Make the Diagnosis

Hypertrophic lichen planus (HLP), also known as LP verrucosus, is a form of lichen planus where lesions are lichenified and thicker than the typical flat, purple, polygonal lesions found in lichen planus. Lesions may have a covering of scale. HLP commonly affects middle aged men and women. Lesions are most commonly located bilaterally on the shins and ankles and can be painful or pruritic. The differential diagnosis for the condition includes lichen simplex chronicus, connective tissue disease, and other skin disorders that cause hyperkeratosis. This wide differential makes histopathological analysis a useful tool in confirming the diagnosis of HLP.

A definitive diagnosis can be made via skin biopsy. Histopathology reveals hyperkeratosis, acanthosis, and a band-like lymphocytic infiltrate in the dermis. An eosinophilic infiltrate may be present. Other common features include saw tooth rete ridges and Civatte bodies, which are apoptotic keratinocytes. The lymphocytic infiltrate may indicate an autoimmune etiology in which the body’s immune system erroneously attacks itself. However, the exact cause is not known and genetic and environmental factors may play a role.

The treatment of HLP includes symptomatic management and control of inflammation. Topical steroids can be prescribed to manage the inflammation and associated pruritus, and emollient creams and moisturizers are helpful in controlling the dryness. Oral steroids, immunosuppressant medications, or retinoids may be necessary in more severe cases. In addition, psoralen plus ultraviolet A (PUVA) light therapy has been found to be beneficial in some cases. Squamous cell carcinoma may arise in lesions.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Arnold DL, Krishnamurthy K. Lichen Planus. [Updated 2023 Jun 1]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK526126/

Jaime TJ et al. An Bras Dermatol. 2011 Jul-Aug;86(4 Suppl 1):S96-9.

Mirchandani S et al. Med Pharm Rep. 2020 Apr;93(2):210-2. .

Whittington CP et al. Arch Pathol Lab Med. 2023 Jun 19. doi: 10.5858/arpa.2022-0515-RA.

Hypertrophic lichen planus (HLP), also known as LP verrucosus, is a form of lichen planus where lesions are lichenified and thicker than the typical flat, purple, polygonal lesions found in lichen planus. Lesions may have a covering of scale. HLP commonly affects middle aged men and women. Lesions are most commonly located bilaterally on the shins and ankles and can be painful or pruritic. The differential diagnosis for the condition includes lichen simplex chronicus, connective tissue disease, and other skin disorders that cause hyperkeratosis. This wide differential makes histopathological analysis a useful tool in confirming the diagnosis of HLP.

A definitive diagnosis can be made via skin biopsy. Histopathology reveals hyperkeratosis, acanthosis, and a band-like lymphocytic infiltrate in the dermis. An eosinophilic infiltrate may be present. Other common features include saw tooth rete ridges and Civatte bodies, which are apoptotic keratinocytes. The lymphocytic infiltrate may indicate an autoimmune etiology in which the body’s immune system erroneously attacks itself. However, the exact cause is not known and genetic and environmental factors may play a role.

The treatment of HLP includes symptomatic management and control of inflammation. Topical steroids can be prescribed to manage the inflammation and associated pruritus, and emollient creams and moisturizers are helpful in controlling the dryness. Oral steroids, immunosuppressant medications, or retinoids may be necessary in more severe cases. In addition, psoralen plus ultraviolet A (PUVA) light therapy has been found to be beneficial in some cases. Squamous cell carcinoma may arise in lesions.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Arnold DL, Krishnamurthy K. Lichen Planus. [Updated 2023 Jun 1]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK526126/

Jaime TJ et al. An Bras Dermatol. 2011 Jul-Aug;86(4 Suppl 1):S96-9.

Mirchandani S et al. Med Pharm Rep. 2020 Apr;93(2):210-2. .

Whittington CP et al. Arch Pathol Lab Med. 2023 Jun 19. doi: 10.5858/arpa.2022-0515-RA.

Hypertrophic lichen planus (HLP), also known as LP verrucosus, is a form of lichen planus where lesions are lichenified and thicker than the typical flat, purple, polygonal lesions found in lichen planus. Lesions may have a covering of scale. HLP commonly affects middle aged men and women. Lesions are most commonly located bilaterally on the shins and ankles and can be painful or pruritic. The differential diagnosis for the condition includes lichen simplex chronicus, connective tissue disease, and other skin disorders that cause hyperkeratosis. This wide differential makes histopathological analysis a useful tool in confirming the diagnosis of HLP.

A definitive diagnosis can be made via skin biopsy. Histopathology reveals hyperkeratosis, acanthosis, and a band-like lymphocytic infiltrate in the dermis. An eosinophilic infiltrate may be present. Other common features include saw tooth rete ridges and Civatte bodies, which are apoptotic keratinocytes. The lymphocytic infiltrate may indicate an autoimmune etiology in which the body’s immune system erroneously attacks itself. However, the exact cause is not known and genetic and environmental factors may play a role.

The treatment of HLP includes symptomatic management and control of inflammation. Topical steroids can be prescribed to manage the inflammation and associated pruritus, and emollient creams and moisturizers are helpful in controlling the dryness. Oral steroids, immunosuppressant medications, or retinoids may be necessary in more severe cases. In addition, psoralen plus ultraviolet A (PUVA) light therapy has been found to be beneficial in some cases. Squamous cell carcinoma may arise in lesions.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Arnold DL, Krishnamurthy K. Lichen Planus. [Updated 2023 Jun 1]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK526126/

Jaime TJ et al. An Bras Dermatol. 2011 Jul-Aug;86(4 Suppl 1):S96-9.

Mirchandani S et al. Med Pharm Rep. 2020 Apr;93(2):210-2. .

Whittington CP et al. Arch Pathol Lab Med. 2023 Jun 19. doi: 10.5858/arpa.2022-0515-RA.

Angiosarcomas are uncommon, high-grade malignant tumors of endothelial cell origin that can arise via the lymphatics or vasculature. They typically occur spontaneously; however, there have been cases reported of benign vascular transformation. These tumors are more commonly found in elderly men on the head and neck in sun-damaged skin. Radiation-induced angiosarcoma occurs in an estimated 0.05%-0.3% of patients with breast cancer who underwent surgery and radiation therapy. This is a late complication, typically occurring about 5-10 years after radiation. Stewart-Treves syndrome, chronic lymphedema occurring after breast cancer treatment with axillary node dissection, increases the risk of angiosarcoma. As a vascular tumor, angiosarcoma spreads hematogenously and carries a poor prognosis if not caught early. Differential diagnoses include other vascular tumors such as retiform hemangioendothelioma. In this specific patient, the differential diagnosis includes Paget’s disease, chronic radiation skin changes, and eczema.

Histopathologically, angiosarcomas exhibit abnormal, pleomorphic, malignant endothelial cells. As the tumor progresses, the cell architecture becomes more distorted and cells form layers with papillary projections into the vascular lumen. Malignant cells may stain positive for CD31, CD34, the oncogene ERG and the proto-oncogene FLI-1. Histology in this patient revealed radiation changes in the dermis, as well as few vascular channels lined by large endothelial cells with marked nuclear atypia, in the form of large nucleoli and variably coarse chromatin. The cells were positive for MYC.

Treatment of angiosarcoma involves a multidisciplinary approach. Resection with wide margins is generally the treatment of choice. However, recurrence is relatively common, which may be a result of microsatellite deposits of the tumor. Perioperative radiation is recommended, and adjuvant chemotherapy often is recommended for metastatic disease. Specifically, paclitaxel has been found to promote survival in some cases of cutaneous angiosarcoma. Metastatic disease may be treated with cytotoxic drugs such as anthracyclines and taxanes. Additionally, targeted therapy including anti-VEGF drugs and tyrosine kinase inhibitors have been tested.

The case and photo were submitted by Mr. Shapiro of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Dr. Bilu Martin. The column was edited by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Cohen-Hallaleh RB et al. Clin Sarcoma Res. 2017 Aug 7:7:15.

Cozzi S et al. Rep Pract Oncol Radiother. 2021 Sep 30;26(5):827-32.

Spiker AM, Mangla A, Ramsey ML. Angiosarcoma. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island, Fla.: StatPearls Publishing; 2023 Jan-. Available from: www.ncbi.nlm.nih.gov/books/NBK441983/

Angiosarcomas are uncommon, high-grade malignant tumors of endothelial cell origin that can arise via the lymphatics or vasculature. They typically occur spontaneously; however, there have been cases reported of benign vascular transformation. These tumors are more commonly found in elderly men on the head and neck in sun-damaged skin. Radiation-induced angiosarcoma occurs in an estimated 0.05%-0.3% of patients with breast cancer who underwent surgery and radiation therapy. This is a late complication, typically occurring about 5-10 years after radiation. Stewart-Treves syndrome, chronic lymphedema occurring after breast cancer treatment with axillary node dissection, increases the risk of angiosarcoma. As a vascular tumor, angiosarcoma spreads hematogenously and carries a poor prognosis if not caught early. Differential diagnoses include other vascular tumors such as retiform hemangioendothelioma. In this specific patient, the differential diagnosis includes Paget’s disease, chronic radiation skin changes, and eczema.

Histopathologically, angiosarcomas exhibit abnormal, pleomorphic, malignant endothelial cells. As the tumor progresses, the cell architecture becomes more distorted and cells form layers with papillary projections into the vascular lumen. Malignant cells may stain positive for CD31, CD34, the oncogene ERG and the proto-oncogene FLI-1. Histology in this patient revealed radiation changes in the dermis, as well as few vascular channels lined by large endothelial cells with marked nuclear atypia, in the form of large nucleoli and variably coarse chromatin. The cells were positive for MYC.

Treatment of angiosarcoma involves a multidisciplinary approach. Resection with wide margins is generally the treatment of choice. However, recurrence is relatively common, which may be a result of microsatellite deposits of the tumor. Perioperative radiation is recommended, and adjuvant chemotherapy often is recommended for metastatic disease. Specifically, paclitaxel has been found to promote survival in some cases of cutaneous angiosarcoma. Metastatic disease may be treated with cytotoxic drugs such as anthracyclines and taxanes. Additionally, targeted therapy including anti-VEGF drugs and tyrosine kinase inhibitors have been tested.

The case and photo were submitted by Mr. Shapiro of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Dr. Bilu Martin. The column was edited by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Cohen-Hallaleh RB et al. Clin Sarcoma Res. 2017 Aug 7:7:15.

Cozzi S et al. Rep Pract Oncol Radiother. 2021 Sep 30;26(5):827-32.

Spiker AM, Mangla A, Ramsey ML. Angiosarcoma. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island, Fla.: StatPearls Publishing; 2023 Jan-. Available from: www.ncbi.nlm.nih.gov/books/NBK441983/

Angiosarcomas are uncommon, high-grade malignant tumors of endothelial cell origin that can arise via the lymphatics or vasculature. They typically occur spontaneously; however, there have been cases reported of benign vascular transformation. These tumors are more commonly found in elderly men on the head and neck in sun-damaged skin. Radiation-induced angiosarcoma occurs in an estimated 0.05%-0.3% of patients with breast cancer who underwent surgery and radiation therapy. This is a late complication, typically occurring about 5-10 years after radiation. Stewart-Treves syndrome, chronic lymphedema occurring after breast cancer treatment with axillary node dissection, increases the risk of angiosarcoma. As a vascular tumor, angiosarcoma spreads hematogenously and carries a poor prognosis if not caught early. Differential diagnoses include other vascular tumors such as retiform hemangioendothelioma. In this specific patient, the differential diagnosis includes Paget’s disease, chronic radiation skin changes, and eczema.

Histopathologically, angiosarcomas exhibit abnormal, pleomorphic, malignant endothelial cells. As the tumor progresses, the cell architecture becomes more distorted and cells form layers with papillary projections into the vascular lumen. Malignant cells may stain positive for CD31, CD34, the oncogene ERG and the proto-oncogene FLI-1. Histology in this patient revealed radiation changes in the dermis, as well as few vascular channels lined by large endothelial cells with marked nuclear atypia, in the form of large nucleoli and variably coarse chromatin. The cells were positive for MYC.

Treatment of angiosarcoma involves a multidisciplinary approach. Resection with wide margins is generally the treatment of choice. However, recurrence is relatively common, which may be a result of microsatellite deposits of the tumor. Perioperative radiation is recommended, and adjuvant chemotherapy often is recommended for metastatic disease. Specifically, paclitaxel has been found to promote survival in some cases of cutaneous angiosarcoma. Metastatic disease may be treated with cytotoxic drugs such as anthracyclines and taxanes. Additionally, targeted therapy including anti-VEGF drugs and tyrosine kinase inhibitors have been tested.

The case and photo were submitted by Mr. Shapiro of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Dr. Bilu Martin. The column was edited by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Cohen-Hallaleh RB et al. Clin Sarcoma Res. 2017 Aug 7:7:15.

Cozzi S et al. Rep Pract Oncol Radiother. 2021 Sep 30;26(5):827-32.

Spiker AM, Mangla A, Ramsey ML. Angiosarcoma. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island, Fla.: StatPearls Publishing; 2023 Jan-. Available from: www.ncbi.nlm.nih.gov/books/NBK441983/

A fixed drug eruption (FDE) is a rare cutaneous and/or mucosal reaction caused by ingestion of a drug. This is a delayed hypersensitivity reaction in which lesions present in the same location upon repeated intake of the offending drug. The lesions typically present within 30 minutes to 8 hours of administration of the drug. These reactions can be considered allergic or pseudo-allergic, in which case, there is no notable adaptive immune response. CD8+ T cells appear to play a role in the epidermal injury via release of interferons and interactions with other inflammatory cells.

Courtesy Lucas Shapiro and Dr. Igor Chaplik

There are numerous drugs that can precipitate these findings. NSAIDs; antibiotics, such as tetracyclines, sulfonamides; and phenytoin are common offenders. In the case of our patient, naproxen was the offending medication.

The classic presentation of FDE features annular, erythematous to violaceous macules on the skin or mucosa that can be asymptomatic or can produce burning, pain, or pruritus. The most common locations include the trunk and extremities, but the palms, soles, face, scalp, and mucosa can also be impacted. The oral mucosa seems to be the most common mucosal location. Intravenous administration of a drug is associated with more severe symptoms. Systemic symptoms are typically absent, and the eruption may initially be in one location, but may appear elsewhere upon repeated exposure to the offending medication.

The differential diagnosis includes arthropod bite reactions, urticaria, and erythema multiforme. Although FDEs are typically a clinical diagnosis, the histopathology will commonly show a vacuolar interface dermatitis. Furthermore, a variety of immune cells can be found, including neutrophilic, eosinophilic, and lymphocytic infiltrate. A combination of two or more histological patterns often favors the diagnosis of FDE.

Steroid creams can be prescribed to decrease the inflammatory reaction and improve symptoms; however, the definitive treatment of this condition is cessation of the offending agent. Postinflammatory hyperpigmentation is a common symptom after resolution of the condition, and it may take months to fade away. Further darkening can be prevented by practicing sun safety measures such as wearing sunblock, covering the affected areas, and avoiding prolonged sun exposure.

This case and the photos were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Igor Chaplik, DO, Aesthetix Dermatology, Fort Lauderdale. The column was edited by Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Shaker G et al. Cureus. 2022 Aug 23;14(8):e28299.

Srivastava R et al. Indian J Dent. 2015 Apr-Jun;6(2):103-6.

Weyers W, Metze D. Dermatol Pract Concept. 2011 Jan 31;1(1):33-47.

A fixed drug eruption (FDE) is a rare cutaneous and/or mucosal reaction caused by ingestion of a drug. This is a delayed hypersensitivity reaction in which lesions present in the same location upon repeated intake of the offending drug. The lesions typically present within 30 minutes to 8 hours of administration of the drug. These reactions can be considered allergic or pseudo-allergic, in which case, there is no notable adaptive immune response. CD8+ T cells appear to play a role in the epidermal injury via release of interferons and interactions with other inflammatory cells.

Courtesy Lucas Shapiro and Dr. Igor Chaplik

There are numerous drugs that can precipitate these findings. NSAIDs; antibiotics, such as tetracyclines, sulfonamides; and phenytoin are common offenders. In the case of our patient, naproxen was the offending medication.

The classic presentation of FDE features annular, erythematous to violaceous macules on the skin or mucosa that can be asymptomatic or can produce burning, pain, or pruritus. The most common locations include the trunk and extremities, but the palms, soles, face, scalp, and mucosa can also be impacted. The oral mucosa seems to be the most common mucosal location. Intravenous administration of a drug is associated with more severe symptoms. Systemic symptoms are typically absent, and the eruption may initially be in one location, but may appear elsewhere upon repeated exposure to the offending medication.

The differential diagnosis includes arthropod bite reactions, urticaria, and erythema multiforme. Although FDEs are typically a clinical diagnosis, the histopathology will commonly show a vacuolar interface dermatitis. Furthermore, a variety of immune cells can be found, including neutrophilic, eosinophilic, and lymphocytic infiltrate. A combination of two or more histological patterns often favors the diagnosis of FDE.

Steroid creams can be prescribed to decrease the inflammatory reaction and improve symptoms; however, the definitive treatment of this condition is cessation of the offending agent. Postinflammatory hyperpigmentation is a common symptom after resolution of the condition, and it may take months to fade away. Further darkening can be prevented by practicing sun safety measures such as wearing sunblock, covering the affected areas, and avoiding prolonged sun exposure.

This case and the photos were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Igor Chaplik, DO, Aesthetix Dermatology, Fort Lauderdale. The column was edited by Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Shaker G et al. Cureus. 2022 Aug 23;14(8):e28299.

Srivastava R et al. Indian J Dent. 2015 Apr-Jun;6(2):103-6.

Weyers W, Metze D. Dermatol Pract Concept. 2011 Jan 31;1(1):33-47.

A fixed drug eruption (FDE) is a rare cutaneous and/or mucosal reaction caused by ingestion of a drug. This is a delayed hypersensitivity reaction in which lesions present in the same location upon repeated intake of the offending drug. The lesions typically present within 30 minutes to 8 hours of administration of the drug. These reactions can be considered allergic or pseudo-allergic, in which case, there is no notable adaptive immune response. CD8+ T cells appear to play a role in the epidermal injury via release of interferons and interactions with other inflammatory cells.

Courtesy Lucas Shapiro and Dr. Igor Chaplik

There are numerous drugs that can precipitate these findings. NSAIDs; antibiotics, such as tetracyclines, sulfonamides; and phenytoin are common offenders. In the case of our patient, naproxen was the offending medication.

The classic presentation of FDE features annular, erythematous to violaceous macules on the skin or mucosa that can be asymptomatic or can produce burning, pain, or pruritus. The most common locations include the trunk and extremities, but the palms, soles, face, scalp, and mucosa can also be impacted. The oral mucosa seems to be the most common mucosal location. Intravenous administration of a drug is associated with more severe symptoms. Systemic symptoms are typically absent, and the eruption may initially be in one location, but may appear elsewhere upon repeated exposure to the offending medication.

The differential diagnosis includes arthropod bite reactions, urticaria, and erythema multiforme. Although FDEs are typically a clinical diagnosis, the histopathology will commonly show a vacuolar interface dermatitis. Furthermore, a variety of immune cells can be found, including neutrophilic, eosinophilic, and lymphocytic infiltrate. A combination of two or more histological patterns often favors the diagnosis of FDE.

Steroid creams can be prescribed to decrease the inflammatory reaction and improve symptoms; however, the definitive treatment of this condition is cessation of the offending agent. Postinflammatory hyperpigmentation is a common symptom after resolution of the condition, and it may take months to fade away. Further darkening can be prevented by practicing sun safety measures such as wearing sunblock, covering the affected areas, and avoiding prolonged sun exposure.

This case and the photos were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Igor Chaplik, DO, Aesthetix Dermatology, Fort Lauderdale. The column was edited by Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Shaker G et al. Cureus. 2022 Aug 23;14(8):e28299.

Srivastava R et al. Indian J Dent. 2015 Apr-Jun;6(2):103-6.

Weyers W, Metze D. Dermatol Pract Concept. 2011 Jan 31;1(1):33-47.

Erythema chronicum migrans (ECM) is the classical dermatologic manifestation of Lyme disease, a condition caused by Borrelia burgdorferi, a bacterial spirochete. Lyme disease is the most commonly transmitted tick-borne illness in the United States. This infection is typically transmitted through a bite by the Ixodes tick commonly found in the Midwest, Northeast, and mid-Atlantic regions; however, the geographical distribution continues to expand over time in the United States. Ticks must be attached for 24-48 hours to transmit the pathogen. There are three general stages of the disease: early localized, early disseminated, and late disseminated.

The most common presentation is the early localized disease, which manifests between 3 and 30 days after an infected tick bite. Approximately 70%-80% of cases feature a targetlike lesion that expands centrifugally at the site of the bite. Most commonly, lesions appear on the abdomen, groin, axilla, and popliteal fossa. The diagnosis of ECM requires lesions at least 5 cm in size. Lesions may be asymptomatic, although burning may occur in half of patients. Atypical presentations include bullous, vesicular, hemorrhagic, or necrotic lesions. Up to half of patients may develop multiple ECM lesions. Palms and soles are spared. Differential diagnoses include arthropod reactions, pyoderma gangrenosum, cellulitis, herpes simplex virus and varicella zoster virus, contact dermatitis, or granuloma annulare. The rash is often accompanied by systemic symptoms including fatigue, myalgia, headache, and fever.

The next two stages include early and late disseminated infection. Early disseminated infection often occurs 3-12 weeks after infection and is characterized by muscle pain, dizziness, headache, and cardiac symptoms. CNS involvement occurs in about 20% of patients. Joint involvement may include the knee, ankle, and wrist. If symptoms are only in one joint, septic arthritis is part of the differential diagnosis, so clinical correlation and labs must be considered. Late disseminated infection occurs months or years after initial infection and includes neurologic and rheumatologic symptoms including meningitis, Bell’s palsy, arthritis, and dysesthesia. Knee arthritis is a key feature of this stage. Patients commonly have radicular pain and fibromyalgia-type pain. More severe disease processes include encephalomyelitis, arrhythmias, and heart block.

ECM is often a clinical diagnosis because serologic testing may not be positive during the first 2 weeks of infection. The screening serologic test is the ELISA, and a Western blot confirms the results. Skin histopathology for Lyme disease is often nonspecific and reveals a perivascular infiltrate of histiocytes, plasma cells, and lymphocytes. Silver stain or antibody testing may be used to identify the spirochete. In acrodermatitis chronica atrophicans, late Lyme disease presenting on the distal extremities, lymphocytic and plasma cell infiltrates are present. In borrelial lymphocytoma, a dense dermal lymphocytic infiltrate is present.

The standard for treatment of early localized disease is oral doxycycline in adults. Alternatives may be used if a patient is allergic or for children under 9. Disseminated disease may be treated with IV ceftriaxone and topical steroids are used if ocular symptoms are involved. Early treatment is often curative.

This patient’s antibodies were negative initially, but became positive after 6 weeks. He was treated empirically at the time of his office visit with doxycycline for 1 month.

This case and the photo were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Susannah Berke, MD, Three Rivers Dermatology, Coraopolis, Pa. The column was edited by Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at MDedge.com/Dermatology. To submit a case for possible publication, send an email to [email protected].

References
 

Carriveau A et al. Nurs Clin North Am. 2019 Jun;54(2):261-75.

Skar GL and Simonsen KA. Lyme Disease. [Updated 2023 May 31]. In: “StatPearls” [Internet]. Treasure Island, Fla.: StatPearls Publishing; 2023 Jan.

Tiger JB et al. J Am Acad Dermatol. 2014 Oct;71(4):e133-4.

Erythema chronicum migrans (ECM) is the classical dermatologic manifestation of Lyme disease, a condition caused by Borrelia burgdorferi, a bacterial spirochete. Lyme disease is the most commonly transmitted tick-borne illness in the United States. This infection is typically transmitted through a bite by the Ixodes tick commonly found in the Midwest, Northeast, and mid-Atlantic regions; however, the geographical distribution continues to expand over time in the United States. Ticks must be attached for 24-48 hours to transmit the pathogen. There are three general stages of the disease: early localized, early disseminated, and late disseminated.

The most common presentation is the early localized disease, which manifests between 3 and 30 days after an infected tick bite. Approximately 70%-80% of cases feature a targetlike lesion that expands centrifugally at the site of the bite. Most commonly, lesions appear on the abdomen, groin, axilla, and popliteal fossa. The diagnosis of ECM requires lesions at least 5 cm in size. Lesions may be asymptomatic, although burning may occur in half of patients. Atypical presentations include bullous, vesicular, hemorrhagic, or necrotic lesions. Up to half of patients may develop multiple ECM lesions. Palms and soles are spared. Differential diagnoses include arthropod reactions, pyoderma gangrenosum, cellulitis, herpes simplex virus and varicella zoster virus, contact dermatitis, or granuloma annulare. The rash is often accompanied by systemic symptoms including fatigue, myalgia, headache, and fever.

The next two stages include early and late disseminated infection. Early disseminated infection often occurs 3-12 weeks after infection and is characterized by muscle pain, dizziness, headache, and cardiac symptoms. CNS involvement occurs in about 20% of patients. Joint involvement may include the knee, ankle, and wrist. If symptoms are only in one joint, septic arthritis is part of the differential diagnosis, so clinical correlation and labs must be considered. Late disseminated infection occurs months or years after initial infection and includes neurologic and rheumatologic symptoms including meningitis, Bell’s palsy, arthritis, and dysesthesia. Knee arthritis is a key feature of this stage. Patients commonly have radicular pain and fibromyalgia-type pain. More severe disease processes include encephalomyelitis, arrhythmias, and heart block.

ECM is often a clinical diagnosis because serologic testing may not be positive during the first 2 weeks of infection. The screening serologic test is the ELISA, and a Western blot confirms the results. Skin histopathology for Lyme disease is often nonspecific and reveals a perivascular infiltrate of histiocytes, plasma cells, and lymphocytes. Silver stain or antibody testing may be used to identify the spirochete. In acrodermatitis chronica atrophicans, late Lyme disease presenting on the distal extremities, lymphocytic and plasma cell infiltrates are present. In borrelial lymphocytoma, a dense dermal lymphocytic infiltrate is present.

The standard for treatment of early localized disease is oral doxycycline in adults. Alternatives may be used if a patient is allergic or for children under 9. Disseminated disease may be treated with IV ceftriaxone and topical steroids are used if ocular symptoms are involved. Early treatment is often curative.

This patient’s antibodies were negative initially, but became positive after 6 weeks. He was treated empirically at the time of his office visit with doxycycline for 1 month.

This case and the photo were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Susannah Berke, MD, Three Rivers Dermatology, Coraopolis, Pa. The column was edited by Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at MDedge.com/Dermatology. To submit a case for possible publication, send an email to [email protected].

References
 

Carriveau A et al. Nurs Clin North Am. 2019 Jun;54(2):261-75.

Skar GL and Simonsen KA. Lyme Disease. [Updated 2023 May 31]. In: “StatPearls” [Internet]. Treasure Island, Fla.: StatPearls Publishing; 2023 Jan.

Tiger JB et al. J Am Acad Dermatol. 2014 Oct;71(4):e133-4.

Erythema chronicum migrans (ECM) is the classical dermatologic manifestation of Lyme disease, a condition caused by Borrelia burgdorferi, a bacterial spirochete. Lyme disease is the most commonly transmitted tick-borne illness in the United States. This infection is typically transmitted through a bite by the Ixodes tick commonly found in the Midwest, Northeast, and mid-Atlantic regions; however, the geographical distribution continues to expand over time in the United States. Ticks must be attached for 24-48 hours to transmit the pathogen. There are three general stages of the disease: early localized, early disseminated, and late disseminated.

The most common presentation is the early localized disease, which manifests between 3 and 30 days after an infected tick bite. Approximately 70%-80% of cases feature a targetlike lesion that expands centrifugally at the site of the bite. Most commonly, lesions appear on the abdomen, groin, axilla, and popliteal fossa. The diagnosis of ECM requires lesions at least 5 cm in size. Lesions may be asymptomatic, although burning may occur in half of patients. Atypical presentations include bullous, vesicular, hemorrhagic, or necrotic lesions. Up to half of patients may develop multiple ECM lesions. Palms and soles are spared. Differential diagnoses include arthropod reactions, pyoderma gangrenosum, cellulitis, herpes simplex virus and varicella zoster virus, contact dermatitis, or granuloma annulare. The rash is often accompanied by systemic symptoms including fatigue, myalgia, headache, and fever.

The next two stages include early and late disseminated infection. Early disseminated infection often occurs 3-12 weeks after infection and is characterized by muscle pain, dizziness, headache, and cardiac symptoms. CNS involvement occurs in about 20% of patients. Joint involvement may include the knee, ankle, and wrist. If symptoms are only in one joint, septic arthritis is part of the differential diagnosis, so clinical correlation and labs must be considered. Late disseminated infection occurs months or years after initial infection and includes neurologic and rheumatologic symptoms including meningitis, Bell’s palsy, arthritis, and dysesthesia. Knee arthritis is a key feature of this stage. Patients commonly have radicular pain and fibromyalgia-type pain. More severe disease processes include encephalomyelitis, arrhythmias, and heart block.

ECM is often a clinical diagnosis because serologic testing may not be positive during the first 2 weeks of infection. The screening serologic test is the ELISA, and a Western blot confirms the results. Skin histopathology for Lyme disease is often nonspecific and reveals a perivascular infiltrate of histiocytes, plasma cells, and lymphocytes. Silver stain or antibody testing may be used to identify the spirochete. In acrodermatitis chronica atrophicans, late Lyme disease presenting on the distal extremities, lymphocytic and plasma cell infiltrates are present. In borrelial lymphocytoma, a dense dermal lymphocytic infiltrate is present.

The standard for treatment of early localized disease is oral doxycycline in adults. Alternatives may be used if a patient is allergic or for children under 9. Disseminated disease may be treated with IV ceftriaxone and topical steroids are used if ocular symptoms are involved. Early treatment is often curative.

This patient’s antibodies were negative initially, but became positive after 6 weeks. He was treated empirically at the time of his office visit with doxycycline for 1 month.

This case and the photo were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Susannah Berke, MD, Three Rivers Dermatology, Coraopolis, Pa. The column was edited by Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at MDedge.com/Dermatology. To submit a case for possible publication, send an email to [email protected].

References
 

Carriveau A et al. Nurs Clin North Am. 2019 Jun;54(2):261-75.

Skar GL and Simonsen KA. Lyme Disease. [Updated 2023 May 31]. In: “StatPearls” [Internet]. Treasure Island, Fla.: StatPearls Publishing; 2023 Jan.

Tiger JB et al. J Am Acad Dermatol. 2014 Oct;71(4):e133-4.

Dermatomyositis is a rare autoimmune condition characterized by muscle inflammation and weakness, and skin findings. The classical presentation includes symmetric proximal muscle weakness and underlying malignancy and is very common in adult patients. The etiology is unknown, however.

Courtesy Lucas Shapiro and Dr. Natalie Y. Nasser

Some studies suggest people with certain HLA subtypes are at higher risk, and various infectious and pharmacological triggers are suspected to play a role in the pathogenesis of dermatomyositis. Infectious causes include Coxsackie B, enterovirus, and parvovirus. Drugs such as antineoplastic agents, antibiotics, and NSAIDs have been found to be triggers.

The pathogenesis of dermatomyositis involves immune-mediated damage to muscle capillaries and the endothelium of arterioles. In the typical humoral immune response, complement activation occurs. One mechanism of damage in dermatomyositis occurs when the membrane attack complex formed at the end of the complement process deposits in blood vessels, causing inflammation. B cells, autoantibodies, and interferon overexpression may also play a role in damaging the vasculature and muscle fibers. Hypoxia leads to muscular atrophy, resulting in degeneration and death of the fibers. On muscle biopsy, a perivascular and perimysial inflammatory infiltrate, perifascicular atrophy, and microangiopathy may be present. Skin histology reveals vacuolar changes in the basal layer, a lymphocytic infiltrate, and increased mucin production in the dermis.

On clinical examination, patients will have proximal muscle weakness and a skin rash that may include Gottron’s papules, heliotrope erythema, V-sign, shawl sign, holster sign, scalp erythema, midfacial erythema, and photosensitivity. Scalp erythema in dermatomyositis is highly linked to pruritus, alopecia, and telogen effluvium. Patients may experience small fiber neuropathy in dermatomyositis.

Serologies for this patient, who had previously been diagnosed and treated for dermatomyositis, were significant for a positive ANA 1:2560. Anti-Jo-1 antibody was negative. Her liver function tests, aldolase, creatinine kinase, sedimentation rate, C-reactive protein, and serum protein electrophoresis were normal. Imaging revealed mild chronic interstitial lung disease. A malignancy workup was negative.

Treatment of dermatomyositis involves lifestyle changes and pharmacologic therapy. Because of the intense photosensitivity, patients should be diligent with their sun protection. Methotrexate, azathioprine, and mycophenolate mofetil are considered first-line therapies for dermatomyositis. Therapies such as cyclophosphamide, rituximab, IVIg, and plasmapheresis may also be indicated in severe or refractory cases. Additionally, patients with pulmonary involvement should be given systemic steroids. The side effects of these drugs must be considered in the context of the patient’s demographics, comorbidities and lifestyle.

This case and the photos were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Natalie Y. Nasser, MD, of Kaiser Permanente Riverside Medical Center, Riverside, Calif. The column was edited by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Qudsiya Z and Waseem M. Dermatomyositis, in “StatPearls.” Treasure Island, Fla.: StatPearls Publishing, 2023 Jan.

2. Kamperman RG et al. Int J Mol Sci. 2022 Apr 13;23(8):4301.

3. Kassamali B et al. Int J WomensDermatol. 2021 Sep 24;7(5Part A):576-82.

4. Vázquez-Herrera NE et al. Skin Appendage Disord. 2018 Aug;4(3):187-99.

Dermatomyositis is a rare autoimmune condition characterized by muscle inflammation and weakness, and skin findings. The classical presentation includes symmetric proximal muscle weakness and underlying malignancy and is very common in adult patients. The etiology is unknown, however.

Courtesy Lucas Shapiro and Dr. Natalie Y. Nasser

Some studies suggest people with certain HLA subtypes are at higher risk, and various infectious and pharmacological triggers are suspected to play a role in the pathogenesis of dermatomyositis. Infectious causes include Coxsackie B, enterovirus, and parvovirus. Drugs such as antineoplastic agents, antibiotics, and NSAIDs have been found to be triggers.

The pathogenesis of dermatomyositis involves immune-mediated damage to muscle capillaries and the endothelium of arterioles. In the typical humoral immune response, complement activation occurs. One mechanism of damage in dermatomyositis occurs when the membrane attack complex formed at the end of the complement process deposits in blood vessels, causing inflammation. B cells, autoantibodies, and interferon overexpression may also play a role in damaging the vasculature and muscle fibers. Hypoxia leads to muscular atrophy, resulting in degeneration and death of the fibers. On muscle biopsy, a perivascular and perimysial inflammatory infiltrate, perifascicular atrophy, and microangiopathy may be present. Skin histology reveals vacuolar changes in the basal layer, a lymphocytic infiltrate, and increased mucin production in the dermis.

On clinical examination, patients will have proximal muscle weakness and a skin rash that may include Gottron’s papules, heliotrope erythema, V-sign, shawl sign, holster sign, scalp erythema, midfacial erythema, and photosensitivity. Scalp erythema in dermatomyositis is highly linked to pruritus, alopecia, and telogen effluvium. Patients may experience small fiber neuropathy in dermatomyositis.

Serologies for this patient, who had previously been diagnosed and treated for dermatomyositis, were significant for a positive ANA 1:2560. Anti-Jo-1 antibody was negative. Her liver function tests, aldolase, creatinine kinase, sedimentation rate, C-reactive protein, and serum protein electrophoresis were normal. Imaging revealed mild chronic interstitial lung disease. A malignancy workup was negative.

Treatment of dermatomyositis involves lifestyle changes and pharmacologic therapy. Because of the intense photosensitivity, patients should be diligent with their sun protection. Methotrexate, azathioprine, and mycophenolate mofetil are considered first-line therapies for dermatomyositis. Therapies such as cyclophosphamide, rituximab, IVIg, and plasmapheresis may also be indicated in severe or refractory cases. Additionally, patients with pulmonary involvement should be given systemic steroids. The side effects of these drugs must be considered in the context of the patient’s demographics, comorbidities and lifestyle.

This case and the photos were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Natalie Y. Nasser, MD, of Kaiser Permanente Riverside Medical Center, Riverside, Calif. The column was edited by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Qudsiya Z and Waseem M. Dermatomyositis, in “StatPearls.” Treasure Island, Fla.: StatPearls Publishing, 2023 Jan.

2. Kamperman RG et al. Int J Mol Sci. 2022 Apr 13;23(8):4301.

3. Kassamali B et al. Int J WomensDermatol. 2021 Sep 24;7(5Part A):576-82.

4. Vázquez-Herrera NE et al. Skin Appendage Disord. 2018 Aug;4(3):187-99.

Dermatomyositis is a rare autoimmune condition characterized by muscle inflammation and weakness, and skin findings. The classical presentation includes symmetric proximal muscle weakness and underlying malignancy and is very common in adult patients. The etiology is unknown, however.

Courtesy Lucas Shapiro and Dr. Natalie Y. Nasser

Some studies suggest people with certain HLA subtypes are at higher risk, and various infectious and pharmacological triggers are suspected to play a role in the pathogenesis of dermatomyositis. Infectious causes include Coxsackie B, enterovirus, and parvovirus. Drugs such as antineoplastic agents, antibiotics, and NSAIDs have been found to be triggers.

The pathogenesis of dermatomyositis involves immune-mediated damage to muscle capillaries and the endothelium of arterioles. In the typical humoral immune response, complement activation occurs. One mechanism of damage in dermatomyositis occurs when the membrane attack complex formed at the end of the complement process deposits in blood vessels, causing inflammation. B cells, autoantibodies, and interferon overexpression may also play a role in damaging the vasculature and muscle fibers. Hypoxia leads to muscular atrophy, resulting in degeneration and death of the fibers. On muscle biopsy, a perivascular and perimysial inflammatory infiltrate, perifascicular atrophy, and microangiopathy may be present. Skin histology reveals vacuolar changes in the basal layer, a lymphocytic infiltrate, and increased mucin production in the dermis.

On clinical examination, patients will have proximal muscle weakness and a skin rash that may include Gottron’s papules, heliotrope erythema, V-sign, shawl sign, holster sign, scalp erythema, midfacial erythema, and photosensitivity. Scalp erythema in dermatomyositis is highly linked to pruritus, alopecia, and telogen effluvium. Patients may experience small fiber neuropathy in dermatomyositis.

Serologies for this patient, who had previously been diagnosed and treated for dermatomyositis, were significant for a positive ANA 1:2560. Anti-Jo-1 antibody was negative. Her liver function tests, aldolase, creatinine kinase, sedimentation rate, C-reactive protein, and serum protein electrophoresis were normal. Imaging revealed mild chronic interstitial lung disease. A malignancy workup was negative.

Treatment of dermatomyositis involves lifestyle changes and pharmacologic therapy. Because of the intense photosensitivity, patients should be diligent with their sun protection. Methotrexate, azathioprine, and mycophenolate mofetil are considered first-line therapies for dermatomyositis. Therapies such as cyclophosphamide, rituximab, IVIg, and plasmapheresis may also be indicated in severe or refractory cases. Additionally, patients with pulmonary involvement should be given systemic steroids. The side effects of these drugs must be considered in the context of the patient’s demographics, comorbidities and lifestyle.

This case and the photos were submitted by Lucas Shapiro, BS, of Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Natalie Y. Nasser, MD, of Kaiser Permanente Riverside Medical Center, Riverside, Calif. The column was edited by Dr. Bilu Martin.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Qudsiya Z and Waseem M. Dermatomyositis, in “StatPearls.” Treasure Island, Fla.: StatPearls Publishing, 2023 Jan.

2. Kamperman RG et al. Int J Mol Sci. 2022 Apr 13;23(8):4301.

3. Kassamali B et al. Int J WomensDermatol. 2021 Sep 24;7(5Part A):576-82.

4. Vázquez-Herrera NE et al. Skin Appendage Disord. 2018 Aug;4(3):187-99.

Cultures for bacteria, varicella zoster virus, herpes simplex virus, and mpox virus were all negative. A biopsy revealed suprabasilar acantholysis with follicular involvement in association with blister formation and inflammation. Direct immunofluorescence was positive for suprabasilar IgG and C₃ deposition, consistent with pemphigus vulgaris (PV).

PV is an autoimmune bullous disease in which antibodies are directed against desmoglein 1 and 3 and less commonly, plakoglobin. There is likely a genetic predisposition. Medications that may induce pemphigus include penicillamine, nifedipine, or captopril.

Clinically, flaccid blistering lesions are present that may be cutaneous and/or mucosal. Bullae can progress to erosions and crusting, which then heal with pigment alteration but not scarring. The most commonly affected sites are the mouth, intertriginous areas, face, and neck. Mucosal lesions may involve the lips, esophagus, conjunctiva, and genitals.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Cultures for bacteria, varicella zoster virus, herpes simplex virus, and mpox virus were all negative. A biopsy revealed suprabasilar acantholysis with follicular involvement in association with blister formation and inflammation. Direct immunofluorescence was positive for suprabasilar IgG and C₃ deposition, consistent with pemphigus vulgaris (PV).

PV is an autoimmune bullous disease in which antibodies are directed against desmoglein 1 and 3 and less commonly, plakoglobin. There is likely a genetic predisposition. Medications that may induce pemphigus include penicillamine, nifedipine, or captopril.

Clinically, flaccid blistering lesions are present that may be cutaneous and/or mucosal. Bullae can progress to erosions and crusting, which then heal with pigment alteration but not scarring. The most commonly affected sites are the mouth, intertriginous areas, face, and neck. Mucosal lesions may involve the lips, esophagus, conjunctiva, and genitals.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Cultures for bacteria, varicella zoster virus, herpes simplex virus, and mpox virus were all negative. A biopsy revealed suprabasilar acantholysis with follicular involvement in association with blister formation and inflammation. Direct immunofluorescence was positive for suprabasilar IgG and C₃ deposition, consistent with pemphigus vulgaris (PV).

PV is an autoimmune bullous disease in which antibodies are directed against desmoglein 1 and 3 and less commonly, plakoglobin. There is likely a genetic predisposition. Medications that may induce pemphigus include penicillamine, nifedipine, or captopril.

Clinically, flaccid blistering lesions are present that may be cutaneous and/or mucosal. Bullae can progress to erosions and crusting, which then heal with pigment alteration but not scarring. The most commonly affected sites are the mouth, intertriginous areas, face, and neck. Mucosal lesions may involve the lips, esophagus, conjunctiva, and genitals.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

Elephantiasis nostras verrucosa (ENV) is a chronic, uncommon and progressively disfiguring disease most commonly presenting on the bilateral lower extremities in the setting of chronic nonfilarial lymphedema resulting in secondary dermatologic sequelae.¹ Clinically, ENV presents as verrucous, hyperkeratotic, cobblestone-like patches, plaques, and nodules with associated nonpitting edema of the affected body area.¹ Secondary bacterial infections are common and often worsen the clinical course. The etiology of ENV involves chronic lymphatic obstruction and venous insufficiency, with additional risk factors including obesity, chronic lymphedema, bacterial infection, surgery or trauma, neoplasia, radiation, congestive heart failure, or scleroderma.^2,3 While most commonly presenting on the lower extremities, cases have been reported involving the abdomen, sacrum, ears, buttocks, and penoscrotal area.^1,2

Regardless of location, the pathogenesis of ENV remains the same. Chronic lymphatic obstruction results in accumulation and lymphostasis of protein-rich dermal fluid, which subsequently precipitates fibroblast proliferation and activation, suppression of the local immune response and development of recurrent lymphangitis, chronic inflammation, and potential secondary bacterial infection.^2,4

There is no standard of care for the treatment and management of ENV and recurrence is common. Interventions often involve those used for chronic lymphedema – including leg elevation, compression stockings or devices, skin hygiene, and lymphatic pumping.^2,3 Medical management with topical and oral retinoids has been reported, as well as emphasis on weight loss and infection control.^1,4 Surgical intervention is often reserved for refractory cases that fail to respond to more conservative management, or severe presentations resulting in extensive functional and aesthetic impairment. Less commonly reported treatment modalities include lymphaticovenular anastomosis and ablative carbon dioxide laser use, although this latter intervention demonstrated minimal improvement in this patient.^5,6

Penoscrotal ENV is a rare form of ENV affecting the genital region of males, often resulting in significant disfigurement, functional impairment, and psychosocial distress. Penoscrotal elephantiasis can be idiopathic, due to filarial infections, scleroinflammatory stricture of the urethra, Chlamydia trachomatis infection, and lymphostasis secondary to chronic inflammatory conditions such as streptococcal infections, radiotherapy, surgery, chronic venous stasis, or Kaposi sarcoma.⁷

In addition, hidradenitis suppurativa (HS) has been documented multiple times in the literature in association with the development of ENV, detailing lymphatic scarring secondary to chronic inguinal HS as the main pathogenic factor.^8,9

Surgery is the mainstay of treatment for penoscrotal ENV, which not only improves functionality and cosmesis, but also aids in prevention of rare malignant sequelae, such as lymphangiosarcoma.¹⁰ Such interventions can involve lymphangioplasty to aid in lymphatic drainage or excision of the mass and subcutaneous tissue with full-thickness skin grafting for reconstruction.⁷ Collaboration between urology, plastic surgery, and dermatology is often essential to obtain adequate care with satisfactory outcomes and minimal recurrence for patients with this uncommon condition.

This case and photo were submitted by Marlee Hill, a medical student at the University of Oklahoma, Oklahoma City; and Michael Franzetti, MD, and Jeffrey McBride, MD, department of dermatology, University of Oklahoma Health Sciences Center. The column was edited by Donna Bilu Martin, MD.
 

Dr. Donna Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to d[email protected].

References

1. Hadian Y et al. Dermatol Online J. 2019 Dec 15;25(12):13030/qt6rn1s8ff.

2. Judge N and Kilic A. J Dermatol Case Rep. 2016 Nov 13;10(2):32-4.

3. Dean SM et al. J Am Acad Dermatol. 2011 Jun;64(6):1104-10.

4. Sisto K and Khachemoune A. Am J Clin Dermatol. 2008;9(3):141-6.

5. Motegi S et al. Dermatology. 2007;215(2):147-51.

6. Robinson CG et al. J Cutan Med Surg. 2018;22(6):611-3.

7. Koualla S et al. Ann Chir Plast Esthet. 2023 Apr 10;S0294-1260(23)00035-3.

8. Lelonek E et al. Acta Derm Venereol. 2021 Feb 11;101(2):adv00389.

9. Good LM et al. J Am Acad Dermatol. 2011 May;64(5):993-4.

10. Cerri A et al. Eur J Dermatol. 1998 Oct-Nov;8(7):511-4.

Elephantiasis nostras verrucosa (ENV) is a chronic, uncommon and progressively disfiguring disease most commonly presenting on the bilateral lower extremities in the setting of chronic nonfilarial lymphedema resulting in secondary dermatologic sequelae.¹ Clinically, ENV presents as verrucous, hyperkeratotic, cobblestone-like patches, plaques, and nodules with associated nonpitting edema of the affected body area.¹ Secondary bacterial infections are common and often worsen the clinical course. The etiology of ENV involves chronic lymphatic obstruction and venous insufficiency, with additional risk factors including obesity, chronic lymphedema, bacterial infection, surgery or trauma, neoplasia, radiation, congestive heart failure, or scleroderma.^2,3 While most commonly presenting on the lower extremities, cases have been reported involving the abdomen, sacrum, ears, buttocks, and penoscrotal area.^1,2

Regardless of location, the pathogenesis of ENV remains the same. Chronic lymphatic obstruction results in accumulation and lymphostasis of protein-rich dermal fluid, which subsequently precipitates fibroblast proliferation and activation, suppression of the local immune response and development of recurrent lymphangitis, chronic inflammation, and potential secondary bacterial infection.^2,4

There is no standard of care for the treatment and management of ENV and recurrence is common. Interventions often involve those used for chronic lymphedema – including leg elevation, compression stockings or devices, skin hygiene, and lymphatic pumping.^2,3 Medical management with topical and oral retinoids has been reported, as well as emphasis on weight loss and infection control.^1,4 Surgical intervention is often reserved for refractory cases that fail to respond to more conservative management, or severe presentations resulting in extensive functional and aesthetic impairment. Less commonly reported treatment modalities include lymphaticovenular anastomosis and ablative carbon dioxide laser use, although this latter intervention demonstrated minimal improvement in this patient.^5,6

Penoscrotal ENV is a rare form of ENV affecting the genital region of males, often resulting in significant disfigurement, functional impairment, and psychosocial distress. Penoscrotal elephantiasis can be idiopathic, due to filarial infections, scleroinflammatory stricture of the urethra, Chlamydia trachomatis infection, and lymphostasis secondary to chronic inflammatory conditions such as streptococcal infections, radiotherapy, surgery, chronic venous stasis, or Kaposi sarcoma.⁷

In addition, hidradenitis suppurativa (HS) has been documented multiple times in the literature in association with the development of ENV, detailing lymphatic scarring secondary to chronic inguinal HS as the main pathogenic factor.^8,9

Surgery is the mainstay of treatment for penoscrotal ENV, which not only improves functionality and cosmesis, but also aids in prevention of rare malignant sequelae, such as lymphangiosarcoma.¹⁰ Such interventions can involve lymphangioplasty to aid in lymphatic drainage or excision of the mass and subcutaneous tissue with full-thickness skin grafting for reconstruction.⁷ Collaboration between urology, plastic surgery, and dermatology is often essential to obtain adequate care with satisfactory outcomes and minimal recurrence for patients with this uncommon condition.

This case and photo were submitted by Marlee Hill, a medical student at the University of Oklahoma, Oklahoma City; and Michael Franzetti, MD, and Jeffrey McBride, MD, department of dermatology, University of Oklahoma Health Sciences Center. The column was edited by Donna Bilu Martin, MD.
 

Dr. Donna Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to d[email protected].

References

1. Hadian Y et al. Dermatol Online J. 2019 Dec 15;25(12):13030/qt6rn1s8ff.

2. Judge N and Kilic A. J Dermatol Case Rep. 2016 Nov 13;10(2):32-4.

3. Dean SM et al. J Am Acad Dermatol. 2011 Jun;64(6):1104-10.

4. Sisto K and Khachemoune A. Am J Clin Dermatol. 2008;9(3):141-6.

5. Motegi S et al. Dermatology. 2007;215(2):147-51.

6. Robinson CG et al. J Cutan Med Surg. 2018;22(6):611-3.

7. Koualla S et al. Ann Chir Plast Esthet. 2023 Apr 10;S0294-1260(23)00035-3.

8. Lelonek E et al. Acta Derm Venereol. 2021 Feb 11;101(2):adv00389.

9. Good LM et al. J Am Acad Dermatol. 2011 May;64(5):993-4.

10. Cerri A et al. Eur J Dermatol. 1998 Oct-Nov;8(7):511-4.

Elephantiasis nostras verrucosa (ENV) is a chronic, uncommon and progressively disfiguring disease most commonly presenting on the bilateral lower extremities in the setting of chronic nonfilarial lymphedema resulting in secondary dermatologic sequelae.¹ Clinically, ENV presents as verrucous, hyperkeratotic, cobblestone-like patches, plaques, and nodules with associated nonpitting edema of the affected body area.¹ Secondary bacterial infections are common and often worsen the clinical course. The etiology of ENV involves chronic lymphatic obstruction and venous insufficiency, with additional risk factors including obesity, chronic lymphedema, bacterial infection, surgery or trauma, neoplasia, radiation, congestive heart failure, or scleroderma.^2,3 While most commonly presenting on the lower extremities, cases have been reported involving the abdomen, sacrum, ears, buttocks, and penoscrotal area.^1,2

Regardless of location, the pathogenesis of ENV remains the same. Chronic lymphatic obstruction results in accumulation and lymphostasis of protein-rich dermal fluid, which subsequently precipitates fibroblast proliferation and activation, suppression of the local immune response and development of recurrent lymphangitis, chronic inflammation, and potential secondary bacterial infection.^2,4

There is no standard of care for the treatment and management of ENV and recurrence is common. Interventions often involve those used for chronic lymphedema – including leg elevation, compression stockings or devices, skin hygiene, and lymphatic pumping.^2,3 Medical management with topical and oral retinoids has been reported, as well as emphasis on weight loss and infection control.^1,4 Surgical intervention is often reserved for refractory cases that fail to respond to more conservative management, or severe presentations resulting in extensive functional and aesthetic impairment. Less commonly reported treatment modalities include lymphaticovenular anastomosis and ablative carbon dioxide laser use, although this latter intervention demonstrated minimal improvement in this patient.^5,6

Penoscrotal ENV is a rare form of ENV affecting the genital region of males, often resulting in significant disfigurement, functional impairment, and psychosocial distress. Penoscrotal elephantiasis can be idiopathic, due to filarial infections, scleroinflammatory stricture of the urethra, Chlamydia trachomatis infection, and lymphostasis secondary to chronic inflammatory conditions such as streptococcal infections, radiotherapy, surgery, chronic venous stasis, or Kaposi sarcoma.⁷

In addition, hidradenitis suppurativa (HS) has been documented multiple times in the literature in association with the development of ENV, detailing lymphatic scarring secondary to chronic inguinal HS as the main pathogenic factor.^8,9

Surgery is the mainstay of treatment for penoscrotal ENV, which not only improves functionality and cosmesis, but also aids in prevention of rare malignant sequelae, such as lymphangiosarcoma.¹⁰ Such interventions can involve lymphangioplasty to aid in lymphatic drainage or excision of the mass and subcutaneous tissue with full-thickness skin grafting for reconstruction.⁷ Collaboration between urology, plastic surgery, and dermatology is often essential to obtain adequate care with satisfactory outcomes and minimal recurrence for patients with this uncommon condition.

This case and photo were submitted by Marlee Hill, a medical student at the University of Oklahoma, Oklahoma City; and Michael Franzetti, MD, and Jeffrey McBride, MD, department of dermatology, University of Oklahoma Health Sciences Center. The column was edited by Donna Bilu Martin, MD.
 

Dr. Donna Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to d[email protected].

References

1. Hadian Y et al. Dermatol Online J. 2019 Dec 15;25(12):13030/qt6rn1s8ff.

2. Judge N and Kilic A. J Dermatol Case Rep. 2016 Nov 13;10(2):32-4.

3. Dean SM et al. J Am Acad Dermatol. 2011 Jun;64(6):1104-10.

4. Sisto K and Khachemoune A. Am J Clin Dermatol. 2008;9(3):141-6.

5. Motegi S et al. Dermatology. 2007;215(2):147-51.

6. Robinson CG et al. J Cutan Med Surg. 2018;22(6):611-3.

7. Koualla S et al. Ann Chir Plast Esthet. 2023 Apr 10;S0294-1260(23)00035-3.

8. Lelonek E et al. Acta Derm Venereol. 2021 Feb 11;101(2):adv00389.

9. Good LM et al. J Am Acad Dermatol. 2011 May;64(5):993-4.

10. Cerri A et al. Eur J Dermatol. 1998 Oct-Nov;8(7):511-4.

In this patient, bacterial and viral cultures were taken and varicella zoster virus (VZV) was isolated. Additionally, Orthopox DNA by PCR and Monkeypox (mpox) virus DNA by PCR were detected. Herpes simplex virus and bacterial viral cultures were negative. Valacyclovir was started at the time of presentation and the patient’s lesions resolved without sequelae.

Mpox is a zoonotic double-stranded DNA virus that is part of the Orthopoxvirus family, including the West African and Central African variants. This disease presents similarly to smallpox, so most mpox research was conducted around the time smallpox was eradicated. It was not until 1970, when the disease was isolated from a patient with suspected smallpox in the Democratic Republic of the Congo (DRC), that human mpox was considered a distinct disease. An epidemic outbreak in the United States occurred in 2003 related to infected prairie dogs, and travel-related outbreaks have been more recently reported up until May 2022, in which mpox was reported in nonendemic areas including North America, Europe, and Australia. Most cases in this outbreak occurred in men who have sex with men (MSM), but this is not always the case, and mpox is not necessarily considered a sexually transmitted infection. Mpox presents similarly to smallpox and VZV, so using laboratory tests is important in diagnosing and tracking this disease.

Although it is not easily transmitted, the disease can spread through bodily secretions both directly and indirectly. Mpox typically begins with a prodrome that includes fever, headache, myalgia, and fatigue. This is followed by lymphadenopathy that precedes and coincides with rash development. The lymph nodes are firm, tender, may be painful, and are a defining factor in presentation that differs from smallpox and varicella. The rash typically starts on the face, then presents on the body in a centrifugal distribution. However, cases related to sexual transmission present with anogenital lesions. The lesions are characterized by a progression from maculopapular to vesiculopustular, and can vary widely in quantity.

Notably, individuals are contagious from the onset of the prodrome until the lesions have scabbed over and fallen off. The eruptive nature of the later lesions poses a threat of secondary infection, and is often accompanied by a second febrile period that signifies deterioration of the patient’s condition. Other signs of secondary infection are variable and include pulmonary symptoms, vomiting, diarrhea, ocular infections, and in rare cases, encephalitis. These sequelae are more common in unvaccinated and immunocompromised individuals. Long-term complications of mpox include pitted scarring from cutaneous lesions with children being more susceptible to severe disease. The mortality rate for the disease is very low. (As of May 10, 2023, there have been 30,395 mpox cases reported in the United States, and 42 deaths, according to the Centers for Disease Control and Prevention.)

There are a variety of diagnostic tests that can aid in mpox identification, but they are most strongly supported when combined with clinical and epidemiological data. The best, least invasive method includes collection of lesion exudate or crust on a swab, and viral DNA is best preserved by keeping the specimen in a cool, dry, and dark environment. PCR is considered the standard, and electron microscopy and immunohistochemistry are valid tests, but all modalities require sophisticated technicians with the proper laboratory equipment. This is limiting because many cases present in underserved areas that lack the facilities for proper, real-time analysis. Antigen and antibody-based tests can be used, but cross-reactivity of other orthopoxviridae limits confirmation of mpox infection. Vaccination status, history and location must be considered.

Vaccination is the chief form of prevention for mpox, although it is not considered entirely protective. Smallpox vaccination provides protection, but widespread administration of the vaccine is no longer practiced, and an estimated 70% of the global population is no longer vaccinated. Vaccination is recommended for anyone at risk of exposure, but as this is a live, attenuated vaccine, the immune status of the patient is important to keep in mind. Tecovirimat and other antiviral medications including cidofovir and brincidofovir may be considered in severe cases.

This case is unique as our patient, who had no known risk factors for mpox, presented with mpox and VZV, simultaneously. Although clinical presentation and epidemiological patterns between these diseases differ, there have been a limited number of cases of coinfection reported in the literature, mainly in the DRC where mpox is endemic. Diagnosis must be made by separate laboratory tests and there are differences in presentation between independent and coinfection for these viruses. Notably, patients with mpox/VZV coinfection may be less likely to present with lesions on the face, thorax, arms, palms, and soles than those with only mpox but experience a higher lesion burden than those afflicted by only VZV. Coinfection may be related to reactivation of dormant VZV, or increased susceptibility to secondary infection when infected with one virus.

This case and photo were submitted by Lucas Shapiro, BS, of the Dr. Kiran C. Patel College of Osteopathic Medicine at Nova Southeastern University, Fort Lauderdale, Fla., and Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Macneil A et al. Clin Infect Dis. 2009 Jan 1;48(1):e6-8.

2. Di Gennaro F et al. Microorganisms. 2022 Aug 12;10(8):1633.

3. Hughes CM et al. Am J Trop Med Hyg. 2020 Dec 7;104(2):604-11.

In this patient, bacterial and viral cultures were taken and varicella zoster virus (VZV) was isolated. Additionally, Orthopox DNA by PCR and Monkeypox (mpox) virus DNA by PCR were detected. Herpes simplex virus and bacterial viral cultures were negative. Valacyclovir was started at the time of presentation and the patient’s lesions resolved without sequelae.

Mpox is a zoonotic double-stranded DNA virus that is part of the Orthopoxvirus family, including the West African and Central African variants. This disease presents similarly to smallpox, so most mpox research was conducted around the time smallpox was eradicated. It was not until 1970, when the disease was isolated from a patient with suspected smallpox in the Democratic Republic of the Congo (DRC), that human mpox was considered a distinct disease. An epidemic outbreak in the United States occurred in 2003 related to infected prairie dogs, and travel-related outbreaks have been more recently reported up until May 2022, in which mpox was reported in nonendemic areas including North America, Europe, and Australia. Most cases in this outbreak occurred in men who have sex with men (MSM), but this is not always the case, and mpox is not necessarily considered a sexually transmitted infection. Mpox presents similarly to smallpox and VZV, so using laboratory tests is important in diagnosing and tracking this disease.

Although it is not easily transmitted, the disease can spread through bodily secretions both directly and indirectly. Mpox typically begins with a prodrome that includes fever, headache, myalgia, and fatigue. This is followed by lymphadenopathy that precedes and coincides with rash development. The lymph nodes are firm, tender, may be painful, and are a defining factor in presentation that differs from smallpox and varicella. The rash typically starts on the face, then presents on the body in a centrifugal distribution. However, cases related to sexual transmission present with anogenital lesions. The lesions are characterized by a progression from maculopapular to vesiculopustular, and can vary widely in quantity.

Notably, individuals are contagious from the onset of the prodrome until the lesions have scabbed over and fallen off. The eruptive nature of the later lesions poses a threat of secondary infection, and is often accompanied by a second febrile period that signifies deterioration of the patient’s condition. Other signs of secondary infection are variable and include pulmonary symptoms, vomiting, diarrhea, ocular infections, and in rare cases, encephalitis. These sequelae are more common in unvaccinated and immunocompromised individuals. Long-term complications of mpox include pitted scarring from cutaneous lesions with children being more susceptible to severe disease. The mortality rate for the disease is very low. (As of May 10, 2023, there have been 30,395 mpox cases reported in the United States, and 42 deaths, according to the Centers for Disease Control and Prevention.)

There are a variety of diagnostic tests that can aid in mpox identification, but they are most strongly supported when combined with clinical and epidemiological data. The best, least invasive method includes collection of lesion exudate or crust on a swab, and viral DNA is best preserved by keeping the specimen in a cool, dry, and dark environment. PCR is considered the standard, and electron microscopy and immunohistochemistry are valid tests, but all modalities require sophisticated technicians with the proper laboratory equipment. This is limiting because many cases present in underserved areas that lack the facilities for proper, real-time analysis. Antigen and antibody-based tests can be used, but cross-reactivity of other orthopoxviridae limits confirmation of mpox infection. Vaccination status, history and location must be considered.

Vaccination is the chief form of prevention for mpox, although it is not considered entirely protective. Smallpox vaccination provides protection, but widespread administration of the vaccine is no longer practiced, and an estimated 70% of the global population is no longer vaccinated. Vaccination is recommended for anyone at risk of exposure, but as this is a live, attenuated vaccine, the immune status of the patient is important to keep in mind. Tecovirimat and other antiviral medications including cidofovir and brincidofovir may be considered in severe cases.

This case is unique as our patient, who had no known risk factors for mpox, presented with mpox and VZV, simultaneously. Although clinical presentation and epidemiological patterns between these diseases differ, there have been a limited number of cases of coinfection reported in the literature, mainly in the DRC where mpox is endemic. Diagnosis must be made by separate laboratory tests and there are differences in presentation between independent and coinfection for these viruses. Notably, patients with mpox/VZV coinfection may be less likely to present with lesions on the face, thorax, arms, palms, and soles than those with only mpox but experience a higher lesion burden than those afflicted by only VZV. Coinfection may be related to reactivation of dormant VZV, or increased susceptibility to secondary infection when infected with one virus.

This case and photo were submitted by Lucas Shapiro, BS, of the Dr. Kiran C. Patel College of Osteopathic Medicine at Nova Southeastern University, Fort Lauderdale, Fla., and Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Macneil A et al. Clin Infect Dis. 2009 Jan 1;48(1):e6-8.

2. Di Gennaro F et al. Microorganisms. 2022 Aug 12;10(8):1633.

3. Hughes CM et al. Am J Trop Med Hyg. 2020 Dec 7;104(2):604-11.

In this patient, bacterial and viral cultures were taken and varicella zoster virus (VZV) was isolated. Additionally, Orthopox DNA by PCR and Monkeypox (mpox) virus DNA by PCR were detected. Herpes simplex virus and bacterial viral cultures were negative. Valacyclovir was started at the time of presentation and the patient’s lesions resolved without sequelae.

Mpox is a zoonotic double-stranded DNA virus that is part of the Orthopoxvirus family, including the West African and Central African variants. This disease presents similarly to smallpox, so most mpox research was conducted around the time smallpox was eradicated. It was not until 1970, when the disease was isolated from a patient with suspected smallpox in the Democratic Republic of the Congo (DRC), that human mpox was considered a distinct disease. An epidemic outbreak in the United States occurred in 2003 related to infected prairie dogs, and travel-related outbreaks have been more recently reported up until May 2022, in which mpox was reported in nonendemic areas including North America, Europe, and Australia. Most cases in this outbreak occurred in men who have sex with men (MSM), but this is not always the case, and mpox is not necessarily considered a sexually transmitted infection. Mpox presents similarly to smallpox and VZV, so using laboratory tests is important in diagnosing and tracking this disease.

Although it is not easily transmitted, the disease can spread through bodily secretions both directly and indirectly. Mpox typically begins with a prodrome that includes fever, headache, myalgia, and fatigue. This is followed by lymphadenopathy that precedes and coincides with rash development. The lymph nodes are firm, tender, may be painful, and are a defining factor in presentation that differs from smallpox and varicella. The rash typically starts on the face, then presents on the body in a centrifugal distribution. However, cases related to sexual transmission present with anogenital lesions. The lesions are characterized by a progression from maculopapular to vesiculopustular, and can vary widely in quantity.

Notably, individuals are contagious from the onset of the prodrome until the lesions have scabbed over and fallen off. The eruptive nature of the later lesions poses a threat of secondary infection, and is often accompanied by a second febrile period that signifies deterioration of the patient’s condition. Other signs of secondary infection are variable and include pulmonary symptoms, vomiting, diarrhea, ocular infections, and in rare cases, encephalitis. These sequelae are more common in unvaccinated and immunocompromised individuals. Long-term complications of mpox include pitted scarring from cutaneous lesions with children being more susceptible to severe disease. The mortality rate for the disease is very low. (As of May 10, 2023, there have been 30,395 mpox cases reported in the United States, and 42 deaths, according to the Centers for Disease Control and Prevention.)

There are a variety of diagnostic tests that can aid in mpox identification, but they are most strongly supported when combined with clinical and epidemiological data. The best, least invasive method includes collection of lesion exudate or crust on a swab, and viral DNA is best preserved by keeping the specimen in a cool, dry, and dark environment. PCR is considered the standard, and electron microscopy and immunohistochemistry are valid tests, but all modalities require sophisticated technicians with the proper laboratory equipment. This is limiting because many cases present in underserved areas that lack the facilities for proper, real-time analysis. Antigen and antibody-based tests can be used, but cross-reactivity of other orthopoxviridae limits confirmation of mpox infection. Vaccination status, history and location must be considered.

Vaccination is the chief form of prevention for mpox, although it is not considered entirely protective. Smallpox vaccination provides protection, but widespread administration of the vaccine is no longer practiced, and an estimated 70% of the global population is no longer vaccinated. Vaccination is recommended for anyone at risk of exposure, but as this is a live, attenuated vaccine, the immune status of the patient is important to keep in mind. Tecovirimat and other antiviral medications including cidofovir and brincidofovir may be considered in severe cases.

This case is unique as our patient, who had no known risk factors for mpox, presented with mpox and VZV, simultaneously. Although clinical presentation and epidemiological patterns between these diseases differ, there have been a limited number of cases of coinfection reported in the literature, mainly in the DRC where mpox is endemic. Diagnosis must be made by separate laboratory tests and there are differences in presentation between independent and coinfection for these viruses. Notably, patients with mpox/VZV coinfection may be less likely to present with lesions on the face, thorax, arms, palms, and soles than those with only mpox but experience a higher lesion burden than those afflicted by only VZV. Coinfection may be related to reactivation of dormant VZV, or increased susceptibility to secondary infection when infected with one virus.

This case and photo were submitted by Lucas Shapiro, BS, of the Dr. Kiran C. Patel College of Osteopathic Medicine at Nova Southeastern University, Fort Lauderdale, Fla., and Donna Bilu Martin, MD.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Macneil A et al. Clin Infect Dis. 2009 Jan 1;48(1):e6-8.

2. Di Gennaro F et al. Microorganisms. 2022 Aug 12;10(8):1633.

3. Hughes CM et al. Am J Trop Med Hyg. 2020 Dec 7;104(2):604-11.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is a specific categorization of cutaneous B-cell lymphoma (CBCL) demonstrating a predominance of immunoblasts and centroblasts with scarce T-cells, classically presenting as rapidly progressive, plum-colored lesions on the lower extremities.^1,2 CBCLs, with PCDLBCL-LT accounting for 4%, make up the minority of cutaneous lymphomas in the Western world.^1-3 The leg type variant, typically demonstrating a female predominance and median age of onset in the 70s, is clinically aggressive and associated with a poorer prognosis, increased recurrence rate, and 40%-60% 5-year survival rate.^1-5

Histologically, this variant demonstrates a diffuse sheet-like growth of enlarged atypical B-cells distinctively separated from the epidermis by a prominent grenz zone. Classic PCDLBCL-LT immunophenotype includes B-cell markers CD20 and IgM; triple expressor phenotype indicating c-MYC, BCL-2, and BCL-6 positivity; as well as CD10 negativity, lack of BCL-2 rearrangement, and presence of a positive MYD-88 molecular result.

Marlee Hill, University of Oklahoma

Other characteristic histopathological findings include positivity for post-germinal markers IRF4/MUM-1 and FOXP-1, positivity for additional B-cell markers, including CD79 and PAX5, and negativity of t(14;18) (q32;21).^1,3-5

This case is of significant interest as it falls within the approximately 10% of PCDLBCL-LT cases demonstrating weak to negative MUM-1 staining, in addition to its presentation in a younger male individual.

Marlee Hill, University of Oklahoma

While MUM-1 positivity is common in this subtype, its presence, or lack thereof, should not be looked at in isolation when evaluating diagnostic criteria, nor has it been shown to have a statistically significant effect on survival rate – in contrast to factors like lesion location on the leg versus non-leg lesions, multiple lesions at diagnosis, and dissemination to other sites.2,6

PCDLBCL-LT can uncommonly present in non-leg locations and only 10% depict associated B-symptoms, such as fatigue, night sweats, weight loss, or lymphadenopathy.^2,6 First-line treatment is with the R-CHOP chemotherapy regimen – consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone – although radiotherapy is sometimes considered in patients with a single small lesion.^1,2

Because of possible cutaneous involvement beyond the legs, common lack of systemic symptoms, and variable immunophenotypes, this case of MUM-1 negative PCDLBCL-LT highlights the importance of a clinicopathological approach to differentiate the subtypes of CBCLs, allowing for proper and individualized stratification of risk, prognosis, and treatment.
 

This case was submitted and written by Marlee Hill, BS, Michael Franzetti, MD, Jeffrey McBride, MD, and Allison Hood, MD, of the University of Oklahoma, Oklahoma City. They also provided the photos. Donna Bilu Martin, MD, edited the column.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Willemze R et al. Blood. 2019;133(16):1703-14.

2. Willemze R et al. Blood. 2005;105(10):3768-85.

3. Sukswai N et al. Pathology. 2020;52(1):53-67.

4. Hristov AC. Arch Pathol Lab Med. 2012;136(8):876-81.

5. Sokol L et al. Cancer Control. 2012;19(3):236-44.

6. Grange F et al. Arch Dermatol. 2007;143(9):1144-50.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is a specific categorization of cutaneous B-cell lymphoma (CBCL) demonstrating a predominance of immunoblasts and centroblasts with scarce T-cells, classically presenting as rapidly progressive, plum-colored lesions on the lower extremities.^1,2 CBCLs, with PCDLBCL-LT accounting for 4%, make up the minority of cutaneous lymphomas in the Western world.^1-3 The leg type variant, typically demonstrating a female predominance and median age of onset in the 70s, is clinically aggressive and associated with a poorer prognosis, increased recurrence rate, and 40%-60% 5-year survival rate.^1-5

Histologically, this variant demonstrates a diffuse sheet-like growth of enlarged atypical B-cells distinctively separated from the epidermis by a prominent grenz zone. Classic PCDLBCL-LT immunophenotype includes B-cell markers CD20 and IgM; triple expressor phenotype indicating c-MYC, BCL-2, and BCL-6 positivity; as well as CD10 negativity, lack of BCL-2 rearrangement, and presence of a positive MYD-88 molecular result.

Marlee Hill, University of Oklahoma

Other characteristic histopathological findings include positivity for post-germinal markers IRF4/MUM-1 and FOXP-1, positivity for additional B-cell markers, including CD79 and PAX5, and negativity of t(14;18) (q32;21).^1,3-5

This case is of significant interest as it falls within the approximately 10% of PCDLBCL-LT cases demonstrating weak to negative MUM-1 staining, in addition to its presentation in a younger male individual.

Marlee Hill, University of Oklahoma

While MUM-1 positivity is common in this subtype, its presence, or lack thereof, should not be looked at in isolation when evaluating diagnostic criteria, nor has it been shown to have a statistically significant effect on survival rate – in contrast to factors like lesion location on the leg versus non-leg lesions, multiple lesions at diagnosis, and dissemination to other sites.2,6

PCDLBCL-LT can uncommonly present in non-leg locations and only 10% depict associated B-symptoms, such as fatigue, night sweats, weight loss, or lymphadenopathy.^2,6 First-line treatment is with the R-CHOP chemotherapy regimen – consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone – although radiotherapy is sometimes considered in patients with a single small lesion.^1,2

Because of possible cutaneous involvement beyond the legs, common lack of systemic symptoms, and variable immunophenotypes, this case of MUM-1 negative PCDLBCL-LT highlights the importance of a clinicopathological approach to differentiate the subtypes of CBCLs, allowing for proper and individualized stratification of risk, prognosis, and treatment.
 

This case was submitted and written by Marlee Hill, BS, Michael Franzetti, MD, Jeffrey McBride, MD, and Allison Hood, MD, of the University of Oklahoma, Oklahoma City. They also provided the photos. Donna Bilu Martin, MD, edited the column.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Willemze R et al. Blood. 2019;133(16):1703-14.

2. Willemze R et al. Blood. 2005;105(10):3768-85.

3. Sukswai N et al. Pathology. 2020;52(1):53-67.

4. Hristov AC. Arch Pathol Lab Med. 2012;136(8):876-81.

5. Sokol L et al. Cancer Control. 2012;19(3):236-44.

6. Grange F et al. Arch Dermatol. 2007;143(9):1144-50.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is a specific categorization of cutaneous B-cell lymphoma (CBCL) demonstrating a predominance of immunoblasts and centroblasts with scarce T-cells, classically presenting as rapidly progressive, plum-colored lesions on the lower extremities.^1,2 CBCLs, with PCDLBCL-LT accounting for 4%, make up the minority of cutaneous lymphomas in the Western world.^1-3 The leg type variant, typically demonstrating a female predominance and median age of onset in the 70s, is clinically aggressive and associated with a poorer prognosis, increased recurrence rate, and 40%-60% 5-year survival rate.^1-5

Histologically, this variant demonstrates a diffuse sheet-like growth of enlarged atypical B-cells distinctively separated from the epidermis by a prominent grenz zone. Classic PCDLBCL-LT immunophenotype includes B-cell markers CD20 and IgM; triple expressor phenotype indicating c-MYC, BCL-2, and BCL-6 positivity; as well as CD10 negativity, lack of BCL-2 rearrangement, and presence of a positive MYD-88 molecular result.

Marlee Hill, University of Oklahoma

Other characteristic histopathological findings include positivity for post-germinal markers IRF4/MUM-1 and FOXP-1, positivity for additional B-cell markers, including CD79 and PAX5, and negativity of t(14;18) (q32;21).^1,3-5

This case is of significant interest as it falls within the approximately 10% of PCDLBCL-LT cases demonstrating weak to negative MUM-1 staining, in addition to its presentation in a younger male individual.

Marlee Hill, University of Oklahoma

While MUM-1 positivity is common in this subtype, its presence, or lack thereof, should not be looked at in isolation when evaluating diagnostic criteria, nor has it been shown to have a statistically significant effect on survival rate – in contrast to factors like lesion location on the leg versus non-leg lesions, multiple lesions at diagnosis, and dissemination to other sites.2,6

PCDLBCL-LT can uncommonly present in non-leg locations and only 10% depict associated B-symptoms, such as fatigue, night sweats, weight loss, or lymphadenopathy.^2,6 First-line treatment is with the R-CHOP chemotherapy regimen – consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone – although radiotherapy is sometimes considered in patients with a single small lesion.^1,2

Because of possible cutaneous involvement beyond the legs, common lack of systemic symptoms, and variable immunophenotypes, this case of MUM-1 negative PCDLBCL-LT highlights the importance of a clinicopathological approach to differentiate the subtypes of CBCLs, allowing for proper and individualized stratification of risk, prognosis, and treatment.
 

This case was submitted and written by Marlee Hill, BS, Michael Franzetti, MD, Jeffrey McBride, MD, and Allison Hood, MD, of the University of Oklahoma, Oklahoma City. They also provided the photos. Donna Bilu Martin, MD, edited the column.

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

1. Willemze R et al. Blood. 2019;133(16):1703-14.

2. Willemze R et al. Blood. 2005;105(10):3768-85.

3. Sukswai N et al. Pathology. 2020;52(1):53-67.

4. Hristov AC. Arch Pathol Lab Med. 2012;136(8):876-81.

5. Sokol L et al. Cancer Control. 2012;19(3):236-44.

6. Grange F et al. Arch Dermatol. 2007;143(9):1144-50.

Two feet–one hand syndrome

Two feet–one hand syndrome is a common term used to describe tinea manuum on one hand with bilateral tinea pedis. This condition, also known as ringworm, is a fungal infection caused by a dermatophyte, and presents as a superficial annular or circular rash with a raised, scaly border.

Symptoms include dryness and itchiness, and the lesions may appear red-pink on lighter skin and gray-brown on darker skin types. Although these infections can arise in a variety of combinations, two feet–one hand syndrome occurs in about 60% of cases. Trichophyton rubrum is the most common agent.

Diagnosis is made by patient history, dermoscopic visualization, and staining of skin scraping with KOH or fungal culture. Dermatophytes prefer moist, warm environments, so this disease is prevalent in tropical conditions and associated with moist public areas such as locker rooms and showers. As a result, tinea pedis is also nicknamed “athlete’s foot” for its common presentation in athletes. The fungus spreads easily through contact and can survive on infected surfaces, so patients often self-inoculate by touching/scratching the affected area then touching another body part. Cautions that should be taken to avoid transmission include not sharing personal care products, washing the area and keeping it dry, and avoiding close, humid environments.

Dr. Donna Bilu Martin

The syndrome is highly associated with onychomycosis, which can be more difficult to treat and often requires oral antifungals. Tinea manuum is commonly misdiagnosed as hand dermatitis or eczema and treated with topical steroids, which will exacerbate or flare the tinea.

Two feet–one hand syndrome can typically be treated with over-the-counter topical antifungal medications such as miconazole or clotrimazole. Topical ketoconazole may be prescribed, and oral terbinafine or itraconazole are used in more severe cases when a larger body surface area is affected or in immunocompromised patients.

Dr. Donna Bilu Martin

This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University, Davie, Fla.; Kiran C. Patel, Tampa Bay Regional Campus; and Dr. Bilu Martin.
 

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Cleveland Clinic. Tinea manuum: Symptoms, causes & treatment. 2022. https://my.clevelandclinic.org/health/diseases/24063-tinea-manuum.

Ugalde-Trejo NX et al. Curr Fungal Infect Rep. 2022 Nov 17. doi: 10.1007/s12281-022-00447-9.

Mizumoto J. Cureus. 2021 Dec 27;13(12):e20758.

Two feet–one hand syndrome

Two feet–one hand syndrome is a common term used to describe tinea manuum on one hand with bilateral tinea pedis. This condition, also known as ringworm, is a fungal infection caused by a dermatophyte, and presents as a superficial annular or circular rash with a raised, scaly border.

Symptoms include dryness and itchiness, and the lesions may appear red-pink on lighter skin and gray-brown on darker skin types. Although these infections can arise in a variety of combinations, two feet–one hand syndrome occurs in about 60% of cases. Trichophyton rubrum is the most common agent.

Diagnosis is made by patient history, dermoscopic visualization, and staining of skin scraping with KOH or fungal culture. Dermatophytes prefer moist, warm environments, so this disease is prevalent in tropical conditions and associated with moist public areas such as locker rooms and showers. As a result, tinea pedis is also nicknamed “athlete’s foot” for its common presentation in athletes. The fungus spreads easily through contact and can survive on infected surfaces, so patients often self-inoculate by touching/scratching the affected area then touching another body part. Cautions that should be taken to avoid transmission include not sharing personal care products, washing the area and keeping it dry, and avoiding close, humid environments.

Dr. Donna Bilu Martin

The syndrome is highly associated with onychomycosis, which can be more difficult to treat and often requires oral antifungals. Tinea manuum is commonly misdiagnosed as hand dermatitis or eczema and treated with topical steroids, which will exacerbate or flare the tinea.

Two feet–one hand syndrome can typically be treated with over-the-counter topical antifungal medications such as miconazole or clotrimazole. Topical ketoconazole may be prescribed, and oral terbinafine or itraconazole are used in more severe cases when a larger body surface area is affected or in immunocompromised patients.

Dr. Donna Bilu Martin

This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University, Davie, Fla.; Kiran C. Patel, Tampa Bay Regional Campus; and Dr. Bilu Martin.
 

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Cleveland Clinic. Tinea manuum: Symptoms, causes & treatment. 2022. https://my.clevelandclinic.org/health/diseases/24063-tinea-manuum.

Ugalde-Trejo NX et al. Curr Fungal Infect Rep. 2022 Nov 17. doi: 10.1007/s12281-022-00447-9.

Mizumoto J. Cureus. 2021 Dec 27;13(12):e20758.

Two feet–one hand syndrome

Two feet–one hand syndrome is a common term used to describe tinea manuum on one hand with bilateral tinea pedis. This condition, also known as ringworm, is a fungal infection caused by a dermatophyte, and presents as a superficial annular or circular rash with a raised, scaly border.

Symptoms include dryness and itchiness, and the lesions may appear red-pink on lighter skin and gray-brown on darker skin types. Although these infections can arise in a variety of combinations, two feet–one hand syndrome occurs in about 60% of cases. Trichophyton rubrum is the most common agent.

Diagnosis is made by patient history, dermoscopic visualization, and staining of skin scraping with KOH or fungal culture. Dermatophytes prefer moist, warm environments, so this disease is prevalent in tropical conditions and associated with moist public areas such as locker rooms and showers. As a result, tinea pedis is also nicknamed “athlete’s foot” for its common presentation in athletes. The fungus spreads easily through contact and can survive on infected surfaces, so patients often self-inoculate by touching/scratching the affected area then touching another body part. Cautions that should be taken to avoid transmission include not sharing personal care products, washing the area and keeping it dry, and avoiding close, humid environments.

Dr. Donna Bilu Martin

The syndrome is highly associated with onychomycosis, which can be more difficult to treat and often requires oral antifungals. Tinea manuum is commonly misdiagnosed as hand dermatitis or eczema and treated with topical steroids, which will exacerbate or flare the tinea.

Two feet–one hand syndrome can typically be treated with over-the-counter topical antifungal medications such as miconazole or clotrimazole. Topical ketoconazole may be prescribed, and oral terbinafine or itraconazole are used in more severe cases when a larger body surface area is affected or in immunocompromised patients.

Dr. Donna Bilu Martin

This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University, Davie, Fla.; Kiran C. Patel, Tampa Bay Regional Campus; and Dr. Bilu Martin.
 

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, MD, in Aventura, Fla. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].

References

Cleveland Clinic. Tinea manuum: Symptoms, causes & treatment. 2022. https://my.clevelandclinic.org/health/diseases/24063-tinea-manuum.

Ugalde-Trejo NX et al. Curr Fungal Infect Rep. 2022 Nov 17. doi: 10.1007/s12281-022-00447-9.

Mizumoto J. Cureus. 2021 Dec 27;13(12):e20758.