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A 50-year-old White male presented with a 4- to 5-year history of progressively growing violaceous lesions on his left lower extremity
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.
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.
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.
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.
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.
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.
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.
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.
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.
There was no cervical, axillary, or inguinal lymphadenopathy.
White male presents with pruritic, scaly, erythematous patches on his feet and left hand
Two feet–one hand syndrome
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.
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.
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
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.
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.
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
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.
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.
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.
A White male presented with a 1½-year history of a progressive hypoesthetic annular, hyperpigmented plaque on the upper arm
Paucibacillary tuberculoid leprosy is characterized by few anesthetic hypo- or hyperpigmented lesions and can be accompanied by palpable peripheral nerve enlargements.
Tuberculoid leprosy presents histologically with epithelioid histiocytes with lymphocytes and Langhans giant cells. Neurotropic granulomas are also characteristic of tuberculoid leprosy. Fite staining allows for the identification of the acid-fast bacilli of M. leprae, which in some cases are quite few in number. The standard mycobacterium stain, Ziehl-Neelsen, is a good option for M. tuberculosis, but because of the relative weak mycolic acid coat of M. leprae, the Fite stain is more appropriate for identifying M. leprae.
Clinically, other than the presence of fewer than five hypoesthetic lesions that are either hypopigmented or erythematous, tuberculoid leprosy often presents with additional peripheral nerve involvement that manifests as numbness and tingling in hands and feet.1 This patient denied any tingling, weakness, or numbness, outside of the anesthetic lesion on his posterior upper arm.
The patient, born in the United States, had a remote history of military travel to Iraq, Kuwait, and the Philippines, but had not traveled internationally within the last 15 years, apart from a cruise to the Bahamas. He denied any known contact with individuals with similar lesions. He denied a history of contact with armadillos, but acknowledged that they are native to where he resides in central Florida, and that he had seen them in his yard.
Histopathological examination revealed an unremarkable epidermis with a superficial and deep perivascular, periadnexal, and perineural lymphohistiocytic infiltrate. Fite stain revealed rare rod-shaped organisms (Figure 2). These findings are consistent with a diagnosis of paucibacillary, tuberculoid leprosy.
The patient’s travel history to highly endemic areas (Middle East), as well as possible environmental contact with armadillos – including contact with soil that the armadillos occupied – could explain plausible modes of transmission. Following consultation with our infectious disease department and the National Hansen’s Disease Program, our patient began a planned course of therapy with 18 months of minocycline, rifampin, and moxifloxacin.
Human-to-human transmission of HD has been well documented; however, zoonotic transmission – specifically via the nine-banded armadillo (Dasypus novemcinctus) – serves as another suggested means of transmission, especially in the Southeastern United States.2-6 Travel to highly-endemic areas increases the risk of contracting HD, which may take up to 20 years following contact with the bacteria to manifest clinically.
While central Florida was previously thought to be a nonendemic area of disease, the incidence of the disease in this region has increased in recent years.7 Human-to-human transmission, which remains a concern with immigration from highly-endemic regions, occurs via long-term contact with nasal droplets of an infected person.8,9
Many patients in regions with very few cases of leprosy deny travel to other endemic regions and contact with infected people. Thus, zoonotic transmission remains a legitimate concern in the Southeastern United States – accounting, at least in part, for many of the non–human-transmitted cases of leprosy.2,10 We encourage clinicians to maintain a high level of clinical suspicion for leprosy when evaluating patients presenting with hypoesthetic cutaneous lesions and to obtain a travel history and to ask about armadillo exposure.
This case and the photos were submitted by Ms. Smith, from the University of South Florida, Tampa; Dr. Hatch and Dr. Sarriera-Lazaro, from the department of dermatology and cutaneous surgery, University of South Florida; and Dr. Turner and Dr. Beachkofsky, from the department of pathology and laboratory medicine at the James A. Haley Veterans’ Hospital, Tampa. Dr. Bilu Martin edited this case. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].
References
1. Leprosy (Hansen’s Disease), in: “Goldman’s Cecil Medicine,” 24th ed. (Philadelphia: W.B. Saunders, 2012: pp. 1950-4.
2. Sharma R et al. Emerg Infect Dis. 2015 Dec;21(12):2127-34.
3. Lane JE et al. J Am Acad Dermatol. 2006 Oct;55(4):714-6.
4. Clark BM et al. Am J Trop Med Hyg. 2008 Jun;78(6):962-7.
5. Bruce S et al. J Am Acad Dermatol. 2000 Aug;43(2 Pt 1):223-8.
6. Loughry WJ et al. J Wildl Dis. 2009 Jan;45(1):144-52.
7. FDo H. Florida charts: Hansen’s Disease (Leprosy). Health FDo. 2019. https://www.flhealthcharts.gov/ChartsReports/rdPage.aspx?rdReport=NonVitalIndNoGrpCounts.DataViewer&cid=174.
8. Maymone MBC et al. J Am Acad Dermatol. 2020 Jul;83(1):1-14.
9. Scollard DM et al. Clin Microbiol Rev. 2006 Apr;19(2):338-81.
10. Domozych R et al. JAAD Case Rep. 2016 May 12;2(3):189-92.
Paucibacillary tuberculoid leprosy is characterized by few anesthetic hypo- or hyperpigmented lesions and can be accompanied by palpable peripheral nerve enlargements.
Tuberculoid leprosy presents histologically with epithelioid histiocytes with lymphocytes and Langhans giant cells. Neurotropic granulomas are also characteristic of tuberculoid leprosy. Fite staining allows for the identification of the acid-fast bacilli of M. leprae, which in some cases are quite few in number. The standard mycobacterium stain, Ziehl-Neelsen, is a good option for M. tuberculosis, but because of the relative weak mycolic acid coat of M. leprae, the Fite stain is more appropriate for identifying M. leprae.
Clinically, other than the presence of fewer than five hypoesthetic lesions that are either hypopigmented or erythematous, tuberculoid leprosy often presents with additional peripheral nerve involvement that manifests as numbness and tingling in hands and feet.1 This patient denied any tingling, weakness, or numbness, outside of the anesthetic lesion on his posterior upper arm.
The patient, born in the United States, had a remote history of military travel to Iraq, Kuwait, and the Philippines, but had not traveled internationally within the last 15 years, apart from a cruise to the Bahamas. He denied any known contact with individuals with similar lesions. He denied a history of contact with armadillos, but acknowledged that they are native to where he resides in central Florida, and that he had seen them in his yard.
Histopathological examination revealed an unremarkable epidermis with a superficial and deep perivascular, periadnexal, and perineural lymphohistiocytic infiltrate. Fite stain revealed rare rod-shaped organisms (Figure 2). These findings are consistent with a diagnosis of paucibacillary, tuberculoid leprosy.
The patient’s travel history to highly endemic areas (Middle East), as well as possible environmental contact with armadillos – including contact with soil that the armadillos occupied – could explain plausible modes of transmission. Following consultation with our infectious disease department and the National Hansen’s Disease Program, our patient began a planned course of therapy with 18 months of minocycline, rifampin, and moxifloxacin.
Human-to-human transmission of HD has been well documented; however, zoonotic transmission – specifically via the nine-banded armadillo (Dasypus novemcinctus) – serves as another suggested means of transmission, especially in the Southeastern United States.2-6 Travel to highly-endemic areas increases the risk of contracting HD, which may take up to 20 years following contact with the bacteria to manifest clinically.
While central Florida was previously thought to be a nonendemic area of disease, the incidence of the disease in this region has increased in recent years.7 Human-to-human transmission, which remains a concern with immigration from highly-endemic regions, occurs via long-term contact with nasal droplets of an infected person.8,9
Many patients in regions with very few cases of leprosy deny travel to other endemic regions and contact with infected people. Thus, zoonotic transmission remains a legitimate concern in the Southeastern United States – accounting, at least in part, for many of the non–human-transmitted cases of leprosy.2,10 We encourage clinicians to maintain a high level of clinical suspicion for leprosy when evaluating patients presenting with hypoesthetic cutaneous lesions and to obtain a travel history and to ask about armadillo exposure.
This case and the photos were submitted by Ms. Smith, from the University of South Florida, Tampa; Dr. Hatch and Dr. Sarriera-Lazaro, from the department of dermatology and cutaneous surgery, University of South Florida; and Dr. Turner and Dr. Beachkofsky, from the department of pathology and laboratory medicine at the James A. Haley Veterans’ Hospital, Tampa. Dr. Bilu Martin edited this case. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].
References
1. Leprosy (Hansen’s Disease), in: “Goldman’s Cecil Medicine,” 24th ed. (Philadelphia: W.B. Saunders, 2012: pp. 1950-4.
2. Sharma R et al. Emerg Infect Dis. 2015 Dec;21(12):2127-34.
3. Lane JE et al. J Am Acad Dermatol. 2006 Oct;55(4):714-6.
4. Clark BM et al. Am J Trop Med Hyg. 2008 Jun;78(6):962-7.
5. Bruce S et al. J Am Acad Dermatol. 2000 Aug;43(2 Pt 1):223-8.
6. Loughry WJ et al. J Wildl Dis. 2009 Jan;45(1):144-52.
7. FDo H. Florida charts: Hansen’s Disease (Leprosy). Health FDo. 2019. https://www.flhealthcharts.gov/ChartsReports/rdPage.aspx?rdReport=NonVitalIndNoGrpCounts.DataViewer&cid=174.
8. Maymone MBC et al. J Am Acad Dermatol. 2020 Jul;83(1):1-14.
9. Scollard DM et al. Clin Microbiol Rev. 2006 Apr;19(2):338-81.
10. Domozych R et al. JAAD Case Rep. 2016 May 12;2(3):189-92.
Paucibacillary tuberculoid leprosy is characterized by few anesthetic hypo- or hyperpigmented lesions and can be accompanied by palpable peripheral nerve enlargements.
Tuberculoid leprosy presents histologically with epithelioid histiocytes with lymphocytes and Langhans giant cells. Neurotropic granulomas are also characteristic of tuberculoid leprosy. Fite staining allows for the identification of the acid-fast bacilli of M. leprae, which in some cases are quite few in number. The standard mycobacterium stain, Ziehl-Neelsen, is a good option for M. tuberculosis, but because of the relative weak mycolic acid coat of M. leprae, the Fite stain is more appropriate for identifying M. leprae.
Clinically, other than the presence of fewer than five hypoesthetic lesions that are either hypopigmented or erythematous, tuberculoid leprosy often presents with additional peripheral nerve involvement that manifests as numbness and tingling in hands and feet.1 This patient denied any tingling, weakness, or numbness, outside of the anesthetic lesion on his posterior upper arm.
The patient, born in the United States, had a remote history of military travel to Iraq, Kuwait, and the Philippines, but had not traveled internationally within the last 15 years, apart from a cruise to the Bahamas. He denied any known contact with individuals with similar lesions. He denied a history of contact with armadillos, but acknowledged that they are native to where he resides in central Florida, and that he had seen them in his yard.
Histopathological examination revealed an unremarkable epidermis with a superficial and deep perivascular, periadnexal, and perineural lymphohistiocytic infiltrate. Fite stain revealed rare rod-shaped organisms (Figure 2). These findings are consistent with a diagnosis of paucibacillary, tuberculoid leprosy.
The patient’s travel history to highly endemic areas (Middle East), as well as possible environmental contact with armadillos – including contact with soil that the armadillos occupied – could explain plausible modes of transmission. Following consultation with our infectious disease department and the National Hansen’s Disease Program, our patient began a planned course of therapy with 18 months of minocycline, rifampin, and moxifloxacin.
Human-to-human transmission of HD has been well documented; however, zoonotic transmission – specifically via the nine-banded armadillo (Dasypus novemcinctus) – serves as another suggested means of transmission, especially in the Southeastern United States.2-6 Travel to highly-endemic areas increases the risk of contracting HD, which may take up to 20 years following contact with the bacteria to manifest clinically.
While central Florida was previously thought to be a nonendemic area of disease, the incidence of the disease in this region has increased in recent years.7 Human-to-human transmission, which remains a concern with immigration from highly-endemic regions, occurs via long-term contact with nasal droplets of an infected person.8,9
Many patients in regions with very few cases of leprosy deny travel to other endemic regions and contact with infected people. Thus, zoonotic transmission remains a legitimate concern in the Southeastern United States – accounting, at least in part, for many of the non–human-transmitted cases of leprosy.2,10 We encourage clinicians to maintain a high level of clinical suspicion for leprosy when evaluating patients presenting with hypoesthetic cutaneous lesions and to obtain a travel history and to ask about armadillo exposure.
This case and the photos were submitted by Ms. Smith, from the University of South Florida, Tampa; Dr. Hatch and Dr. Sarriera-Lazaro, from the department of dermatology and cutaneous surgery, University of South Florida; and Dr. Turner and Dr. Beachkofsky, from the department of pathology and laboratory medicine at the James A. Haley Veterans’ Hospital, Tampa. Dr. Bilu Martin edited this case. More diagnostic cases are available at mdedge.com/dermatology. To submit a case for possible publication, send an email to [email protected].
References
1. Leprosy (Hansen’s Disease), in: “Goldman’s Cecil Medicine,” 24th ed. (Philadelphia: W.B. Saunders, 2012: pp. 1950-4.
2. Sharma R et al. Emerg Infect Dis. 2015 Dec;21(12):2127-34.
3. Lane JE et al. J Am Acad Dermatol. 2006 Oct;55(4):714-6.
4. Clark BM et al. Am J Trop Med Hyg. 2008 Jun;78(6):962-7.
5. Bruce S et al. J Am Acad Dermatol. 2000 Aug;43(2 Pt 1):223-8.
6. Loughry WJ et al. J Wildl Dis. 2009 Jan;45(1):144-52.
7. FDo H. Florida charts: Hansen’s Disease (Leprosy). Health FDo. 2019. https://www.flhealthcharts.gov/ChartsReports/rdPage.aspx?rdReport=NonVitalIndNoGrpCounts.DataViewer&cid=174.
8. Maymone MBC et al. J Am Acad Dermatol. 2020 Jul;83(1):1-14.
9. Scollard DM et al. Clin Microbiol Rev. 2006 Apr;19(2):338-81.
10. Domozych R et al. JAAD Case Rep. 2016 May 12;2(3):189-92.
A 44-year-old White male presented with a 1½-year history of a progressive hypoesthetic annular, mildly hyperpigmented plaque on the left posterior upper arm.
He denied pruritus, pain, or systemic symptoms including weight loss, visual changes, cough, dyspnea, and abdominal pain. He also denied any paresthesia or weakness. On physical examination, there is a subtle, solitary 4-cm annular skin-colored thin plaque on the patient's left posterior upper arm (Figure 1).
Punch biopsy of the lesion was performed, and the histopathological findings are illustrated in Figure 2.
A 50-year-old woman with no significant history presented with erythematous, annular plaques, and papules on the dorsal hands and arms
. The prevalence and incidence is approximately 0.1%-0.4%. Although the condition is benign, it may be associated with more serious conditions such as HIV and malignancy. GA affects women more frequently than men but can affect any age group, although it most commonly presents in those ages 30 years and younger. While the exact etiology is unknown, GA has been most strongly associated with diabetes mellitus, hyperlipidemia, and autoimmune diseases.
The disease presents as localized, annular erythematous plaques and papules on the dorsal hands and feet in approximately 75% of cases. However, eruptions may appear on the trunk and extremities and can be categorized into patchy, generalized, interstitial, subcutaneous, or perforating subtypes. The lesions are often asymptomatic and typically not associated with any other symptoms.
The pathogenesis of GA is still under investigation, but recent studies suggest that a Th1-mediated dysregulation of the JAK-STAT pathway may contribute to the disease. Other hypotheses include a delayed hypersensitivity reaction or cell mediated immune response. The mechanism may be multifaceted, and epidemiologic research suggests a genetic predisposition in White individuals, but these findings may be associated with socioeconomic factors and disparities in health care.
GA presents on histology with palisading histiocytes surrounding focal collagen necrobiosis with mucin deposition. Tissue samples also display leukocytic infiltration of the dermis featuring multinucleated giant cells. There are defining features of the different subtypes, but focal collagen necrosis, the presence of histiocytes, and mucin deposition are consistent findings across all presentations.
GA lesions commonly regress on their own, but they tend to recur and can be functionally and visually unappealing to patients. The most common treatments for GA include topical corticosteroids, intralesional corticosteroid injections, and other anti-inflammatory drugs. These interventions can be administered in a variety of ways as the inflammation caused by GA exists on a spectrum, and less severe cases can be managed with topical or intralesional treatment. Systemic therapy may be necessary for severe and recalcitrant cases. Other interventions that have shown promise in smaller studies include phototherapy, hydroxychloroquine, and TNF-alpha inhibitors.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, 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
Joshi TP and Duvic M. Am J Clin Dermatol. 2022 Jan;23(1):37-50. doi: 10.1007/s40257-021-00636-1.
Muse M et al. Dermatol Online J. 2021 Apr 15;27(4):13030/qt0m50398n.
Schmieder SJ et al. Granuloma Annulare. NIH National Center for Biotechnology Information [Updated 2022 Nov 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan. 7.
. The prevalence and incidence is approximately 0.1%-0.4%. Although the condition is benign, it may be associated with more serious conditions such as HIV and malignancy. GA affects women more frequently than men but can affect any age group, although it most commonly presents in those ages 30 years and younger. While the exact etiology is unknown, GA has been most strongly associated with diabetes mellitus, hyperlipidemia, and autoimmune diseases.
The disease presents as localized, annular erythematous plaques and papules on the dorsal hands and feet in approximately 75% of cases. However, eruptions may appear on the trunk and extremities and can be categorized into patchy, generalized, interstitial, subcutaneous, or perforating subtypes. The lesions are often asymptomatic and typically not associated with any other symptoms.
The pathogenesis of GA is still under investigation, but recent studies suggest that a Th1-mediated dysregulation of the JAK-STAT pathway may contribute to the disease. Other hypotheses include a delayed hypersensitivity reaction or cell mediated immune response. The mechanism may be multifaceted, and epidemiologic research suggests a genetic predisposition in White individuals, but these findings may be associated with socioeconomic factors and disparities in health care.
GA presents on histology with palisading histiocytes surrounding focal collagen necrobiosis with mucin deposition. Tissue samples also display leukocytic infiltration of the dermis featuring multinucleated giant cells. There are defining features of the different subtypes, but focal collagen necrosis, the presence of histiocytes, and mucin deposition are consistent findings across all presentations.
GA lesions commonly regress on their own, but they tend to recur and can be functionally and visually unappealing to patients. The most common treatments for GA include topical corticosteroids, intralesional corticosteroid injections, and other anti-inflammatory drugs. These interventions can be administered in a variety of ways as the inflammation caused by GA exists on a spectrum, and less severe cases can be managed with topical or intralesional treatment. Systemic therapy may be necessary for severe and recalcitrant cases. Other interventions that have shown promise in smaller studies include phototherapy, hydroxychloroquine, and TNF-alpha inhibitors.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, 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
Joshi TP and Duvic M. Am J Clin Dermatol. 2022 Jan;23(1):37-50. doi: 10.1007/s40257-021-00636-1.
Muse M et al. Dermatol Online J. 2021 Apr 15;27(4):13030/qt0m50398n.
Schmieder SJ et al. Granuloma Annulare. NIH National Center for Biotechnology Information [Updated 2022 Nov 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan. 7.
. The prevalence and incidence is approximately 0.1%-0.4%. Although the condition is benign, it may be associated with more serious conditions such as HIV and malignancy. GA affects women more frequently than men but can affect any age group, although it most commonly presents in those ages 30 years and younger. While the exact etiology is unknown, GA has been most strongly associated with diabetes mellitus, hyperlipidemia, and autoimmune diseases.
The disease presents as localized, annular erythematous plaques and papules on the dorsal hands and feet in approximately 75% of cases. However, eruptions may appear on the trunk and extremities and can be categorized into patchy, generalized, interstitial, subcutaneous, or perforating subtypes. The lesions are often asymptomatic and typically not associated with any other symptoms.
The pathogenesis of GA is still under investigation, but recent studies suggest that a Th1-mediated dysregulation of the JAK-STAT pathway may contribute to the disease. Other hypotheses include a delayed hypersensitivity reaction or cell mediated immune response. The mechanism may be multifaceted, and epidemiologic research suggests a genetic predisposition in White individuals, but these findings may be associated with socioeconomic factors and disparities in health care.
GA presents on histology with palisading histiocytes surrounding focal collagen necrobiosis with mucin deposition. Tissue samples also display leukocytic infiltration of the dermis featuring multinucleated giant cells. There are defining features of the different subtypes, but focal collagen necrosis, the presence of histiocytes, and mucin deposition are consistent findings across all presentations.
GA lesions commonly regress on their own, but they tend to recur and can be functionally and visually unappealing to patients. The most common treatments for GA include topical corticosteroids, intralesional corticosteroid injections, and other anti-inflammatory drugs. These interventions can be administered in a variety of ways as the inflammation caused by GA exists on a spectrum, and less severe cases can be managed with topical or intralesional treatment. Systemic therapy may be necessary for severe and recalcitrant cases. Other interventions that have shown promise in smaller studies include phototherapy, hydroxychloroquine, and TNF-alpha inhibitors.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, 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
Joshi TP and Duvic M. Am J Clin Dermatol. 2022 Jan;23(1):37-50. doi: 10.1007/s40257-021-00636-1.
Muse M et al. Dermatol Online J. 2021 Apr 15;27(4):13030/qt0m50398n.
Schmieder SJ et al. Granuloma Annulare. NIH National Center for Biotechnology Information [Updated 2022 Nov 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan. 7.
A 9-year old female presented with 1 day of fever, fatigue, and sore throat
This condition typically presents in the setting of Streptococcus pyogenes pharyngitis, or strep throat, and is spread via mucosal transfer in close proximity such as classrooms and nurseries. The dermatologic symptoms are a result of the endotoxin produced by S. pyogenes, which is part of the group A Strep bacteria. Clinically, the presentation can be differentiated from an allergic eruption by its relation to acute pharyngitis, insidious onset, and lack of confluence of the lesions. Diagnosis is supported by a throat culture and rapid strep test, although a rapid test lacks reliability in older patients who are less commonly affected and likely to be carriers. First-line treatment is penicillin or amoxicillin, but first-generation cephalosporins, clindamycin, or erythromycin are sufficient if the patient is allergic to penicillins. Prognosis worsens as time between onset and treatment increases, but is overall excellent now with the introduction of antibiotics and improved hygiene.
Scarlet fever is among a list of many common childhood rashes, and it can be difficult to differentiate between these pathologies on clinical presentation. A few notable childhood dermatologic eruptions include erythema infectiosum (fifth disease), roseola (exanthema subitum or sixth disease), and measles. These cases can be distinguished clinically by the age of the patient, distribution, and quality of the symptoms. Laboratory testing may be used to confirm the diagnosis.
Erythema infectiosum is known as fifth disease or slapped-cheek rash because it commonly presents on the cheeks as a pink, maculopapular rash in a reticular pattern. The disease is caused by parvovirus B19 and is accompanied by low fever, malaise, headache, sore throat, and nausea, which precedes the erythematous rash. The facial rash appears first and is followed by patchy eruptions on the extremities. Appearance of the rash typically indicates the patient is no longer contagious, and patients are treated symptomatically with NSAIDs and antihistamines for associated pruritus.
Roseola infantum is commonly caused by human herpesvirus 6 and is usually found in children 3 years and younger. The defining symptom is a high fever, which is paired with a mild cough, runny nose, and diarrhea. A maculopapular rash appears after the fever subsides, starting centrally and spreading outward to the extremities. Although this rash is similar to measles, they can be differentiated by the order of onset. The rash caused by measles begins on the face and mouth (Koplik spots) and moves downward. Additionally, the patient appears generally healthy and the disease is self-limiting in roseola, while patients with measles will appear more ill and require further attention. Measles is caused by the measles virus of the genus Morbillivirus and is highly contagious. It is spread via respiratory route presenting with fever, cough, coryza, and conjunctivitis followed by the rash. Fortunately, the measles vaccine is in widespread use, so cases have declined over the years.
Our patient had a positive strep test. Influenza and coronavirus tests were negative. She was started on daily amoxicillin and the rash resolved within 2 days of taking the antibiotics.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University, Tampa, 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
Allmon A et al.. Am Fam Physician. 2015 Aug 1;92(3):211-6.
Moss WJ. Lancet. 2017 Dec 2;390(10111):2490-502.
Mullins TB and Krishnamurthy K. Roseola Infantum, in “StatPearls.” Treasure Islan, Fla.: StatPearls Publishing, 2022.
Pardo S and Perera TB. Scarlet Fever, in “StatPearls.” Treasure Island, Fla.: StatPearls Publishing, 2022.
This condition typically presents in the setting of Streptococcus pyogenes pharyngitis, or strep throat, and is spread via mucosal transfer in close proximity such as classrooms and nurseries. The dermatologic symptoms are a result of the endotoxin produced by S. pyogenes, which is part of the group A Strep bacteria. Clinically, the presentation can be differentiated from an allergic eruption by its relation to acute pharyngitis, insidious onset, and lack of confluence of the lesions. Diagnosis is supported by a throat culture and rapid strep test, although a rapid test lacks reliability in older patients who are less commonly affected and likely to be carriers. First-line treatment is penicillin or amoxicillin, but first-generation cephalosporins, clindamycin, or erythromycin are sufficient if the patient is allergic to penicillins. Prognosis worsens as time between onset and treatment increases, but is overall excellent now with the introduction of antibiotics and improved hygiene.
Scarlet fever is among a list of many common childhood rashes, and it can be difficult to differentiate between these pathologies on clinical presentation. A few notable childhood dermatologic eruptions include erythema infectiosum (fifth disease), roseola (exanthema subitum or sixth disease), and measles. These cases can be distinguished clinically by the age of the patient, distribution, and quality of the symptoms. Laboratory testing may be used to confirm the diagnosis.
Erythema infectiosum is known as fifth disease or slapped-cheek rash because it commonly presents on the cheeks as a pink, maculopapular rash in a reticular pattern. The disease is caused by parvovirus B19 and is accompanied by low fever, malaise, headache, sore throat, and nausea, which precedes the erythematous rash. The facial rash appears first and is followed by patchy eruptions on the extremities. Appearance of the rash typically indicates the patient is no longer contagious, and patients are treated symptomatically with NSAIDs and antihistamines for associated pruritus.
Roseola infantum is commonly caused by human herpesvirus 6 and is usually found in children 3 years and younger. The defining symptom is a high fever, which is paired with a mild cough, runny nose, and diarrhea. A maculopapular rash appears after the fever subsides, starting centrally and spreading outward to the extremities. Although this rash is similar to measles, they can be differentiated by the order of onset. The rash caused by measles begins on the face and mouth (Koplik spots) and moves downward. Additionally, the patient appears generally healthy and the disease is self-limiting in roseola, while patients with measles will appear more ill and require further attention. Measles is caused by the measles virus of the genus Morbillivirus and is highly contagious. It is spread via respiratory route presenting with fever, cough, coryza, and conjunctivitis followed by the rash. Fortunately, the measles vaccine is in widespread use, so cases have declined over the years.
Our patient had a positive strep test. Influenza and coronavirus tests were negative. She was started on daily amoxicillin and the rash resolved within 2 days of taking the antibiotics.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University, Tampa, 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
Allmon A et al.. Am Fam Physician. 2015 Aug 1;92(3):211-6.
Moss WJ. Lancet. 2017 Dec 2;390(10111):2490-502.
Mullins TB and Krishnamurthy K. Roseola Infantum, in “StatPearls.” Treasure Islan, Fla.: StatPearls Publishing, 2022.
Pardo S and Perera TB. Scarlet Fever, in “StatPearls.” Treasure Island, Fla.: StatPearls Publishing, 2022.
This condition typically presents in the setting of Streptococcus pyogenes pharyngitis, or strep throat, and is spread via mucosal transfer in close proximity such as classrooms and nurseries. The dermatologic symptoms are a result of the endotoxin produced by S. pyogenes, which is part of the group A Strep bacteria. Clinically, the presentation can be differentiated from an allergic eruption by its relation to acute pharyngitis, insidious onset, and lack of confluence of the lesions. Diagnosis is supported by a throat culture and rapid strep test, although a rapid test lacks reliability in older patients who are less commonly affected and likely to be carriers. First-line treatment is penicillin or amoxicillin, but first-generation cephalosporins, clindamycin, or erythromycin are sufficient if the patient is allergic to penicillins. Prognosis worsens as time between onset and treatment increases, but is overall excellent now with the introduction of antibiotics and improved hygiene.
Scarlet fever is among a list of many common childhood rashes, and it can be difficult to differentiate between these pathologies on clinical presentation. A few notable childhood dermatologic eruptions include erythema infectiosum (fifth disease), roseola (exanthema subitum or sixth disease), and measles. These cases can be distinguished clinically by the age of the patient, distribution, and quality of the symptoms. Laboratory testing may be used to confirm the diagnosis.
Erythema infectiosum is known as fifth disease or slapped-cheek rash because it commonly presents on the cheeks as a pink, maculopapular rash in a reticular pattern. The disease is caused by parvovirus B19 and is accompanied by low fever, malaise, headache, sore throat, and nausea, which precedes the erythematous rash. The facial rash appears first and is followed by patchy eruptions on the extremities. Appearance of the rash typically indicates the patient is no longer contagious, and patients are treated symptomatically with NSAIDs and antihistamines for associated pruritus.
Roseola infantum is commonly caused by human herpesvirus 6 and is usually found in children 3 years and younger. The defining symptom is a high fever, which is paired with a mild cough, runny nose, and diarrhea. A maculopapular rash appears after the fever subsides, starting centrally and spreading outward to the extremities. Although this rash is similar to measles, they can be differentiated by the order of onset. The rash caused by measles begins on the face and mouth (Koplik spots) and moves downward. Additionally, the patient appears generally healthy and the disease is self-limiting in roseola, while patients with measles will appear more ill and require further attention. Measles is caused by the measles virus of the genus Morbillivirus and is highly contagious. It is spread via respiratory route presenting with fever, cough, coryza, and conjunctivitis followed by the rash. Fortunately, the measles vaccine is in widespread use, so cases have declined over the years.
Our patient had a positive strep test. Influenza and coronavirus tests were negative. She was started on daily amoxicillin and the rash resolved within 2 days of taking the antibiotics.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University, Tampa, 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
Allmon A et al.. Am Fam Physician. 2015 Aug 1;92(3):211-6.
Moss WJ. Lancet. 2017 Dec 2;390(10111):2490-502.
Mullins TB and Krishnamurthy K. Roseola Infantum, in “StatPearls.” Treasure Islan, Fla.: StatPearls Publishing, 2022.
Pardo S and Perera TB. Scarlet Fever, in “StatPearls.” Treasure Island, Fla.: StatPearls Publishing, 2022.
A 95-year-old White male with hypertension presented with itchy patches and bullae on the trunk and extremities
and is associated with various predisposing factors, including HLA genes, comorbidities, aging, and trigger factors such as drugs, trauma, radiation, chemotherapy, and infections. The autoimmune reaction is mediated by a dysregulation of T cells in which IgG and IgE autoantibodies form against hemidesmosomal proteins (BP180 and BP230). These autoantibodies induce neutrophil activation, recruitment, and degradation in the basement membrane of the skin.
Typically, patients present with intense pruritus followed by an urticarial or eczematous eruption. Tense blisters and bullae occur commonly on the trunk and extremities. Drug-associated bullous pemphigoid (DABP) is a common manifestation of the disease with histologic and immunologic features similar to those of the idiopathic version. Eruptions can be triggered by systemic or topical medications, and incidence of these reactions may be related to a genetic predisposition for the disease.
Some research suggests that drug-induced changes to the antigenic properties of the epidermal basement membrane result in an augmented immune response, while others point to structural modification in these zones that stimulate the immune system. Thiol- and phenol-based drugs have been largely implicated in the development of DABP because they are capable of structural modification and disruption of the dermo-epidermal junction in the basement membrane.
DABP often presents with patients taking multiple medications. Some of the most common medications are gliptins, PD-1 inhibitors, diuretics, antibiotics, anti-inflammatory drugs, and ACE-inhibitors, and other cardiovascular drugs. DABP may present with mucosal eruptions unlike its idiopathic counterpart that is mostly contained to the skin.
On this patient, two punch biopsies were taken. Histopathology revealed an eosinophil-rich subepidermal blister with a smooth epidermal undersurface consistent with bullous pemphigoid. Direct immunofluorescence was positive with a deposition of IgG and C3 at the epidermal side of salt split basement membrane zone.
Treatment for BP includes high potency topical and systemic steroids. Tetracyclines and niacinamide have been reported to improve the condition. Treatment is tailored to allow for cutaneous healing and control pruritus, but the physician must be mindful of the patient’s comorbidities and capacity for self-care. Prognosis is often better for DABP as withdrawal of the medication greatly accelerates clearance of the lesions. Worse prognosis is related to increased number of comorbidities and older age. Our patient’s BP is controlled currently with topical steroids and oral doxycycline.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, Tampa, 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
1. Miyamoto D et al. An Bras Dermatol. 2019 Mar-Apr;94(2):133-46.
2. Moro et al. Biomolecules. 2020 Oct 10;10(10):1432.
3. Verheyden M et al. Acta Derm Venereol. 2020 Aug 17;100(15):adv00224.
and is associated with various predisposing factors, including HLA genes, comorbidities, aging, and trigger factors such as drugs, trauma, radiation, chemotherapy, and infections. The autoimmune reaction is mediated by a dysregulation of T cells in which IgG and IgE autoantibodies form against hemidesmosomal proteins (BP180 and BP230). These autoantibodies induce neutrophil activation, recruitment, and degradation in the basement membrane of the skin.
Typically, patients present with intense pruritus followed by an urticarial or eczematous eruption. Tense blisters and bullae occur commonly on the trunk and extremities. Drug-associated bullous pemphigoid (DABP) is a common manifestation of the disease with histologic and immunologic features similar to those of the idiopathic version. Eruptions can be triggered by systemic or topical medications, and incidence of these reactions may be related to a genetic predisposition for the disease.
Some research suggests that drug-induced changes to the antigenic properties of the epidermal basement membrane result in an augmented immune response, while others point to structural modification in these zones that stimulate the immune system. Thiol- and phenol-based drugs have been largely implicated in the development of DABP because they are capable of structural modification and disruption of the dermo-epidermal junction in the basement membrane.
DABP often presents with patients taking multiple medications. Some of the most common medications are gliptins, PD-1 inhibitors, diuretics, antibiotics, anti-inflammatory drugs, and ACE-inhibitors, and other cardiovascular drugs. DABP may present with mucosal eruptions unlike its idiopathic counterpart that is mostly contained to the skin.
On this patient, two punch biopsies were taken. Histopathology revealed an eosinophil-rich subepidermal blister with a smooth epidermal undersurface consistent with bullous pemphigoid. Direct immunofluorescence was positive with a deposition of IgG and C3 at the epidermal side of salt split basement membrane zone.
Treatment for BP includes high potency topical and systemic steroids. Tetracyclines and niacinamide have been reported to improve the condition. Treatment is tailored to allow for cutaneous healing and control pruritus, but the physician must be mindful of the patient’s comorbidities and capacity for self-care. Prognosis is often better for DABP as withdrawal of the medication greatly accelerates clearance of the lesions. Worse prognosis is related to increased number of comorbidities and older age. Our patient’s BP is controlled currently with topical steroids and oral doxycycline.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, Tampa, 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
1. Miyamoto D et al. An Bras Dermatol. 2019 Mar-Apr;94(2):133-46.
2. Moro et al. Biomolecules. 2020 Oct 10;10(10):1432.
3. Verheyden M et al. Acta Derm Venereol. 2020 Aug 17;100(15):adv00224.
and is associated with various predisposing factors, including HLA genes, comorbidities, aging, and trigger factors such as drugs, trauma, radiation, chemotherapy, and infections. The autoimmune reaction is mediated by a dysregulation of T cells in which IgG and IgE autoantibodies form against hemidesmosomal proteins (BP180 and BP230). These autoantibodies induce neutrophil activation, recruitment, and degradation in the basement membrane of the skin.
Typically, patients present with intense pruritus followed by an urticarial or eczematous eruption. Tense blisters and bullae occur commonly on the trunk and extremities. Drug-associated bullous pemphigoid (DABP) is a common manifestation of the disease with histologic and immunologic features similar to those of the idiopathic version. Eruptions can be triggered by systemic or topical medications, and incidence of these reactions may be related to a genetic predisposition for the disease.
Some research suggests that drug-induced changes to the antigenic properties of the epidermal basement membrane result in an augmented immune response, while others point to structural modification in these zones that stimulate the immune system. Thiol- and phenol-based drugs have been largely implicated in the development of DABP because they are capable of structural modification and disruption of the dermo-epidermal junction in the basement membrane.
DABP often presents with patients taking multiple medications. Some of the most common medications are gliptins, PD-1 inhibitors, diuretics, antibiotics, anti-inflammatory drugs, and ACE-inhibitors, and other cardiovascular drugs. DABP may present with mucosal eruptions unlike its idiopathic counterpart that is mostly contained to the skin.
On this patient, two punch biopsies were taken. Histopathology revealed an eosinophil-rich subepidermal blister with a smooth epidermal undersurface consistent with bullous pemphigoid. Direct immunofluorescence was positive with a deposition of IgG and C3 at the epidermal side of salt split basement membrane zone.
Treatment for BP includes high potency topical and systemic steroids. Tetracyclines and niacinamide have been reported to improve the condition. Treatment is tailored to allow for cutaneous healing and control pruritus, but the physician must be mindful of the patient’s comorbidities and capacity for self-care. Prognosis is often better for DABP as withdrawal of the medication greatly accelerates clearance of the lesions. Worse prognosis is related to increased number of comorbidities and older age. Our patient’s BP is controlled currently with topical steroids and oral doxycycline.
This case and photo were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, Tampa, 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
1. Miyamoto D et al. An Bras Dermatol. 2019 Mar-Apr;94(2):133-46.
2. Moro et al. Biomolecules. 2020 Oct 10;10(10):1432.
3. Verheyden M et al. Acta Derm Venereol. 2020 Aug 17;100(15):adv00224.
A 95-year-old White male with hypertension presented with a history of very itchy patches and bullae on the trunk and extremities.
A White female presented with pustules and erythematous macules on the left palm
Psoriasis is an immune-mediated chronic inflammatory disease characterized by well-demarcated, scaly, erythematous plaques. Those who present with the condition often have a family history, which supports recent research uncovering various genes implicated in its pathogenesis. The disease is also associated with other systemic complications, most notably cardiovascular disease.
This condition is found in a small percentage of patients with psoriasis and presentation varies from hyperkeratotic plaques to pustular lesions. The pustular form is known as palmoplantar pustulosis and is within the spectrum of palmoplantar psoriasis.
Psoriasis is typically a clinical diagnosis and its severity can be measured using the Psoriasis Area and Severity Index. If biopsy is performed, the histology demonstrates parakeratosis, orthokeratosis, loss of the stratum granulosum, and dilated vasculature with an inflammatory cell infiltrate. The keratinocytes present with abnormal differentiation and hyperplasia, and the presence of foci of neutrophils known as “Munro’s microabscesses” in the stratum corneum serve as the hallmark of histological diagnosis. However, it is important to note that appearance can vary based on the stage of the lesion and the subtype of psoriasis present.
Palmoplantar psoriasis can be especially limiting and difficult to treat because of its distribution. Topical steroids, topical vitamin D analogues, and narrow band ultraviolet light therapy can be effective for less severe cases. Methotrexate, biologic treatments, and apremilast can be used for more extensive disease.
This patient is HLA-B27 positive and has uveitis. The presence of the HLA-B27 allele has been associated with inflammatory bowel disease, uveitis, psoriatic arthritis, and reactive arthritis. It has also been reported to be associated with pustular psoriasis. She responded well to topical steroids and vitamin D analogues.
This case and photo were submitted by Mr. Shapiro at Nova Southeastern University College of Osteopathic Medicine, Davie, Fla., 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
1. Psoriasis: Overview and Diagnosis, in “Evidence-Based Psoriasis. Updates in Clinical Dermatology.” (Cham, Switzerland: Springer International, 2018).
2. Merola JF et al. Dermatol Ther. 2018 May;31(3):e12589.
3. Chung J et al. J Am Acad Dermatol. 2014 Oct;71(4):623-32.
Psoriasis is an immune-mediated chronic inflammatory disease characterized by well-demarcated, scaly, erythematous plaques. Those who present with the condition often have a family history, which supports recent research uncovering various genes implicated in its pathogenesis. The disease is also associated with other systemic complications, most notably cardiovascular disease.
This condition is found in a small percentage of patients with psoriasis and presentation varies from hyperkeratotic plaques to pustular lesions. The pustular form is known as palmoplantar pustulosis and is within the spectrum of palmoplantar psoriasis.
Psoriasis is typically a clinical diagnosis and its severity can be measured using the Psoriasis Area and Severity Index. If biopsy is performed, the histology demonstrates parakeratosis, orthokeratosis, loss of the stratum granulosum, and dilated vasculature with an inflammatory cell infiltrate. The keratinocytes present with abnormal differentiation and hyperplasia, and the presence of foci of neutrophils known as “Munro’s microabscesses” in the stratum corneum serve as the hallmark of histological diagnosis. However, it is important to note that appearance can vary based on the stage of the lesion and the subtype of psoriasis present.
Palmoplantar psoriasis can be especially limiting and difficult to treat because of its distribution. Topical steroids, topical vitamin D analogues, and narrow band ultraviolet light therapy can be effective for less severe cases. Methotrexate, biologic treatments, and apremilast can be used for more extensive disease.
This patient is HLA-B27 positive and has uveitis. The presence of the HLA-B27 allele has been associated with inflammatory bowel disease, uveitis, psoriatic arthritis, and reactive arthritis. It has also been reported to be associated with pustular psoriasis. She responded well to topical steroids and vitamin D analogues.
This case and photo were submitted by Mr. Shapiro at Nova Southeastern University College of Osteopathic Medicine, Davie, Fla., 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
1. Psoriasis: Overview and Diagnosis, in “Evidence-Based Psoriasis. Updates in Clinical Dermatology.” (Cham, Switzerland: Springer International, 2018).
2. Merola JF et al. Dermatol Ther. 2018 May;31(3):e12589.
3. Chung J et al. J Am Acad Dermatol. 2014 Oct;71(4):623-32.
Psoriasis is an immune-mediated chronic inflammatory disease characterized by well-demarcated, scaly, erythematous plaques. Those who present with the condition often have a family history, which supports recent research uncovering various genes implicated in its pathogenesis. The disease is also associated with other systemic complications, most notably cardiovascular disease.
This condition is found in a small percentage of patients with psoriasis and presentation varies from hyperkeratotic plaques to pustular lesions. The pustular form is known as palmoplantar pustulosis and is within the spectrum of palmoplantar psoriasis.
Psoriasis is typically a clinical diagnosis and its severity can be measured using the Psoriasis Area and Severity Index. If biopsy is performed, the histology demonstrates parakeratosis, orthokeratosis, loss of the stratum granulosum, and dilated vasculature with an inflammatory cell infiltrate. The keratinocytes present with abnormal differentiation and hyperplasia, and the presence of foci of neutrophils known as “Munro’s microabscesses” in the stratum corneum serve as the hallmark of histological diagnosis. However, it is important to note that appearance can vary based on the stage of the lesion and the subtype of psoriasis present.
Palmoplantar psoriasis can be especially limiting and difficult to treat because of its distribution. Topical steroids, topical vitamin D analogues, and narrow band ultraviolet light therapy can be effective for less severe cases. Methotrexate, biologic treatments, and apremilast can be used for more extensive disease.
This patient is HLA-B27 positive and has uveitis. The presence of the HLA-B27 allele has been associated with inflammatory bowel disease, uveitis, psoriatic arthritis, and reactive arthritis. It has also been reported to be associated with pustular psoriasis. She responded well to topical steroids and vitamin D analogues.
This case and photo were submitted by Mr. Shapiro at Nova Southeastern University College of Osteopathic Medicine, Davie, Fla., 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
1. Psoriasis: Overview and Diagnosis, in “Evidence-Based Psoriasis. Updates in Clinical Dermatology.” (Cham, Switzerland: Springer International, 2018).
2. Merola JF et al. Dermatol Ther. 2018 May;31(3):e12589.
3. Chung J et al. J Am Acad Dermatol. 2014 Oct;71(4):623-32.
A White male presented with a 1-month history of recurrent, widespread, painful sores
Coinfection of staphylococci and streptococci can make it more challenging to treat. Lesions typically begin as a vesicle that enlarges and forms an ulcer with a hemorrhagic crust. Even with treatment, the depth of the lesions may result in scarring. Shins and dorsal feet are nearly always involved. Systemic involvement is rare.
Open wounds, bites, or dermatoses are risk factors for the development of ecthyma. Additionally, poor hygiene and malnutrition play a major role in inoculation and severity of the disease. Poor hygiene may serve as the initiating factor for infection, but malnutrition permits further development because of the body’s inability to mount a sufficient immune response. Intravenous drug users and patients with HIV tend to be affected.
When diagnosing ecthyma, it is important to correlate clinical signs with a bacterial culture. This condition can be difficult to treat because of both coinfection and growing antibiotic resistance in staphylococcal and streptococcal species. Specifically, S. aureus has been found to be resistant to beta-lactam antibiotics for many years, with methicillin-resistant S. aureus (MRSA) being first detected in 1961. While a variety of antibiotics are indicated, the prescription should be tailored to cover the cultured organism.
Topical antibiotics are sufficient for more superficial lesions. Both topical and oral antibiotics may be recommended for ecthyma as the infection can spread more deeply into the skin, eventually causing a cellulitis. Treatment protocol for oral agents varies based on which drug is indicated. This patient was seen in the emergency room. His white blood cell count was elevated at 9 × 109/L. He was started empirically on amoxicillin/clavulanate (Augmentin) and ciprofloxacin. Bacterial cultures grew out Streptococcus pyogenes.
The case and photos were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Susannah Berke, MD, Three Rivers Dermatology, Coraopolis, Pa. Dr. Bilu Martin 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. Kwak Y et al. Infect Chemother. 2017 Dec;49(4):301-25.
2. Pereira LB. An Bras Dermatol. 2014 Mar-Apr;89(2):293-9.
3. Wasserzug O et al. Clin Infect Dis. 2009 May 1;48(9):1213-9.
Coinfection of staphylococci and streptococci can make it more challenging to treat. Lesions typically begin as a vesicle that enlarges and forms an ulcer with a hemorrhagic crust. Even with treatment, the depth of the lesions may result in scarring. Shins and dorsal feet are nearly always involved. Systemic involvement is rare.
Open wounds, bites, or dermatoses are risk factors for the development of ecthyma. Additionally, poor hygiene and malnutrition play a major role in inoculation and severity of the disease. Poor hygiene may serve as the initiating factor for infection, but malnutrition permits further development because of the body’s inability to mount a sufficient immune response. Intravenous drug users and patients with HIV tend to be affected.
When diagnosing ecthyma, it is important to correlate clinical signs with a bacterial culture. This condition can be difficult to treat because of both coinfection and growing antibiotic resistance in staphylococcal and streptococcal species. Specifically, S. aureus has been found to be resistant to beta-lactam antibiotics for many years, with methicillin-resistant S. aureus (MRSA) being first detected in 1961. While a variety of antibiotics are indicated, the prescription should be tailored to cover the cultured organism.
Topical antibiotics are sufficient for more superficial lesions. Both topical and oral antibiotics may be recommended for ecthyma as the infection can spread more deeply into the skin, eventually causing a cellulitis. Treatment protocol for oral agents varies based on which drug is indicated. This patient was seen in the emergency room. His white blood cell count was elevated at 9 × 109/L. He was started empirically on amoxicillin/clavulanate (Augmentin) and ciprofloxacin. Bacterial cultures grew out Streptococcus pyogenes.
The case and photos were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Susannah Berke, MD, Three Rivers Dermatology, Coraopolis, Pa. Dr. Bilu Martin 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. Kwak Y et al. Infect Chemother. 2017 Dec;49(4):301-25.
2. Pereira LB. An Bras Dermatol. 2014 Mar-Apr;89(2):293-9.
3. Wasserzug O et al. Clin Infect Dis. 2009 May 1;48(9):1213-9.
Coinfection of staphylococci and streptococci can make it more challenging to treat. Lesions typically begin as a vesicle that enlarges and forms an ulcer with a hemorrhagic crust. Even with treatment, the depth of the lesions may result in scarring. Shins and dorsal feet are nearly always involved. Systemic involvement is rare.
Open wounds, bites, or dermatoses are risk factors for the development of ecthyma. Additionally, poor hygiene and malnutrition play a major role in inoculation and severity of the disease. Poor hygiene may serve as the initiating factor for infection, but malnutrition permits further development because of the body’s inability to mount a sufficient immune response. Intravenous drug users and patients with HIV tend to be affected.
When diagnosing ecthyma, it is important to correlate clinical signs with a bacterial culture. This condition can be difficult to treat because of both coinfection and growing antibiotic resistance in staphylococcal and streptococcal species. Specifically, S. aureus has been found to be resistant to beta-lactam antibiotics for many years, with methicillin-resistant S. aureus (MRSA) being first detected in 1961. While a variety of antibiotics are indicated, the prescription should be tailored to cover the cultured organism.
Topical antibiotics are sufficient for more superficial lesions. Both topical and oral antibiotics may be recommended for ecthyma as the infection can spread more deeply into the skin, eventually causing a cellulitis. Treatment protocol for oral agents varies based on which drug is indicated. This patient was seen in the emergency room. His white blood cell count was elevated at 9 × 109/L. He was started empirically on amoxicillin/clavulanate (Augmentin) and ciprofloxacin. Bacterial cultures grew out Streptococcus pyogenes.
The case and photos were submitted by Lucas Shapiro, BS, Nova Southeastern University College of Osteopathic Medicine, Fort Lauderdale, Fla., and Susannah Berke, MD, Three Rivers Dermatology, Coraopolis, Pa. Dr. Bilu Martin 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. Kwak Y et al. Infect Chemother. 2017 Dec;49(4):301-25.
2. Pereira LB. An Bras Dermatol. 2014 Mar-Apr;89(2):293-9.
3. Wasserzug O et al. Clin Infect Dis. 2009 May 1;48(9):1213-9.
A White female presented with pruritic, reticulated, erythematous plaques on the abdomen
It is characterized by pruritic, erythematous papules, papulovesicles, and vesicles that appear in a reticular pattern, most commonly on the trunk. The lesions are typically followed by postinflammatory hyperpigmentation (PIH).
Although PP has been described in people of all races, ages, and sexes, it is predominantly observed in Japan, often in female young adults. Triggers may include a ketogenic diet, diabetes mellitus, and pregnancy. Friction and contact allergic reactions to chrome or nickel have been proposed as exogenous trigger factors. Individual cases of Sjögren’s syndrome, Helicobacter pylori infections, and adult Still syndrome have also been associated with recurrent eruptions.
The diagnosis of PP is made both clinically and by biopsy. The histological features vary according to the stage of the disease. In early-stage disease, superficial and perivascular infiltration of neutrophils are prominent. Later stages are characterized by spongiosis and necrotic keratinocytes.
The first-line therapy for prurigo pigmentosa is oral minocycline. However, for some patients, doxycycline, macrolide antibiotics, or dapsone may be indicated. Adding carbohydrates to a keto diet may be helpful. In this patient, a punch biopsy was performed, which revealed an interface dermatitis with eosinophils and neutrophils, consistent with prurigo pigmentosa. The cause of her PP remains idiopathic. She was treated with 100 mg doxycycline twice a day, which resulted in a resolution of active lesions. The patient did have postinflammatory hyperpigmentation.
This case and photo were submitted by Brooke Resh Sateesh, MD, of San Diego Family Dermatology, San Diego, California, and Mina Zulal, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany. Dr. Bilu Martin 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. Beutler et al. Am J Clin Dermatol. 2015 Dec;16(6):533-43.
2. Kim et al. J Dermatol. 2012 Nov;39(11):891-7.
3. Mufti et al. JAAD Int. 2021 Apr 10;3:79-87.
It is characterized by pruritic, erythematous papules, papulovesicles, and vesicles that appear in a reticular pattern, most commonly on the trunk. The lesions are typically followed by postinflammatory hyperpigmentation (PIH).
Although PP has been described in people of all races, ages, and sexes, it is predominantly observed in Japan, often in female young adults. Triggers may include a ketogenic diet, diabetes mellitus, and pregnancy. Friction and contact allergic reactions to chrome or nickel have been proposed as exogenous trigger factors. Individual cases of Sjögren’s syndrome, Helicobacter pylori infections, and adult Still syndrome have also been associated with recurrent eruptions.
The diagnosis of PP is made both clinically and by biopsy. The histological features vary according to the stage of the disease. In early-stage disease, superficial and perivascular infiltration of neutrophils are prominent. Later stages are characterized by spongiosis and necrotic keratinocytes.
The first-line therapy for prurigo pigmentosa is oral minocycline. However, for some patients, doxycycline, macrolide antibiotics, or dapsone may be indicated. Adding carbohydrates to a keto diet may be helpful. In this patient, a punch biopsy was performed, which revealed an interface dermatitis with eosinophils and neutrophils, consistent with prurigo pigmentosa. The cause of her PP remains idiopathic. She was treated with 100 mg doxycycline twice a day, which resulted in a resolution of active lesions. The patient did have postinflammatory hyperpigmentation.
This case and photo were submitted by Brooke Resh Sateesh, MD, of San Diego Family Dermatology, San Diego, California, and Mina Zulal, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany. Dr. Bilu Martin 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. Beutler et al. Am J Clin Dermatol. 2015 Dec;16(6):533-43.
2. Kim et al. J Dermatol. 2012 Nov;39(11):891-7.
3. Mufti et al. JAAD Int. 2021 Apr 10;3:79-87.
It is characterized by pruritic, erythematous papules, papulovesicles, and vesicles that appear in a reticular pattern, most commonly on the trunk. The lesions are typically followed by postinflammatory hyperpigmentation (PIH).
Although PP has been described in people of all races, ages, and sexes, it is predominantly observed in Japan, often in female young adults. Triggers may include a ketogenic diet, diabetes mellitus, and pregnancy. Friction and contact allergic reactions to chrome or nickel have been proposed as exogenous trigger factors. Individual cases of Sjögren’s syndrome, Helicobacter pylori infections, and adult Still syndrome have also been associated with recurrent eruptions.
The diagnosis of PP is made both clinically and by biopsy. The histological features vary according to the stage of the disease. In early-stage disease, superficial and perivascular infiltration of neutrophils are prominent. Later stages are characterized by spongiosis and necrotic keratinocytes.
The first-line therapy for prurigo pigmentosa is oral minocycline. However, for some patients, doxycycline, macrolide antibiotics, or dapsone may be indicated. Adding carbohydrates to a keto diet may be helpful. In this patient, a punch biopsy was performed, which revealed an interface dermatitis with eosinophils and neutrophils, consistent with prurigo pigmentosa. The cause of her PP remains idiopathic. She was treated with 100 mg doxycycline twice a day, which resulted in a resolution of active lesions. The patient did have postinflammatory hyperpigmentation.
This case and photo were submitted by Brooke Resh Sateesh, MD, of San Diego Family Dermatology, San Diego, California, and Mina Zulal, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany. Dr. Bilu Martin 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. Beutler et al. Am J Clin Dermatol. 2015 Dec;16(6):533-43.
2. Kim et al. J Dermatol. 2012 Nov;39(11):891-7.
3. Mufti et al. JAAD Int. 2021 Apr 10;3:79-87.
A healthy White male presented with a rash consisting of erythematous to purpuric macules
Vasculitis is a process in which blood vessels become inflamed and necrotic. Classic small vessel vasculitis reveals a leukocytoclastic vasculitis and most commonly presents as palpable purpura. .” A form of EIV has been described in the literature as “Disney dermatitis.” It is often seen in healthy adults after a long day of walking at the parks. Other forms of exercise, such as jogging, hiking, or swimming, may also cause the condition.
Clinically, EIV affects the lower legs and presents as purpuric macules. Edema may be present. Lesions may be asymptomatic or may present with pruritus or burning. Diagnosis is often made clinically. Skin biopsies for H&E and DIF (direct immunofluorescence) can help distinguish the type of vasculitis that is present. Laboratory tests may be needed to exclude other causes of vasculitis. Episodes may be recurrent.
Henoch-Schönlein purpura (HSP), also called anaphylactoid purpura, is a subtype of small-vessel vasculitis where IgA immunoglobulin is deposited in the vessel walls. It is the most common form of vasculitis is children (usually ages 4-8). In addition to skin, organs such as joints, kidneys, and intestines can be involved. Schamberg’s disease, or capillaritis, is also called pigmented purpura. In this benign condition, leakage from capillaries results in erythematous to brown patches on the lower extremities. A true vasculitis is not seen. The brown discoloration is due to hemosiderin deposition. Cryoglobulinemia is a rare condition in which abnormal immunoglobulin complexes deposit in tissues and vessels. Leukocytoclastic vasculitis is present in small vessels. Palpable purpura and livedo may be seen clinically, and systemic symptoms may be present.
Treatment of EIV is largely supportive as lesions will resolve on their own over 3-4 weeks. Postinflammatory hyperpigmentation may result. Temporary cessation of exercise and compression stockings can help speed up the resolution of lesions. Systemic medications used in the treatment of severe vasculitis, such as systemic steroids, dapsone, and colchicine, are not needed in EIV.
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].
Vasculitis is a process in which blood vessels become inflamed and necrotic. Classic small vessel vasculitis reveals a leukocytoclastic vasculitis and most commonly presents as palpable purpura. .” A form of EIV has been described in the literature as “Disney dermatitis.” It is often seen in healthy adults after a long day of walking at the parks. Other forms of exercise, such as jogging, hiking, or swimming, may also cause the condition.
Clinically, EIV affects the lower legs and presents as purpuric macules. Edema may be present. Lesions may be asymptomatic or may present with pruritus or burning. Diagnosis is often made clinically. Skin biopsies for H&E and DIF (direct immunofluorescence) can help distinguish the type of vasculitis that is present. Laboratory tests may be needed to exclude other causes of vasculitis. Episodes may be recurrent.
Henoch-Schönlein purpura (HSP), also called anaphylactoid purpura, is a subtype of small-vessel vasculitis where IgA immunoglobulin is deposited in the vessel walls. It is the most common form of vasculitis is children (usually ages 4-8). In addition to skin, organs such as joints, kidneys, and intestines can be involved. Schamberg’s disease, or capillaritis, is also called pigmented purpura. In this benign condition, leakage from capillaries results in erythematous to brown patches on the lower extremities. A true vasculitis is not seen. The brown discoloration is due to hemosiderin deposition. Cryoglobulinemia is a rare condition in which abnormal immunoglobulin complexes deposit in tissues and vessels. Leukocytoclastic vasculitis is present in small vessels. Palpable purpura and livedo may be seen clinically, and systemic symptoms may be present.
Treatment of EIV is largely supportive as lesions will resolve on their own over 3-4 weeks. Postinflammatory hyperpigmentation may result. Temporary cessation of exercise and compression stockings can help speed up the resolution of lesions. Systemic medications used in the treatment of severe vasculitis, such as systemic steroids, dapsone, and colchicine, are not needed in EIV.
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].
Vasculitis is a process in which blood vessels become inflamed and necrotic. Classic small vessel vasculitis reveals a leukocytoclastic vasculitis and most commonly presents as palpable purpura. .” A form of EIV has been described in the literature as “Disney dermatitis.” It is often seen in healthy adults after a long day of walking at the parks. Other forms of exercise, such as jogging, hiking, or swimming, may also cause the condition.
Clinically, EIV affects the lower legs and presents as purpuric macules. Edema may be present. Lesions may be asymptomatic or may present with pruritus or burning. Diagnosis is often made clinically. Skin biopsies for H&E and DIF (direct immunofluorescence) can help distinguish the type of vasculitis that is present. Laboratory tests may be needed to exclude other causes of vasculitis. Episodes may be recurrent.
Henoch-Schönlein purpura (HSP), also called anaphylactoid purpura, is a subtype of small-vessel vasculitis where IgA immunoglobulin is deposited in the vessel walls. It is the most common form of vasculitis is children (usually ages 4-8). In addition to skin, organs such as joints, kidneys, and intestines can be involved. Schamberg’s disease, or capillaritis, is also called pigmented purpura. In this benign condition, leakage from capillaries results in erythematous to brown patches on the lower extremities. A true vasculitis is not seen. The brown discoloration is due to hemosiderin deposition. Cryoglobulinemia is a rare condition in which abnormal immunoglobulin complexes deposit in tissues and vessels. Leukocytoclastic vasculitis is present in small vessels. Palpable purpura and livedo may be seen clinically, and systemic symptoms may be present.
Treatment of EIV is largely supportive as lesions will resolve on their own over 3-4 weeks. Postinflammatory hyperpigmentation may result. Temporary cessation of exercise and compression stockings can help speed up the resolution of lesions. Systemic medications used in the treatment of severe vasculitis, such as systemic steroids, dapsone, and colchicine, are not needed in EIV.
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].
