Familial reactive perforating collagenosis.

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.