Indian dermatology online journal
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Pachyonychia congenita is a rare type of ectodermal dysplasia further classified into 4 types. Cutaneous manifestations seen in most of the cases of Pachyonychia congenita include palmoplantar keratoderma, follicular hyperkeratosis, wedge shaped nails, oral leukokeratosis and woolly hair. A 25-year-old male presented to us with thickened nails and scanty scalp hair. ⋯ The patient had history of natal teeth and on dental examination, lower central incisors were absent. All cutaneous changes in our case had manifested first in the 2(nd) decade except for natal teeth. All the above features suggested the diagnosis of pachyonychia congenita with late onset (PC tarda), which is an infrequently reported rare variant.
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Indian Dermatol Online J · May 2016
Case ReportsKey messages from a rare case of annular sarcoidosis of scalp.
Sarcoidosis, a multisystem disease of obscure etiology, is characterized by the formation of noncaseating epithelioid cell granulomas in several organs or tissues. The diagnosis of sarcoidosis requires a compatible clinical picture, histologic demonstration of noncaseating granulomas, and exclusion of other diseases capable of producing similar histology or clinical features. The lung is the most commonly affected organ, but the skin is frequently involved. ⋯ Alopecia is common in sarcoidosis and is generally scarring in nature. Annular sarcoidosis is a rare morphology and annular sarcoid of scalp is seldom reported. Herein we present a case of annular scalp sarcoid with systemic involvement and without alopecia.
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Sweet's syndrome is characterized by erythematous tender nodules and plaques over face and extremities. Fever, leukocytosis with neutrophilia, and a neutrophilic infiltrate in the dermis are characteristic features. Neutrophilic dermatosis of dorsal hands is a rare localized variant of Sweet's syndrome occurring predominantly over dorsa of hands. ⋯ Both Sweet's syndrome and its dorsal hand variant have been reported in association with malignancies, inflammatory bowel diseases, and drugs. We report a patient with neutrophilic dermatoses of dorsal hands associated with erythema nodosum. He showed an excellent response to corticosteroids and dapsone.
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Indian Dermatol Online J · Nov 2014
Case ReportsBerardinelli-Seip congenital lipodystrophy in two siblings.
Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare autosomal recessive disorder characterized by various dermatological and systemic manifestations such as lipoatrophy, hypertriglyceridemia, hepatomegaly, acanthosis nigricans, and acromegaloid features. BSCL type 2 is more common and severe, with onset in the neonatal period or in early infancy. ⋯ Early recognition and differentiation from other congenital generalized lipodystrophies help in the initiation of appropriate preventive and therapeutic measures such as lifestyle modification and pharmacotherapy that helps postpone the onset of metabolic syndrome. We report BSCL type 2 in two siblings with several cutaneous manifestations like acanthosis nigricans, hypertrichosis, prominent subcutaneous veins, and increased lanugo hair.
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Indian Dermatol Online J · Jan 2014
Clinico-etiological study of 30 erythroderma cases from tertiary center in South India.
Erythroderma is a morphological reaction pattern of skin having many underlying causes and finding the etiology helps in the proper management of erythroderma cases. ⋯ Clinical features were identical irrespective of etiology. Detailed clinico-histopathological examination helps to establish the etiology of erythroderma.