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Pachydermodactyly
Pachydermodactyly
Specialty	Dermatology

Pachydermodactyly is a superficial dermal fibromatosis that presents as a poorly circumscribed symmetric, infiltrative, asymptomatic soft-tissue hypertrophy of the proximal fingers, typically sparing the thumbs and fifth fingers and rarely extending proximally to the wrists or occurring distally.^[1]^: 990

Signs and symptoms

Pachydermodactyly is defined by gradual, asymptomatic thickening of the periarticular skin and soft tissue swelling, mainly affecting the lateral sides of the fingers' proximal interphalangeal (PIP) joints.^[2]^[3] The most commonly affected fingers are the second, third, and fourth; however, the dorsum of the hand and the fifth finger may also be affected.^[4] Although moderate erythema, fine desquamation, or lichenification may occasionally be seen, epidermal alterations are not typically present.^[5] There is no impact on hand movements.^[6]

Causes

Although the specific cause and etiology are yet unknown, excessive mechanical manipulation of PIP joints is likely to be a contributing factor.^[7] The basis for this suggestion is the discovery that pachydermodactyly is related to a variety of jobs and pursuits, including weightlifting,^[6] employment in food processing facilities,^[8] martial arts, climbing,^[9] and individuals with OCD who have hand tics.^[10]

Diagnosis

The final diagnosis is clinical and made after ruling out other possible causes of joint swelling. It calls for a high degree of suspicion, especially in individuals who have progressive soft tissue swelling without any pain, soreness, or diminished function.^[11] Unless clinically indicated, laboratory testing such as extractable nuclear antigen test, rheumatoid factor, C-reactive protein, complete blood count, and antinuclear factor are not required.^[12]

A thickening of the dermis, possibly associated with a benign proliferation of fibroblasts, and an increase in collagen fibers that extend to the subcutaneous cellular tissue are the most common histological findings.^[6] In addition, there may be a decrease in mucin and elastic fibers, as well as hyperkeratosis (orthokeratosis or parakeratosis).^[4]

Hand radiographs usually show soft tissue swelling without interphalangeal joint structural involvement.^[13] An X-ray examination shows no abnormalities in the periosteum or bones in pachydermodactyly patients.^[14] Additionally, no changes are seen in the synovium or joints during ultrasound.^[15]

Chen et al. published a diagnostic criterion that consists of six elements: the patient having no symptoms; there being no stiffness in the morning; no restriction on movement; swelling on the lateral aspect of the finger; normal laboratory values; and soft tissue swelling on radiography.^[16]

Differential diagnoses include rheumatoid arthritis, acromegaly, juvenile idiopathic arthritis, pachydermoperiostosis, knuckle pads, and juvenile digital fibromatosis.^[17]

Treatment

Pachydermodactyly does not currently have a commonly accepted treatment.^[18] Nevertheless, aggressive therapy is usually not necessary due to the disease's benign course.^[19] Since the majority of pachydermodactyly cases appear with symptoms caused by recurrent joint trauma, discontinuing stimulating activities is sufficient to promote regression or stabilization.^[20] It has also been demonstrated that intralesional corticosteroids can sometimes reduce swelling.^{[citation needed]} Intralesional triamcinolone injections have also been reported to alleviate symptoms.^[6] Pachydermodactyly patients frequently have surgery to improve the appearance of a digit.^[21]

References

^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
^ Liew, Amanda Ling Fung; Ting, Yonghan (2020-05-19). "Pachydermodactyly: A Case Report of a Little-Known and Benign Form of Digital Fibromatosis". American Journal of Case Reports. 21. International Scientific Information, Inc.: e923344. doi:10.12659/ajcr.923344. ISSN 1941-5923. PMC 7262483. PMID 32424112.
^ Vázquez Fernández, Ricardo; Maneiro Fernández, José Ramón; Cervantes Pérez, Evelin Cecilia; Mera Varela, Antonio (2020-10-01). "Pachydermodactyly: a systematic review". Irish Journal of Medical Science (1971 -). 190 (3). Springer Science and Business Media LLC: 1005–1014. doi:10.1007/s11845-020-02378-1. ISSN 0021-1265. PMID 33006046.
^ ^a ^b Beltraminelli, Helmut; Itin, Peter (2009). "Pachydermodactyly – Just a sign of emotional distress". European Journal of Dermatology. 19 (1): 005–013. doi:10.1684/ejd.2008.0543. ISSN 1167-1122. PMID 19059823.
^ Meunier, L.; Pailler, C.; Barneon, G.; Meynadier, J. (1994). "Pachydermodactyly or acquired digital fibromatosis". British Journal of Dermatology. 131 (5). Oxford University Press (OUP): 744–746. doi:10.1111/j.1365-2133.1994.tb05006.x. ISSN 0007-0963. PMID 7999628.
^ ^a ^b ^c ^d Plana Pla, A.; Bassas Vila, J.; Toro Montecinos, M.A.; Ferrandiz Foraster, C. (2014). "Pachydermodactyly Successfully Treated With Triamcinolone Injections". Actas Dermo-Sifiliográficas (English Edition). 105 (3). Elsevier BV: 319–321. doi:10.1016/j.adengl.2013.04.024. ISSN 1578-2190. PMID 24657023.
^ Dallos, Tomáš; Oppl, Bastian; Kovács, László; Zwerina, Jochen (2014). "Pachydermodactyly: A Review". Current Rheumatology Reports. 16 (9). doi:10.1007/s11926-014-0442-7. ISSN 1523-3774. PMID 25173950.
^ Sagransky, Matthew J.; Pichardo-Geisinger, Rita O.; Muñoz-Ali, Diana; Feldman, Steven R.; Mora, Dana C.; Quandt, Sara A. (2012-08-01). "Pachydermodactyly From Repetitive Motion in Poultry Processing Workers: A Report of 2 Cases". Archives of Dermatology. 148 (8): 925–928. doi:10.1001/archdermatol.2012.983. ISSN 0003-987X. PMID 22911188.
^ Morales Callaghan, A. M.; Horndler Argarate, C.; García Latasa de Araníbar, F. J.; Zubiri Ara, M. L. (2010). "[Pachydermodactyly: a rare form of acquired digital fibromatosis]". Actas Dermo-Sifiliograficas. 101 (7): 652–654. doi:10.1016/S1578-2190(10)70693-4. ISSN 1578-2190. PMID 20858397.
^ Lautenschlager, S.; Itin, P. H.; Rufli, T. (1994). "Pachydermodactyly: reflecting obsessive-compulsive behavior?". Archives of Dermatology. 130 (3): 387. doi:10.1001/archderm.1994.01690030123021. ISSN 0003-987X. PMID 8129422.
^ Barnes, Leandra A.; Bae, Gordon H.; Lewis, Matthew A.; Rieger, Kerri E. (2018). "Pachydermodactyly: Case report including clinical and histopathologic diagnostic pitfalls". Journal of Cutaneous Pathology. 45 (12): 949–953. doi:10.1111/cup.13359. ISSN 0303-6987. PMID 30221379.
^ Sinha, NK; Ling, SP; Nema, SK; Pai, DR (2013). "Pachydermodactyly does not need rheumatologic work-up". Journal of Postgraduate Medicine. 59 (4). Medknow: 335–336. doi:10.4103/0022-3859.123182. ISSN 0022-3859. PMID 24346401.
^ Leung, Alexander K.C.; Lam, Joseph M. (2021). "Pachydermodactyly". The Journal of Pediatrics. 236. Elsevier BV: 316–317. doi:10.1016/j.jpeds.2021.05.049. ISSN 0022-3476. PMID 34029598.
^ Żuber, Zbigniew; Dyduch, Grzegorz; Jaworek, Andrzej; Turowska-Heydel, Dorota; Sobczyk, Małgorzata; Banach-Górnicka, Marta; Rusnak, Katarzyna; Górecki, Wojciech (2016-06-30). "Pachydermodactyly – a report of two cases". Rheumatology. 54 (3). Termedia Sp. z.o.o.: 136–140. doi:10.5114/reum.2016.61215. ISSN 0034-6233. PMC 4967981. PMID 27504024.
^ Rachowska, Regina; Filipowska-Grońska, Agata; Weryńska-Kalemba, Maria; Bożek, Andrzej; Filipowska, Barbara; Zajęcki, Wojciech; Ilczuk, Danuta; Herba, Monika; Jarząb, Jerzy (2010). "Case report Pachydermodactyly – a case report". Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii (in Polish). 27 (6). Termedia. ISSN 1642-395X. Retrieved 2024-05-01.
^ Chen, Chen-Kuan; Shyur, Shyh-Dar; Chu, Szu-Hung; Huang, Li-Hsin; Kao, Yu-Hsuan; Liu, Ling-Chun (2015). "Pachydermodactyly: Three new cases in Taiwan". Journal of Microbiology, Immunology and Infection. 48 (3). Elsevier BV: 340–344. doi:10.1016/j.jmii.2012.09.002. ISSN 1684-1182. PMID 23218407.
^ Liu, Wei; Ma, Dong-Lai (2020). "Pachydermodactyly". Mayo Clinic Proceedings. 95 (10). Elsevier BV: 2280–2281. doi:10.1016/j.mayocp.2020.08.013. ISSN 0025-6196. PMID 33012355.
^ Hussain, Salman; Ehtesham, Maryam; Almas, Talal; Aldei, Ali (2021). "Painful pachydermodactyly in a 39-year-old woman: A case report and review of the literature". Annals of Medicine and Surgery. 70. Ovid Technologies (Wolters Kluwer Health): 102871. doi:10.1016/j.amsu.2021.102871. ISSN 2049-0801. PMC 8519772. PMID 34691419.
^ CHU, HOWARD; SONG, JUNGSIK; KIM, DO YOUNG (2016). "Pachydermodactyly: A Benign Cutaneous Condition that May Be Misdiagnosed as a Joint Disorder". The Journal of Rheumatology. 43 (8): 1615–1616. doi:10.3899/jrheum.160242. ISSN 0315-162X.
^ Tariq, Halla; Ihsan, Aroosha; Khan, Dr Asadullah; Shamim, Roshila (2023-01-25). "Pachydermodactyly, mimicker of rheumatoid hands, presents in a patient with Tuberous Sclerosis". Pakistan Journal of Medical Sciences. 39 (2): 624–627. doi:10.12669/pjms.39.2.6631. ISSN 1681-715X. PMC 10025706. PMID 36950423.
^ Kato, Naoki; Niitsu, Mamoru; Kawabe, Tetsuya; Arai, Eiichi; Fukumoto, Keizo (2015). "Surgical Management of Unilateral Soft Tissue Swelling around the Proximal Interphalangeal Joint in an Adolescent: A Case Report of Pachydermodactyly". Case Reports in Orthopedics. 2015. Hindawi Limited: 1–4. doi:10.1155/2015/242078. ISSN 2090-6749. PMC 4672109. PMID 26688765.