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Extragenital lichen sclerosus: A series of seven cases from a third level hospital in Mexico City
4716
4315
Extensive facial cutaneous leishmaniasis treated successfully with liposomal amphotericin B Abdullah Abualiat, MD, Departments of Dermatology, King Fahad Armed Forces Hospital, Khamis Mushayt; Eman Alamer, MD, Departments of Dermatology, King Fahad Armed Forces Hospital, Khamis Mushayt; Tariq Aziz, MD, Departments of Dermatology, King Fahd Armed Forces Hospital; Esmat Eid, DDS, Departments of Dermatology, King Fahad Armed Forces Hospital, Khamis Mushayt Cutaneous leishmaniasis is a protozoal infection caused by Leishmania species. It is endemic in some parts of Saudi Arabia. The treatment often includes topical treatments and intralesional sodium stibogluconate but treatment failures are common. Liposomal amphotericin B, a drug approved for treatment for visceral leishmaniasis, has rarely been used for cutaneous leishmaniasis but recent studies have been found it to be efficacious. We report here a case of a 43-year-old male with extensive cutaneous leishmaniasis for 6 years involving 70% of the face. Leishmania smear was positive and confirmed by biopsy. Patient had received intralesional sodium stibogluconate without any improvement. The patient was then treated with intravenous Liposomal amphotericin B at a dose of 5 mg/kg/day. He received total 20 infusions and was clinically cured without any complications. Patient clinically clear at five months follow up with mild scar. Thus, liposomal amphotericin B, although expensive, should be considered when systemic treatment is needed for cutaneous leishmaniasis, especially in cases with multiple facial lesions or large extensive lesion.
Extensive necrotic lesions of atypical hemolytic uremic syndrome treated with eculizumab Maya Debbaneh, MD, Kaiser Permanente; Valentina Sosa, MD, Kaiser Permanente; David Cassarino, MD, Kaiser Permanente Atypical hemolytic uremic syndrome (aHUS) is an uncommon form of thrombotic microangiopathy. It has a poor outcome, with death rates as high as 25% and up to 50% of cases progressing to end-stage renal failure. It presents with microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Although dermatologic findings in aHUS is quite rare, we report a case of a 72year-old female presenting with extensive necrotic lesions our patient presented with extensive necrotic lesions found to have atypical HUS. After failing initial treatment with prednisone and plasmapheresis, she was started on eculizumab. This recombinant humanized monoclonal antibody directed against C5, blocks cleavage of C5 into C5a and C5b. The lesions resolved with residual scarring after 7 months of treatment. Commercial support: None identified.
Commercial support: None identified.
4772 Extensive mucocutaneous blastomycosis: A case report Julie Lecours, MD, Clinique de Dermatologie, Centre Hospitalier de l’Universite de Montreal; Deana Funaro, MD, Clinique de Dermatologie, Centre Hospitalier de l’Universite de Montreal; Melanie Baril, MD, H^ opital du Haut-Richelieu; Introduction: Blastomycosis is an infection caused by a dimorphic fungus, Blastomyces dermatitidis. The disease is endemic to the United States and Canada, particularly in areas close to water. This fungus lives in moist soils and in decomposing wood and leaves. We report a case of extensive mucocutaneous blastomycosis. Material and Methods: A 49-year-old carpenter and chronic cocaine user, without prior medical history, was investigated for numerous rapidly progressive skin lesions over 6 weeks. Histologic examination of mucosal and skin biopsy specimens, special stains (PASD and Grocott), cultures and PCR, serologies (hepatitis B and C, syphilis, HIV), serum and urine assay for B dermatitidis antigen and an investigation by gallium and CT scan were performed. Results: The patient described the initial lesions as pruritic erythematous papules. On examination, he presented with 25 impressively hyperkeratotic plaques with necrotic and warty centers and indurated erythematous/violaceous borders on his face, torso, arms and legs. A left frontotemporal lesion was partially ulcerated. A large wet, bourgeoning, infiltrated and painful lesion that exuded a fetid discharge involved his right calf. A week later, a 2-cm lesion appeared on his palate. An incisional biopsy taken from a lesion on the arm revealed florid pseudoepitheliomatous hyperplasia of the epidermis, suppurative and granulomatous inflammation in the dermis and budding yeast forms throughout. Special stains were suggestive of blastomycosis. The presence of B dermatitidis was confirmed by culture, PCR and antigen detection. Serologies and the imaging were negative. The patient was initially treated with liposomal amphotericin B for 2 weeks followed by itraconazole PO. He showed rapid improvement within a month and an almost complete resolution by 4 months. Discussion: This patient with extensive mucocutaneous blastomycosis did respond very well to treatment. The most likely hypothesis to explain the extent of his lesions is inhalation followed by hematogenous dissemination. Primary cutaneous blastomycosis caused by direct inoculation due to trauma seems unlikely. Carpenters in contact with wet wood are at risk. Conclusion: Faced with such lesions, a skin biopsy and adequate investigation are essential in order to eliminate a systemic fungal infection that could be fatal in an immunocompromised host.
4721 Extragenital lichen sclerosus: A series of seven cases from a third level hospital in Mexico City Karen Ferez-Blando, MD, Instituto Nacional de Ciencias medicas y Nutricion Salvador Zubiran Background: Lichen sclerosus (LS) is a chronic inflammatory mucocutaneous disease. Its etiology is multifactorial, including genetic, immunologic, infectious, traumatic and hormonal factors. It has a predilection for the anogenital region and rarely presents in the extragenital area. Methods: All cases of pathologically confirmed extragenital LS reported in our hospital from 1987 to 2016 were retrospectively reviewed. Results: Seven patients e all females e with extragenital LS were identified. The median age was 61 years and 50% of them had previously diagnosed autoimmune diseases (eg, Graves’ disease and type 1 diabetes). The lesions were located on the trunk, affecting the neck and abdomen. The most common morphology consisted of bright atrophic hypopigmented plaques. The dermatitis had a duration of 6 months on average and pruritus was the most common symptom (83%). Thirty percent resolved completely and the rest reported partial improvement. Conclusion: The clinical features observed in our study are consistent with the published literature, such as female predominance and age of presentation. We described the comorbidities of our patients, which interestingly shared type 2 diabetes, and autoimmune diseases including thyroid disease and type-1 diabetes. This may serve as a clue to explain LS’s potential autoimmune etiology.
Commercial support: None identified. Commercial support: None identified.
AB136
J AM ACAD DERMATOL
JUNE 2017
4315
Extensive facial cutaneous leishmaniasis treated successfully with liposomal amphotericin B Abdullah Abualiat, MD, Departments of Dermatology, King Fahad Armed Forces Hospital, Khamis Mushayt; Eman Alamer, MD, Departments of Dermatology, King Fahad Armed Forces Hospital, Khamis Mushayt; Tariq Aziz, MD, Departments of Dermatology, King Fahd Armed Forces Hospital; Esmat Eid, DDS, Departments of Dermatology, King Fahad Armed Forces Hospital, Khamis Mushayt Cutaneous leishmaniasis is a protozoal infection caused by Leishmania species. It is endemic in some parts of Saudi Arabia. The treatment often includes topical treatments and intralesional sodium stibogluconate but treatment failures are common. Liposomal amphotericin B, a drug approved for treatment for visceral leishmaniasis, has rarely been used for cutaneous leishmaniasis but recent studies have been found it to be efficacious. We report here a case of a 43-year-old male with extensive cutaneous leishmaniasis for 6 years involving 70% of the face. Leishmania smear was positive and confirmed by biopsy. Patient had received intralesional sodium stibogluconate without any improvement. The patient was then treated with intravenous Liposomal amphotericin B at a dose of 5 mg/kg/day. He received total 20 infusions and was clinically cured without any complications. Patient clinically clear at five months follow up with mild scar. Thus, liposomal amphotericin B, although expensive, should be considered when systemic treatment is needed for cutaneous leishmaniasis, especially in cases with multiple facial lesions or large extensive lesion.
Extensive necrotic lesions of atypical hemolytic uremic syndrome treated with eculizumab Maya Debbaneh, MD, Kaiser Permanente; Valentina Sosa, MD, Kaiser Permanente; David Cassarino, MD, Kaiser Permanente Atypical hemolytic uremic syndrome (aHUS) is an uncommon form of thrombotic microangiopathy. It has a poor outcome, with death rates as high as 25% and up to 50% of cases progressing to end-stage renal failure. It presents with microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Although dermatologic findings in aHUS is quite rare, we report a case of a 72year-old female presenting with extensive necrotic lesions our patient presented with extensive necrotic lesions found to have atypical HUS. After failing initial treatment with prednisone and plasmapheresis, she was started on eculizumab. This recombinant humanized monoclonal antibody directed against C5, blocks cleavage of C5 into C5a and C5b. The lesions resolved with residual scarring after 7 months of treatment. Commercial support: None identified.
Commercial support: None identified.
4772 Extensive mucocutaneous blastomycosis: A case report Julie Lecours, MD, Clinique de Dermatologie, Centre Hospitalier de l’Universite de Montreal; Deana Funaro, MD, Clinique de Dermatologie, Centre Hospitalier de l’Universite de Montreal; Melanie Baril, MD, H^ opital du Haut-Richelieu; Introduction: Blastomycosis is an infection caused by a dimorphic fungus, Blastomyces dermatitidis. The disease is endemic to the United States and Canada, particularly in areas close to water. This fungus lives in moist soils and in decomposing wood and leaves. We report a case of extensive mucocutaneous blastomycosis. Material and Methods: A 49-year-old carpenter and chronic cocaine user, without prior medical history, was investigated for numerous rapidly progressive skin lesions over 6 weeks. Histologic examination of mucosal and skin biopsy specimens, special stains (PASD and Grocott), cultures and PCR, serologies (hepatitis B and C, syphilis, HIV), serum and urine assay for B dermatitidis antigen and an investigation by gallium and CT scan were performed. Results: The patient described the initial lesions as pruritic erythematous papules. On examination, he presented with 25 impressively hyperkeratotic plaques with necrotic and warty centers and indurated erythematous/violaceous borders on his face, torso, arms and legs. A left frontotemporal lesion was partially ulcerated. A large wet, bourgeoning, infiltrated and painful lesion that exuded a fetid discharge involved his right calf. A week later, a 2-cm lesion appeared on his palate. An incisional biopsy taken from a lesion on the arm revealed florid pseudoepitheliomatous hyperplasia of the epidermis, suppurative and granulomatous inflammation in the dermis and budding yeast forms throughout. Special stains were suggestive of blastomycosis. The presence of B dermatitidis was confirmed by culture, PCR and antigen detection. Serologies and the imaging were negative. The patient was initially treated with liposomal amphotericin B for 2 weeks followed by itraconazole PO. He showed rapid improvement within a month and an almost complete resolution by 4 months. Discussion: This patient with extensive mucocutaneous blastomycosis did respond very well to treatment. The most likely hypothesis to explain the extent of his lesions is inhalation followed by hematogenous dissemination. Primary cutaneous blastomycosis caused by direct inoculation due to trauma seems unlikely. Carpenters in contact with wet wood are at risk. Conclusion: Faced with such lesions, a skin biopsy and adequate investigation are essential in order to eliminate a systemic fungal infection that could be fatal in an immunocompromised host.
4721 Extragenital lichen sclerosus: A series of seven cases from a third level hospital in Mexico City Karen Ferez-Blando, MD, Instituto Nacional de Ciencias medicas y Nutricion Salvador Zubiran Background: Lichen sclerosus (LS) is a chronic inflammatory mucocutaneous disease. Its etiology is multifactorial, including genetic, immunologic, infectious, traumatic and hormonal factors. It has a predilection for the anogenital region and rarely presents in the extragenital area. Methods: All cases of pathologically confirmed extragenital LS reported in our hospital from 1987 to 2016 were retrospectively reviewed. Results: Seven patients e all females e with extragenital LS were identified. The median age was 61 years and 50% of them had previously diagnosed autoimmune diseases (eg, Graves’ disease and type 1 diabetes). The lesions were located on the trunk, affecting the neck and abdomen. The most common morphology consisted of bright atrophic hypopigmented plaques. The dermatitis had a duration of 6 months on average and pruritus was the most common symptom (83%). Thirty percent resolved completely and the rest reported partial improvement. Conclusion: The clinical features observed in our study are consistent with the published literature, such as female predominance and age of presentation. We described the comorbidities of our patients, which interestingly shared type 2 diabetes, and autoimmune diseases including thyroid disease and type-1 diabetes. This may serve as a clue to explain LS’s potential autoimmune etiology.
Commercial support: None identified. Commercial support: None identified.
AB136
J AM ACAD DERMATOL
JUNE 2017