Disabling pansclerotic morphea of childhood with extracutaneous manifestations

Disabling pansclerotic morphea (DPM) of childhood is a rare generalized type of localized scleroderma (LS) that is known to follow an aggressive course with pansclerotic lesions leading to severe joint contractures and consequent immobility. Mortality is due to complications of the disease such as bronchopneumonia, sepsis, or gangrene. There is no specific laboratory finding. Treatment protocols are still evolving for this severe recalcitrant disorder. Extracutaneous manifestations are rarely reported in DPM. We present the case of a 7-year-old girl with DPM with severe extracutaneous manifestations in the form of gastrointestinal and vascular disease, whose disease progressed rapidly. In spite of treatment with methotrexate, corticosteroids, and PUVA therapy, she ultimately succumbed to her illness due to sepsis

Cervicofacial actinomycosis mimicking lymphangioma circumscriptum

Primary cutaneous actinomycosis caused by Actinomyces israelii occurs most commonly in the cervicofacial area. It commonly presents as "lumpy jaw" with draining sinuses which discharge the characteristic "sulfur granules". A low index of suspicion and a low sensitivity in culturing the organism, due to its fastidious nature often delays the diagnosis. An atypical clinical presentation mimicking lymphangioma circumscriptum with grouped papulovesicular and nodular lesions along the lower jaw extending from skin to the inner buccal mucosa, confirmed on histology and an excellent therapeutic response to penicillin is reported

Nail changes in immuno-bullous disorders: A cross-sectional study

Context: Autoimmune blistering (AIBD) disorders affect mucosae, skin, and appendages. Appendageal involvement has not been studied extensively to date. However, they are important as nail changes are commonly encountered during a flare-up of the disease. Aims: To determine the prevalence and patterns of nail changes in various Immunobullous disorders and to study the relationship between the nail changes and the disease duration and severity. Settings and Design: A cross-sectional study was conducted at the Department of Dermatology, venerology, and leprosy at a tertiary care center in Mumbai. Materials and Methods: A cross-sectional study including a total of 74 cases of Immunobullous diseases was conducted and the prevalence of nail changes was determined. The association between the mean number of nail changes and the disease duration and severity was analyzed using ANOVA (Analysis of variance) and unpaired t-test. Statistical Analysis: The Statistical Package for Social Studies (SPSS) software was used for statistical evaluation. Results: We found that the prevalence of nail changes was 91%. There was a significant difference in the mean number of nail changes with respect to the severity grades of mucosal involvement in AIBD (P value < 0.05). There was no significant difference in the mean number of nail changes with respect to the severity grades of Pemphigus vulgaris (PV), Pemphigus foliaceous (PF), subepidermal blistering diseases, and the duration of AIBD. Conclusions: Nails are frequently affected in AIBD. The number of nail changes is related to the severity of mucosal disease but not to duration

Acute hemorrhagic edema of infancy-a rare entity

A patient presenting with fever and purpura often presents a diagnostic challenge for the dermatologist. Acute hemorrhagic edema of infancy (AHEI) is a rare acute cutaneous leukocytoclastic vasculitic syndrome of infants leading to this presentation. We present an 18-month-old girl with AHEI who presented with fever, edema, and purpuric lesions involving the face, ears and extremities with uneventful complete recovery

Acral acanthosis nigricans in a case of scleroderma

Acanthosis nigricans (AN) is a dermatosis characterized by velvety, papillomatous, brownish-black, hyperkeratotic plaques, typically on the intertriginous surfaces and neck. The majority (80%) of AN occurs idiopathically or in benign conditions such as endocrinopathies like diabetes mellitus, polycystic ovary syndrome; metabolic syndrome and/or heritable disease. Malignancy-associated AN is rare. AN may rarely be associated with autoimmune diseases including systemic lupus erythematosus, due to antibodies to the insulin receptor, so-called type B insulin resistance. Here we report a case of AN in a case of diffuse progressive systemic sclerosis without evidence of insulin resistance

Primary Cutaneous Actinomycosis Mimicking Cutaneous Leishmaniasis

Actinomycosis is a chronic suppurative bacterial infection commonly seen in the tropics, caused by gram-positive, anaerobic bacilli of the genus Actinomyces. There are very few reported cases of primary cutaneous actinomycosis. It can mimic mycetoma, tuberculosis, nocardiosis, and botryomycosis. A high index of clinical suspicion is required for diagnosis in the absence of sinuses. Even with repeated attempts, cultures are mostly negative; and hence, histology reveals the diagnosis in most cases. Here, we report an unusual case of primary cutaneous actinomycosis in a 21-year-old female patient, following a road traffic accident (RTA). A positive Splendore–Hoeppli phenomenon and special stains demonstrated the ray fungus and helped us reach the diagnosis. The patient was started on oral penicillin G and showed good response

Aggressive angiomyxoma of the vulva: An uncommon entity

Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor occurring usually in women of reproductive age, which carries a high risk for local relapse; hence the need to differentiate it from the other mesenchymal tumors occurring in this region. We describe a case of a 44-year-old female presenting with a large pedunculated swelling on the right labia majora