right abdomen and right thigh, which showed focal thinning of the epidermis, loss of the granular layer, a discrete column of parakeratosis and a characteristic feature of a coronoid lamella in the epidermis (Figure 2). The patient received 14 narrowband UVB (NB-UVB) treatments; however, he could not continue due to transportation issues. He visited the clinic sporadically for 6 months thereafter and reportedly went to tanning salons daily. The patient was subsequently lost to follow-up.

Figure 1. Multiple 4- to 5-mm, discrete, round to oval, reddish brown papules on the abdomen (A) and leg (B) that were rough to palpate.

Disseminated porokeratosis, or disseminated superficial actinic porokeratosis (DSAP), was first described in 1967 by Chernosky and Freeman.¹ It is the most common variant of porokeratosis. Other variants include Mibelli type, porokeratosis palmaris et plantaris disseminata, punctuate porokeratosis, and linear porokeratosis.^2-4 Porokeratosis is inherited in an autosomal-dominant fashion, presenting in the third or fourth decades of life; however, most cases are sporadic.⁵ Pruritus is a common symptom and can be debilitating.⁵

Disseminated superficial actinic porokeratosis can be precipitated by excessive sun exposure, with a reported increase in lesions during summer months and resolution during the winter months.⁶ The lesions of DSAP can be experimentally induced by exposure to daily use of artificial UV sunlamps.⁷ Patients with psoriasis undergoing psoralen plus UVA and NB-UVB treatments also have been reported to trigger DSAP.⁸ A study by Neumann et al⁶ suggested that a combination of both UVB and UVA wavelengths may be most effective in inducing DSAP. Exposure to UVA and UVB light may explain an increased number of DSAP lesions in patients who excessively visit tanning salons, as the bulbs emit a combination of wavelengths with UVA in much greater amounts than UVB.

Our patient developed DSAP secondary to artificial UV light exposure from excessive tanning salon use. Medications (allopurinol and lisinopril) were initially thought to be etiologic agents for the eruption and also corroborated with histologic findings of a drug eruption on the initial biopsy. However, new lesions continued to develop even after cessation of medications and NB-UVB treatments. A subsequent biopsy and further history of daily tanning salon use confirmed the diagnosis of DSAP.

Figure 2. Biopsy revealed a discrete column of parakeratosis, a characteristic feature of a coronoid lamella in the epidermis, and moderate papillary dermis lymphocytic infiltrate (H&E, original magnification ×10).

Therapies for this condition are limited with variable degrees of success. Cryotherapy, 5-fluorouracil cream, imiquimod cream 5%, Q-switched ruby laser, diclofenac gel 3%, and acitretin for more widespread or refractory lesions have been used with partial to complete resolution of DSAP.⁹

We present this case to highlight the occurrence of DSAP secondary to UV light exposure from excessive tanning salon use.