Familial reactive perforating collagenosis

Abstract

Background: Reactive perforating collagenosis (RPC) is one of the rare forms of transepidermal elimination in which genetically altered collagen is extruded from the epidermis. This disease usually starts in early childhood as asymptomatic umbilicated papules on extremities, and the lesions become more conspicuous with age. Aims: The objective of our study was to determine the clinico-pathological features of RPC and the response to various treatment modalities. Methods: Ten patients of RPC, belonging to five different families, were studied clinically. Various laboratory investigations were carried out and diagnosis was made by histopathology of the lesions. Patients were given various topical and oral treatments. Results: RPC is familial in most cases without any definite inheritance pattern. It begins in childhood and the lesions are usually recurrent and become profuse and large with age. Systemic diseases have no role in the onset of lesions. Conclusion: Oral and topical retinoids in combination with emollients is the best treatment option.

Keywords

• Familial
• umbilicated
• transepidermal elimination
• retinoids