Case for diagnosis

Abstract

Pachydermodactyly is a rare and benign form of acquired digital fibromatosis characterized by the expansion of soft tissue around proximal phalanges and interphalangeal joints. The etiology remains unknown, although it is suggested that repetitive mechanic trauma like the interlacing or rubbing of the fingers can lead to skin thickening. We report a case of a young man with skin thickening around interphalangeal joints and compulsive finger manipulation habit. The histopathology disclosed hyperkeratosis, discrete papillomatosis, mild increase of fibroblasts and dermal mucinosis. The lesions regressed partially, after finger manipulation was stopped.

Pachydermodactyly – a report of two cases

“…Histological examination of a biopsy specimen shows mostly non – specific changes: epidermal hyperkeratosis with hypergranulosis, sometimes with thickening of the collagen fibers and disturbance of their linear system, slight proliferation of vascular superficial plexus with or without minimal inflammatory infiltration and occasionally small deposits of connective-tissue mucin [ 3 ].…”

“…Based on clinical manifestation (presence of symptoms suggesting sclerodactyly), outcome of the disease, diagnostic tests (results of capillaroscopy and MRI examination, positive anti-nuclear antibodies titer), undifferentiated connective tissue disease (UCTD) with concomitance of symptoms suggesting pachydermodactyly was diagnosed [ 1 , 3 , 7 , 14 – 17 ].…”

“…On the basis of clinical manifestation, imaging examination and histopathology (hyperkeratosis, hypergranulosis in epidermis, slightly thickened collagen fibers in the dermis) pachydermodactyly was diagnosed [ 1 , 3 , 7 , 9 ].…”

“…So far in the literature only about 150 cases of PDD have been reported [ 2 – 4 , 6 , 9 ]. The etiology of the disease remains unknown, PDD is usually acquired, even though there are some publications that document family cases [ 3 , 6 , 9 , 10 ]. It affects mainly adolescent men [ 5 ].…”

“…It affects mainly adolescent men [ 5 ]. It has been suggested that multiple mechanical traumas, such as the habit of interlacing and rubbing the fingers, could lead to skin thickness, what causes secondary, mechanical dermatosis based on compulsive behaviours [ 3 – 11 ]. The classic type of disease can even simulate polyarticular JIA with involvement of small joints of the hands [ 3 , 8 – 10 , 12 ].…”