A 32 years old female presented with a single asymptomatic lesion on right thigh since 5 years. On examination, a single ill defined, irregular hyperpigmented plaque was present on medial aspect of right thigh. The plaque was firm in consistency and was non-tender. No scaling was evident. Clinically a differential diagnosis of Hansen's disease, lupus vulgaris and deep fungal infection were made. The histopathology interestingly showed features suggestive of Kaposi's sarcoma. The patient was HIV negative and otherwise completely asymptomatic.
Gorlin syndrome or basal cell nevus syndrome (BCNS) is a rare autosomal dominant disorder. The condition appears to have complete penetrance and variable expressivity, which makes clinilcal presentation among families variable. All known BCNS carry mutations in PATCHED gene. A 65 years old male patient presented with complaints of characteristic skin lesions on his face, back, palms since early adulthood. The lesions were pigmented nodules with characteristic border. The histopathology showed characteristic features suggestive of Basal Cell Carcinoma (BCC). This case was atypical due to appearance of lesions quite later in life.
Acrokeratosis verruciformis of Hopf (AKV) is a rare disorder of keratinization inherited in an autosomal dominant fashion. A 15-year-old female presented with numerous skin-colored papular lesions over the neck as well as dorsa of the hands and feet of 7 years duration. Similar lesions were noted in her mother. Six other members of her family showed similar lesions with similar site of involvement. Presence of characteristic warty papules and histopathology led to diagnosis of a rare condition of acrokeratosis verruciformis.
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