Pachydermodactyly Explained

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]

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. Intralesional triamcinolone injections have also been reported to alleviate symptoms.[6] Pachydermodactyly patients frequently have surgery to improve the appearance of a digit.[21]

See also

Further reading

External links

Notes and References

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