Pachydermodactyly: a systematic review

Pachydermodactyly is a rare, benign fibromatosis located around the proximal interphalangeal joints. It is often misdiagnosed as juvenile idiopathic arthritis and may cause unnecessary treatments and anxiety in patients. The goal of this paper is to describe this condition through all the existing information in the scientific literature.

A systematic review and a descriptive study have been conducted. A systematic research was performed in PubMed, Embase, Cochrane Library and WOS.

Pachydermodactyly was four times more frequent in male subjects and usually started in adolescence. Bilateral presentation was more frequent. History of microtrauma in both hands due to digital manipulation was found in almost half of the patients, many of them showed some neuropsychiatric disorder. In women, the onset happened later, unilateral involvement and family history were more frequent. Swelling of soft tissue without joint implication was found in imaging tests. The progression was usually positive and the treatment included stopping the microtrauma, administrating intralesional corticoids and/or surgery.

Diagnosis can be established in asymptomatic young patients through a congruent physical exam, regular analytic results and imaging tests that simply show swelling of soft tissue-a biopsy is generally not required for diagnosis. As pachydermodactyly's course is asymptomatic and benign, knowledge about this condition is limited, which increases the likelihood of its underdiagnosis-it is important that clinicians know of pachydermodactyly in order to avoid misdiagnosis.