Catalog

Familial reactive perforating collagenosis

No MMS code

Link to WHO Definition

Familial reactive perforating collagenosis – http://id.who.int/icd/entity/563155724

Familial reactive perforating collagenosis is a rare autosomal dominant skin disorder characterised by the development of multiple small umbilicated papules with central horny plugs, typically on the extensor surfaces of the hands, elbows and knees. The plugs are formed from trans-epidermal extrusion of abnormal dermal collagen, though the precise defect is not known. The condition usually starts in early childhood but persists into adult life. It is usually exacerbated by environmental cold or trauma.