Pachydermodactyly: A case report and literature review

Abstract

Pachydermodactyly is a rare, benign form of digital fibromatosis, characterized by asymptomatic and progressive, periarticular and usually symmetrical soft tissue finger swelling, specifically on the lateral aspects of the proximal interphalangeal joints mainly of the second, third, and fourth fingers; it mostly affects young adolescents and is probably due to repeated mechanical injury of the skin (such as repeated clasping or rubbing of crossed fingers), sometimes as a result of obsessive-compulsive disorder, which must be distinguished from obsessive "chewing pads". This paper presents a male patient aged 19, who presented with first symptoms at the age of 12, and was diagnosed with periarticular hypertrophy: localized soft tissue thickening around the proximal interphalangeal joints of all fingers except the thumbs; slight hypertrophy of the skin; absence of subjective complaints; normal joint function. Dermatological status on admission revealed: symmetrical soft tissue swelling of all fingers of both hands except the thumbs at the level of the proximal interphalangeal joints; normal appearance of the distal parts of all fingers; thickening at the level of the proximal interphalangeal joints, bilateral, almost symmetrical hypertrophy (ulnar and radial) of phalanges of the affected fingers except both index fingers, affecting only the ulnar side. The skin lesions were pain-free on palpation, with homogeneous texture and elastic consistency, freely movable over underlying structures. The affected joints showed no functional deficit. The test results, sonography, radiography and histopathology confirmed our clinical diagnosis - pachydermodactyly. The review of the currently available literature, published between 1973 and 2014, including 99 papers and 160 patients, provided important insight into the characteristics and variations of the disease.