Abstract

Perforating granuloma annulare (PGA) is a rare subtype of granuloma annulare (GA) with an unknown etiology. Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare autosomal dominant disorder comprised of a number of abnormalities, including multiple nevoid basal cell carcinomas (BCC), skeletal abnormalities, and multiple keratocystic odontogenic tumors. No association of nevoid basal cell carcinoma syndrome and perforating granuloma annulare has been documented. Here, we report a case of a patient with a history of NBCCS with multiple BCC and PGA. A 60-year-old female presented with numerous BCC and a pink papule with central superficial ulceration on the right fifth metacarpophalangeal joint (MCP). The papule on the right fifth MCP was previously treated with liquid nitrogen cryotherapy two months prior and was still present upon follow-up examination. Shave biopsy showed dermal palisaded granulomas with overlying epidermal hyperplasia and transepidermal elimination of degenerated collagen. Although PGA is a benign disease, its histopathological findings can mimic epithelioid sarcoma; thus, it is important to rule out this more serious entity.

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