A 35-year-old male presented with a 6-month history of asymptomatic, generalised, self-healing lesions. On clinical examination, there were diffuse, ulcerated, necrotic papules and nodules with lymphoedema of the face. Histology sections confirmed atypical lymphoid-type cells which appeared round-to-oval with irregular nuclei (horseshoe-shaped). Immunohistochemistry stains were positive for CD30, CD3, and epithelial membrane antigen. The features were in keeping with an anaplastic large-cell lymphoma, T cell type. This transformed into a systemic variant of the disease after the patient had completed chemotherapy.
{"title":"Primary Cutaneous Anaplastic Large-Cell Lymphoma","authors":"N. Moodley, Patiswa Nombona, A. Mosam","doi":"10.1159/000500259","DOIUrl":"https://doi.org/10.1159/000500259","url":null,"abstract":"A 35-year-old male presented with a 6-month history of asymptomatic, generalised, self-healing lesions. On clinical examination, there were diffuse, ulcerated, necrotic papules and nodules with lymphoedema of the face. Histology sections confirmed atypical lymphoid-type cells which appeared round-to-oval with irregular nuclei (horseshoe-shaped). Immunohistochemistry stains were positive for CD30, CD3, and epithelial membrane antigen. The features were in keeping with an anaplastic large-cell lymphoma, T cell type. This transformed into a systemic variant of the disease after the patient had completed chemotherapy.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"13 6","pages":"163 - 169"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000500259","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41292738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Keratolytic winter erythema (KWE) is a rare autosomal dominant keratoderma affecting primarily the palms and soles, manifesting with recurrent waves of erythema followed by epidermal peeling. The condition is so named in view of its anecdotal worsening during the winter months. It is highly penetrant but shows considerable individual clinical variability, waning and reappearing throughout the life course. Histologically, early established lesions of KWE manifest with degenerative changes involving the Malpighian layer, with associated absence of the stratum granulosum. The damaged zone undergoes parakeratotic transformation and subsequent centrifugal ejection. Thick peeling occurs when the stratum corneum eventually separates off as a result of a keratolytic split occurring above, through or below the parakeratotic zone. Reconstitution of the stratum granulosum ensues. KWE is caused by a duplication of an intergenic enhancer element upstream of the cathepsin B gene on chromosome 8. This leads to the upregulation of cathepsin B in the stratum granulosum and subsequent peeling of the epidermis as the end result. With elucidation of the molecular pathology of KWE, new therapeutic approaches to KWE may be considered.
{"title":"Keratolytic Winter Erythema: An Update","authors":"M. Ramsay, Thandiswa Ngcungcu, W. Grayson","doi":"10.1159/000496338","DOIUrl":"https://doi.org/10.1159/000496338","url":null,"abstract":"Keratolytic winter erythema (KWE) is a rare autosomal dominant keratoderma affecting primarily the palms and soles, manifesting with recurrent waves of erythema followed by epidermal peeling. The condition is so named in view of its anecdotal worsening during the winter months. It is highly penetrant but shows considerable individual clinical variability, waning and reappearing throughout the life course. Histologically, early established lesions of KWE manifest with degenerative changes involving the Malpighian layer, with associated absence of the stratum granulosum. The damaged zone undergoes parakeratotic transformation and subsequent centrifugal ejection. Thick peeling occurs when the stratum corneum eventually separates off as a result of a keratolytic split occurring above, through or below the parakeratotic zone. Reconstitution of the stratum granulosum ensues. KWE is caused by a duplication of an intergenic enhancer element upstream of the cathepsin B gene on chromosome 8. This leads to the upregulation of cathepsin B in the stratum granulosum and subsequent peeling of the epidermis as the end result. With elucidation of the molecular pathology of KWE, new therapeutic approaches to KWE may be considered.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"126 - 132"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000496338","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48374146","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Scleroderma is a rare complication of carcinoid syndrome and is usually encountered in the setting of a metastatic primary neuroendocrine tumour of the distal ileum. Associated endocardial fibrosis is a frequent finding and the condition carries a poor prognosis. We report a case of scleroderma occurring in a 72-year-old female with metastatic neuroendocrine carcinoma and associated pericardial fibrosis. The use of an alternative nomenclature such as “scleroderma-like” or “sclerodermoid” disease is proposed in order to emphasise its distinction from true idiopathic scleroderma, despite the histopathological similarities on skin biopsy.
{"title":"Carcinoid Syndrome-Induced Scleroderma-Like Disease","authors":"K. Koch, W. Grayson","doi":"10.1159/000496388","DOIUrl":"https://doi.org/10.1159/000496388","url":null,"abstract":"Scleroderma is a rare complication of carcinoid syndrome and is usually encountered in the setting of a metastatic primary neuroendocrine tumour of the distal ileum. Associated endocardial fibrosis is a frequent finding and the condition carries a poor prognosis. We report a case of scleroderma occurring in a 72-year-old female with metastatic neuroendocrine carcinoma and associated pericardial fibrosis. The use of an alternative nomenclature such as “scleroderma-like” or “sclerodermoid” disease is proposed in order to emphasise its distinction from true idiopathic scleroderma, despite the histopathological similarities on skin biopsy.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"99 - 104"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000496388","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47096032","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Malignant chondroid syringoma (MCS; malignant mixed tumour) is a rare neoplasm typically arising on the extremities and trunk. We are report 2 unique cases of MCS, one occurring on the scalp of a 78-year-old man and the other on the trunk of a 72-year-old woman. Both tumours harboured malignant epithelial and malignant mesenchymal components. The latter was represented by liposarcoma in the first case. The malignant components of the second tumour comprised spindle cell squamous cell carcinoma (SCC) and osteosarcoma. Origin from a pre-existing benign chondroid syringoma was clearly evident in both neoplasms. The presence of heterologous malignant mesenchymal components, however, is hitherto unreported in the context of MCS, while a spindle cell SCC component is exceptionally rare. The 2 cases presented herein highlight an expanded morphological spectrum of MCS, with resultant blurring of the boundaries between MCS and cutaneous carcinosarcoma.
{"title":"Malignant Chondroid Syringoma: A Report of Two Cases with a Sarcomatous Mesenchymal Component","authors":"C. Nel, Dawn van der Byl, W. Grayson","doi":"10.1159/000495610","DOIUrl":"https://doi.org/10.1159/000495610","url":null,"abstract":"Malignant chondroid syringoma (MCS; malignant mixed tumour) is a rare neoplasm typically arising on the extremities and trunk. We are report 2 unique cases of MCS, one occurring on the scalp of a 78-year-old man and the other on the trunk of a 72-year-old woman. Both tumours harboured malignant epithelial and malignant mesenchymal components. The latter was represented by liposarcoma in the first case. The malignant components of the second tumour comprised spindle cell squamous cell carcinoma (SCC) and osteosarcoma. Origin from a pre-existing benign chondroid syringoma was clearly evident in both neoplasms. The presence of heterologous malignant mesenchymal components, however, is hitherto unreported in the context of MCS, while a spindle cell SCC component is exceptionally rare. The 2 cases presented herein highlight an expanded morphological spectrum of MCS, with resultant blurring of the boundaries between MCS and cutaneous carcinosarcoma.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"77 - 84"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000495610","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43733698","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
E. Larivé, M. Nicolas, G. Kaya, Nicolò Riggi, A. Moulin
Purpose: To assess the role of the canonical Wnt pathway via activation of β-catenin in tumor progression of conjunctival melanoma. Methods: β-Catenin localization was assessed by immunohistochemistry in 43 conjunctival nevi, 48 primary acquired melanoses (PAM; conjunctival melanocytic intraepithelial neoplasia), and 44 conjunctival melanomas. Activation of the canonical or the noncanonical Wnt pathway was tested in vitro in 4 conjunctival melanoma cell lines with stimulation of either Wnt5a or Wnt3a. Wound healing assays were performed with Wnt5a. Results: Nuclear β-catenin expression was found in 16% of the nevi, in 15% of the melanomas, and in 4% of the PAM. Membranous β-catenin expression was identified in all the nevi and PAM and in 97.7% of the melanomas. In vitro, Wnt5a stimulation in the 4 conjunctival melanomas and in 1 skin melanoma cell line did not induce nuclear translocation of β-catenin, nor did it increase cell motility in the wound healing assays. Wnt3a stimulation did not induce nuclear localization of β-catenin in any of the cell lines tested. Conclusions: In conjunctival melanoma, nuclear localization and activation of β-catenin appear to be limited, suggesting that inhibition of ARF6, responsible for β-catenin activation, in subsets of skin melanoma may not represent a treatment option for this tumor. In vitro, Wnt3a or Wnt5a did not induce nuclear β-catenin localization.
{"title":"β-Catenin Expression and Activation in Conjunctival Melanoma","authors":"E. Larivé, M. Nicolas, G. Kaya, Nicolò Riggi, A. Moulin","doi":"10.1159/000500682","DOIUrl":"https://doi.org/10.1159/000500682","url":null,"abstract":"Purpose: To assess the role of the canonical Wnt pathway via activation of β-catenin in tumor progression of conjunctival melanoma. Methods: β-Catenin localization was assessed by immunohistochemistry in 43 conjunctival nevi, 48 primary acquired melanoses (PAM; conjunctival melanocytic intraepithelial neoplasia), and 44 conjunctival melanomas. Activation of the canonical or the noncanonical Wnt pathway was tested in vitro in 4 conjunctival melanoma cell lines with stimulation of either Wnt5a or Wnt3a. Wound healing assays were performed with Wnt5a. Results: Nuclear β-catenin expression was found in 16% of the nevi, in 15% of the melanomas, and in 4% of the PAM. Membranous β-catenin expression was identified in all the nevi and PAM and in 97.7% of the melanomas. In vitro, Wnt5a stimulation in the 4 conjunctival melanomas and in 1 skin melanoma cell line did not induce nuclear translocation of β-catenin, nor did it increase cell motility in the wound healing assays. Wnt3a stimulation did not induce nuclear localization of β-catenin in any of the cell lines tested. Conclusions: In conjunctival melanoma, nuclear localization and activation of β-catenin appear to be limited, suggesting that inhibition of ARF6, responsible for β-catenin activation, in subsets of skin melanoma may not represent a treatment option for this tumor. In vitro, Wnt3a or Wnt5a did not induce nuclear β-catenin localization.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"50 - 62"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000500682","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47177966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Giant congenital melanocytic naevi (GCN) are rare, disfiguring lesions which carry a significant risk of malignant transformation. Melanoma is the most common malignancy documented in association with these lesions. Although exceedingly rare, other malignant neoplasms, including mesenchymal tumours such as rhabdomyosarcoma (RMS), may complicate GCN. This report documents a fatal embryonal RMS arising in a GCN on the distal left lower limb of a 4-month-old female infant, who had ipsilateral inguinal lymph node metastases at the time of presentation. To date there have been only 7 prior reports in the English literature of RMS complicating GCN. Differential diagnoses include small cell melanoma, rhabdoid melanoma, and melanoma with divergent RMS differentiation. A distinction between the latter and de novo RMS arising in GCN may have potential prognostic and therapeutic implications.
{"title":"Rhabdomyosarcoma Arising in a Giant Congenital Melanocytic Naevus: Case Report and Literature Review","authors":"Tirelo M. Pitjadi, R. Wadee, W. Grayson","doi":"10.1159/000496337","DOIUrl":"https://doi.org/10.1159/000496337","url":null,"abstract":"Giant congenital melanocytic naevi (GCN) are rare, disfiguring lesions which carry a significant risk of malignant transformation. Melanoma is the most common malignancy documented in association with these lesions. Although exceedingly rare, other malignant neoplasms, including mesenchymal tumours such as rhabdomyosarcoma (RMS), may complicate GCN. This report documents a fatal embryonal RMS arising in a GCN on the distal left lower limb of a 4-month-old female infant, who had ipsilateral inguinal lymph node metastases at the time of presentation. To date there have been only 7 prior reports in the English literature of RMS complicating GCN. Differential diagnoses include small cell melanoma, rhabdoid melanoma, and melanoma with divergent RMS differentiation. A distinction between the latter and de novo RMS arising in GCN may have potential prognostic and therapeutic implications.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"91 - 98"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000496337","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49352147","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Epstein-Barr virus-associated smooth muscle tumours (EBV-SMTs) are rare neoplasms of uncertain biological potential. They are seen in the setting of immune suppression from a variety of causes, including HIV infection and post-transplant immunosuppression. Most of the literature pertaining to these neoplasms comprises case reports and small case series, with a dearth of documented cases from South Africa. Objective: To expand on the literature of these rare neoplasms in the South African context, with an emphasis on a subset showing a predilection for the cutaneous soft tissues. Method: Twenty-one EBV-SMTs from 19 consecutive patients were retrieved from the archives of the Division of Anatomical Pathology in the Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, and the National Health Laboratory Service. Clinical and pathological characteristics of each case were recorded, including patient age, tumour site, H&E morphology, immunophenotypic features and the tumoural EBV status. Results: The patients’ ages ranged from 12 to 63 years, with a mean of 36 years. Thirteen (68%) of the patients in whom the HIV status was known were HIV-positive. Two of the 19 patients each had 2 tumours, thus accounting for the total of 21 neoplasms studied. Although 12 of the 21 tumours (57.1%) were from a variety of visceral organs, 9 (42.9%) originated in the dermis and superficial subcutaneous tissues, making the cutaneous soft tissues the most commonly affected site. Morphologically, all of the neoplasms were characterised by fascicles of myoid cells, admixed rounder tumour cells, scattered intratumoural lymphocytes and variable immunohistochemical staining with markers of smooth muscle differentiation. All 21 neoplasms were proven to harbour EBV DNA. Conclusion: A significant proportion of EBV-SMTs may present in the cutaneous soft tissues. This neoplasm should, therefore, be included in the histopathological differential diagnosis of any cutaneous or superficial subcutaneous spindle cell tumour, especially in patients with a history of underlying immune suppression. Accurate diagnosis thereof and its distinction from other spindle cell neoplasms is important in view of management implications and the potential for multicentricity in some patients.
{"title":"Epstein-Barr Virus-Associated Smooth Muscle Tumour: A Case Series with a Significant Proportion of Tumours Showing Proclivity for Cutaneous Soft Tissues","authors":"Tirelo M. Pitjadi, W. Grayson","doi":"10.1159/000497075","DOIUrl":"https://doi.org/10.1159/000497075","url":null,"abstract":"Background: Epstein-Barr virus-associated smooth muscle tumours (EBV-SMTs) are rare neoplasms of uncertain biological potential. They are seen in the setting of immune suppression from a variety of causes, including HIV infection and post-transplant immunosuppression. Most of the literature pertaining to these neoplasms comprises case reports and small case series, with a dearth of documented cases from South Africa. Objective: To expand on the literature of these rare neoplasms in the South African context, with an emphasis on a subset showing a predilection for the cutaneous soft tissues. Method: Twenty-one EBV-SMTs from 19 consecutive patients were retrieved from the archives of the Division of Anatomical Pathology in the Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, and the National Health Laboratory Service. Clinical and pathological characteristics of each case were recorded, including patient age, tumour site, H&E morphology, immunophenotypic features and the tumoural EBV status. Results: The patients’ ages ranged from 12 to 63 years, with a mean of 36 years. Thirteen (68%) of the patients in whom the HIV status was known were HIV-positive. Two of the 19 patients each had 2 tumours, thus accounting for the total of 21 neoplasms studied. Although 12 of the 21 tumours (57.1%) were from a variety of visceral organs, 9 (42.9%) originated in the dermis and superficial subcutaneous tissues, making the cutaneous soft tissues the most commonly affected site. Morphologically, all of the neoplasms were characterised by fascicles of myoid cells, admixed rounder tumour cells, scattered intratumoural lymphocytes and variable immunohistochemical staining with markers of smooth muscle differentiation. All 21 neoplasms were proven to harbour EBV DNA. Conclusion: A significant proportion of EBV-SMTs may present in the cutaneous soft tissues. This neoplasm should, therefore, be included in the histopathological differential diagnosis of any cutaneous or superficial subcutaneous spindle cell tumour, especially in patients with a history of underlying immune suppression. Accurate diagnosis thereof and its distinction from other spindle cell neoplasms is important in view of management implications and the potential for multicentricity in some patients.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"133 - 146"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000497075","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42633775","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dowling-Degos disease (DDD) is a rare genodermatosis primarily presenting with reticulated pigmentation of the flexures. Secondary features include comedones and atrophic scarring. We present a patient with histologically confirmed DDD whose predominant clinical finding was of comedones and scarring, with less prominent pigmentation, thus expanding the clinical spectrum of DDD.
{"title":"Dowling-Degos Disease Presenting Primarily with Comedones and Atrophic Scarring","authors":"B. Tod, Ilana Steenkamp, H. Jordaan, W. Visser","doi":"10.1159/000497177","DOIUrl":"https://doi.org/10.1159/000497177","url":null,"abstract":"Dowling-Degos disease (DDD) is a rare genodermatosis primarily presenting with reticulated pigmentation of the flexures. Secondary features include comedones and atrophic scarring. We present a patient with histologically confirmed DDD whose predominant clinical finding was of comedones and scarring, with less prominent pigmentation, thus expanding the clinical spectrum of DDD.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"153 - 156"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000497177","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48106212","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: The global mortality from HIV and the cutaneous burden of infective, inflammatory and malignant diseases in the setting of AIDS have significantly declined following the advent of highly active antiretroviral therapy. Regrettably, there has been a contemporaneous escalation in the incidence of adverse cutaneous drug reactions (ACDR), with studies attesting that HIV-positive individuals are a hundred times more susceptible to drug reactions than the general population, and advanced immunodeficiency portending an even greater risk. Several variables are accountable for this amplified risk in HIV. Summary: Adverse reactions to trimethoprim-sulfamethoxazole are the most common, increasing from approximately 2–8% in the general population over to 43% amongst HIV-positive individuals to approximately 69% in subjects with AIDS. Antituberculosis drugs and antiretrovirals are also well-known instigators of ACDR. Cutaneous reactions range from mild morbilliform eruptions to severe, life-threatening manifestations in the form of Stevens-Johnson syndrome/toxic epidermal necrolysis. Histological features vary from vacuolar interface changes to full-thickness epidermal necrosis with subepidermal blister formation. A precipitous diagnosis of the ACDR, clinically and histologically if necessary, together with the isolation of the causative drug is critical. The identification process, however, is often complex and multifaceted due to polypharmacy and inconclusive data on which drugs are the most likely offending agents, especially against the background of tuberculosis co-infection. Key Messages: Whilst milder cutaneous reactions are treated symptomatically, severe reactions mandate immediate treatment discontinuation without rechallenge. Further studies are required to establish safe rechallenge guidelines in resource-limited settings with a high HIV and tuberculosis prevalence.
{"title":"An Update on Adverse Cutaneous Drug Reactions in HIV/AIDS","authors":"K. Hoosen, A. Mosam, N. Dlova, W. Grayson","doi":"10.1159/000496389","DOIUrl":"https://doi.org/10.1159/000496389","url":null,"abstract":"Background: The global mortality from HIV and the cutaneous burden of infective, inflammatory and malignant diseases in the setting of AIDS have significantly declined following the advent of highly active antiretroviral therapy. Regrettably, there has been a contemporaneous escalation in the incidence of adverse cutaneous drug reactions (ACDR), with studies attesting that HIV-positive individuals are a hundred times more susceptible to drug reactions than the general population, and advanced immunodeficiency portending an even greater risk. Several variables are accountable for this amplified risk in HIV. Summary: Adverse reactions to trimethoprim-sulfamethoxazole are the most common, increasing from approximately 2–8% in the general population over to 43% amongst HIV-positive individuals to approximately 69% in subjects with AIDS. Antituberculosis drugs and antiretrovirals are also well-known instigators of ACDR. Cutaneous reactions range from mild morbilliform eruptions to severe, life-threatening manifestations in the form of Stevens-Johnson syndrome/toxic epidermal necrolysis. Histological features vary from vacuolar interface changes to full-thickness epidermal necrosis with subepidermal blister formation. A precipitous diagnosis of the ACDR, clinically and histologically if necessary, together with the isolation of the causative drug is critical. The identification process, however, is often complex and multifaceted due to polypharmacy and inconclusive data on which drugs are the most likely offending agents, especially against the background of tuberculosis co-infection. Key Messages: Whilst milder cutaneous reactions are treated symptomatically, severe reactions mandate immediate treatment discontinuation without rechallenge. Further studies are required to establish safe rechallenge guidelines in resource-limited settings with a high HIV and tuberculosis prevalence.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"111 - 125"},"PeriodicalIF":1.9,"publicationDate":"2019-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000496389","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47504297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Epidermolytic acanthoma is a rare benign tumor that appears as a solitary papule or, rarely, multiple small papules on the trunk and extremities, or on genitalia. They are generally asymptomatic, although they can be pruritic. The clinical presentation is often misleading, and the lesions are often misdiagnosed histologically and frequently confused with condyloma acuminatum. Here, we report a case of an epidermolytic acanthoma on the penis of a 57-year-old male, whose final diagnosis was made after several years.
{"title":"Epidermolytic Acanthoma Mimicking Condyloma: A Case Report","authors":"A. Dupont, H. Marescassier, G. Kaya","doi":"10.1159/000499366","DOIUrl":"https://doi.org/10.1159/000499366","url":null,"abstract":"Epidermolytic acanthoma is a rare benign tumor that appears as a solitary papule or, rarely, multiple small papules on the trunk and extremities, or on genitalia. They are generally asymptomatic, although they can be pruritic. The clinical presentation is often misleading, and the lesions are often misdiagnosed histologically and frequently confused with condyloma acuminatum. Here, we report a case of an epidermolytic acanthoma on the penis of a 57-year-old male, whose final diagnosis was made after several years.","PeriodicalId":42885,"journal":{"name":"Dermatopathology","volume":"6 1","pages":"23 - 27"},"PeriodicalIF":1.9,"publicationDate":"2019-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000499366","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49617331","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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