Pachydermodactyly: a systematic review
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REVIEW ARTICLE
Pachydermodactyly: a systematic review Ricardo Vázquez Fernández 1,2 Antonio Mera Varela 1,4
&
José Ramón Maneiro Fernández 3 & Evelin Cecilia Cervantes Pérez 3 &
Received: 18 May 2020 / Accepted: 22 September 2020 # Royal Academy of Medicine in Ireland 2020
Abstract Introduction/objectives 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. Method A systematic review and a descriptive study have been conducted. A systematic research was performed in PubMed, Embase, Cochrane Library and WOS. Results 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. Conclusions 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. Keywords Digital fibromatosis . Pachydermodactyly . Proximal interphalangeal joint and painless swelling . Pseudo knuckle pads
Introduction Pachydermodactyly (PDD) from the Greek pachy (thick), dermo (skin) and dactylos (finger) is a rare and benign digital fibromatosis. [its] Incidence is unknown, although around 150 Electronic supplementary material The online version of this article (https://doi.org/10.1007/s11845-020-02378-1) contains supplementary material, which is available to authorized users. * Ricardo Vázquez Fernández [email protected] 1
Department of Medicine, Medical School, Universidad de Santiago de Compostela, Santiago de Compostela, Spain
2
University of Santiago de Compostela, A Coruña, Spain
3
Rheumatology Unit, Complejo Hospitalario Universitario de Pontevedra, Pontevedra, Spain
4
Rheumatology Unit, Complejo Clínico Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain
cases have been documented in the scientific literature [1]. It was first described in 1973 by Bazex and named 2 years later by Verbov [2], who initially classified it as a variant of knuckle pads due to th
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