Inherited reactive perforating collagenosis – A rare entity

Reactive perforating collagenosis (RPC) is a rare primary perforating dermatosis, showing distinct transepidermal elimination of modified collagen across the epidermis. This condition typically appears in two forms: Hereditary variant (rare) and acquired variant. Patients present with pruritic hyperkeratotic papules with central crusting mainly over extensor areas of extremities and hands, most likely over areas of superficial trauma with vertically oriented collagen fibers on histopathology. Here, we present a case of hereditary variant of RPC with classic histopathological findings confirmed with special stains.