Pachydermodactyly

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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.Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. {{ISBN|0-07-138076-0}}.{{rp|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.{{cite journal | last1=Liew | first1=Amanda Ling Fung | last2=Ting | first2=Yonghan | title=Pachydermodactyly: A Case Report of a Little-Known and Benign Form of Digital Fibromatosis | journal=American Journal of Case Reports | publisher=International Scientific Information, Inc. | volume=21 | date=2020-05-19 | pages=e923344 | issn=1941-5923 | doi=10.12659/ajcr.923344 | pmid=32424112 | pmc=7262483 }}{{cite journal | last1=Vázquez Fernández | first1=Ricardo | last2=Maneiro Fernández | first2=José Ramón | last3=Cervantes Pérez | first3=Evelin Cecilia | last4=Mera Varela | first4=Antonio | title=Pachydermodactyly: a systematic review | journal=Irish Journal of Medical Science (1971 -) | publisher=Springer Science and Business Media LLC | volume=190 | issue=3 | date=2020-10-01 | issn=0021-1265 | doi=10.1007/s11845-020-02378-1 | pages=1005–1014| pmid=33006046 }} 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.{{cite journal | last1=Beltraminelli | first1=Helmut | last2=Itin | first2=Peter | title=Pachydermodactyly – Just a sign of emotional distress | journal=European Journal of Dermatology | volume=19 | issue=1 | date=2009 | issn=1167-1122 | doi=10.1684/ejd.2008.0543 | pages=005–013| pmid=19059823 }} Although moderate erythema, fine desquamation, or lichenification may occasionally be seen, epidermal alterations are not typically present.{{cite journal | last1=Meunier | first1=L. | last2=Pailler | first2=C. | last3=Barneon | first3=G. | last4=Meynadier | first4=J. | title=Pachydermodactyly or acquired digital fibromatosis | journal=British Journal of Dermatology | publisher=Oxford University Press (OUP) | volume=131 | issue=5 | year=1994 | issn=0007-0963 | doi=10.1111/j.1365-2133.1994.tb05006.x | pages=744–746| pmid=7999628 }} There is no impact on hand movements.{{cite journal | last1=Plana Pla | first1=A. | last2=Bassas Vila | first2=J. | last3=Toro Montecinos | first3=M.A. | last4=Ferrandiz Foraster | first4=C. | title=Pachydermodactyly Successfully Treated With Triamcinolone Injections | journal=Actas Dermo-Sifiliográficas (English Edition) | publisher=Elsevier BV | volume=105 | issue=3 | year=2014 | issn=1578-2190 | doi=10.1016/j.adengl.2013.04.024 | pages=319–321| pmid=24657023 | doi-access=free }}

Causes

Although the specific cause and etiology are yet unknown, excessive mechanical manipulation of PIP joints is likely to be a contributing factor.{{cite journal | last1=Dallos | first1=Tomáš | last2=Oppl | first2=Bastian | last3=Kovács | first3=László | last4=Zwerina | first4=Jochen | title=Pachydermodactyly: A Review | journal=Current Rheumatology Reports | volume=16 | issue=9 | date=2014 | issn=1523-3774 | doi=10.1007/s11926-014-0442-7 | page=| pmid=25173950 }} The basis for this suggestion is the discovery that pachydermodactyly is related to a variety of jobs and pursuits, including weightlifting, employment in food processing facilities,{{cite journal | last1=Sagransky | first1=Matthew J. | last2=Pichardo-Geisinger | first2=Rita O. | last3=Muñoz-Ali | first3=Diana | last4=Feldman | first4=Steven R. | last5=Mora | first5=Dana C. | last6=Quandt | first6=Sara A. | title=Pachydermodactyly From Repetitive Motion in Poultry Processing Workers: A Report of 2 Cases | journal=Archives of Dermatology | volume=148 | issue=8 | date=2012-08-01 | pages=925–928 | issn=0003-987X | doi=10.1001/archdermatol.2012.983 | pmid=22911188 }} martial arts, climbing,{{cite journal | last1=Morales Callaghan | first1=A. M. | last2=Horndler Argarate | first2=C. | last3=García Latasa de Araníbar | first3=F. J. | last4=Zubiri Ara | first4=M. L. | title=[Pachydermodactyly: a rare form of acquired digital fibromatosis] | journal=Actas Dermo-Sifiliograficas | volume=101 | issue=7 | date=2010 | issn=1578-2190 | pmid=20858397 | pages=652–654| doi=10.1016/S1578-2190(10)70693-4 | doi-access=free }} and individuals with OCD who have hand tics.{{cite journal | last1=Lautenschlager | first1=S. | last2=Itin | first2=P. H. | last3=Rufli | first3=T. | title=Pachydermodactyly: reflecting obsessive-compulsive behavior? | journal=Archives of Dermatology | volume=130 | issue=3 | date=1994 | issn=0003-987X | pmid=8129422 | doi=10.1001/archderm.1994.01690030123021 | page=387}}

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.{{cite journal | last1=Barnes | first1=Leandra A. | last2=Bae | first2=Gordon H. | last3=Lewis | first3=Matthew A. | last4=Rieger | first4=Kerri E. | title=Pachydermodactyly: Case report including clinical and histopathologic diagnostic pitfalls | journal=Journal of Cutaneous Pathology | volume=45 | issue=12 | date=2018 | issn=0303-6987 | doi=10.1111/cup.13359 | pages=949–953| pmid=30221379 }} 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.{{cite journal | last1=Sinha | first1=NK | last2=Ling | first2=SP | last3=Nema | first3=SK | last4=Pai | first4=DR | title=Pachydermodactyly does not need rheumatologic work-up | journal=Journal of Postgraduate Medicine | publisher=Medknow | volume=59 | issue=4 | year=2013 | issn=0022-3859 | doi=10.4103/0022-3859.123182 | doi-access=free | pages=335–336| pmid=24346401 }}

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. In addition, there may be a decrease in mucin and elastic fibers, as well as hyperkeratosis (orthokeratosis or parakeratosis).

Hand radiographs usually show soft tissue swelling without interphalangeal joint structural involvement.{{cite journal | last1=Leung | first1=Alexander K.C. | last2=Lam | first2=Joseph M. | title=Pachydermodactyly | journal=The Journal of Pediatrics | publisher=Elsevier BV | volume=236 | year=2021 | issn=0022-3476 | doi=10.1016/j.jpeds.2021.05.049 | pages=316–317| pmid=34029598 }} An X-ray examination shows no abnormalities in the periosteum or bones in pachydermodactyly patients.{{cite journal | last1=Żuber | first1=Zbigniew | last2=Dyduch | first2=Grzegorz | last3=Jaworek | first3=Andrzej | last4=Turowska-Heydel | first4=Dorota | last5=Sobczyk | first5=Małgorzata | last6=Banach-Górnicka | first6=Marta | last7=Rusnak | first7=Katarzyna | last8=Górecki | first8=Wojciech | title=Pachydermodactyly – a report of two cases | journal=Rheumatology | publisher=Termedia Sp. z.o.o. | volume=54 | issue=3 | date=2016-06-30 | issn=0034-6233 | doi=10.5114/reum.2016.61215 | pages=136–140| pmid=27504024 | pmc=4967981 }} Additionally, no changes are seen in the synovium or joints during ultrasound.{{cite journal | last1=Rachowska | first1=Regina | last2=Filipowska-Grońska | first2=Agata | last3=Weryńska-Kalemba | first3=Maria | last4=Bożek | first4=Andrzej | last5=Filipowska | first5=Barbara | last6=Zajęcki | first6=Wojciech | last7=Ilczuk | first7=Danuta | last8=Herba | first8=Monika | last9=Jarząb | first9=Jerzy | title=Case report Pachydermodactyly – a case report | journal=Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii | publisher=Termedia | volume=27 | issue=6 | date=2010 | issn=1642-395X |url=https://www.termedia.pl/Case-report-Pachydermodactyly-a-case-report,7,15962,0,1.html | language=pl | access-date=2024-05-01 | page=}}

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.{{cite journal | last1=Chen | first1=Chen-Kuan | last2=Shyur | first2=Shyh-Dar | last3=Chu | first3=Szu-Hung | last4=Huang | first4=Li-Hsin | last5=Kao | first5=Yu-Hsuan | last6=Liu | first6=Ling-Chun | title=Pachydermodactyly: Three new cases in Taiwan | journal=Journal of Microbiology, Immunology and Infection | publisher=Elsevier BV | volume=48 | issue=3 | year=2015 | issn=1684-1182 | doi=10.1016/j.jmii.2012.09.002 | pages=340–344| pmid=23218407 | doi-access=free }}

Differential diagnoses include rheumatoid arthritis, acromegaly, juvenile idiopathic arthritis, pachydermoperiostosis, knuckle pads, and juvenile digital fibromatosis.{{cite journal | last1=Liu | first1=Wei | last2=Ma | first2=Dong-Lai | title=Pachydermodactyly | journal=Mayo Clinic Proceedings | publisher=Elsevier BV | volume=95 | issue=10 | year=2020 | issn=0025-6196 | doi=10.1016/j.mayocp.2020.08.013 | pages=2280–2281| pmid=33012355 }}

Treatment

Pachydermodactyly does not currently have a commonly accepted treatment.{{cite journal | last1=Hussain | first1=Salman | last2=Ehtesham | first2=Maryam | last3=Almas | first3=Talal | last4=Aldei | first4=Ali | title=Painful pachydermodactyly in a 39-year-old woman: A case report and review of the literature | journal=Annals of Medicine and Surgery | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=70 | year=2021 | issn=2049-0801 | doi=10.1016/j.amsu.2021.102871 | page=102871| pmid=34691419 | pmc=8519772 }} Nevertheless, aggressive therapy is usually not necessary due to the disease's benign course.{{cite journal | last1=CHU | first1=HOWARD | last2=SONG | first2=JUNGSIK | last3=KIM | first3=DO YOUNG | title=Pachydermodactyly: A Benign Cutaneous Condition that May Be Misdiagnosed as a Joint Disorder | journal=The Journal of Rheumatology | volume=43 | issue=8 | year=2016 | issn=0315-162X | doi=10.3899/jrheum.160242 | pages=1615–1616}} Since the majority of pachydermodactyly cases appear with symptoms caused by recurrent joint trauma, discontinuing stimulating activities is sufficient to promote regression or stabilization.{{cite journal | last1=Tariq | first1=Halla | last2=Ihsan | first2=Aroosha | last3=Khan | first3=Dr Asadullah | last4=Shamim | first4=Roshila | title=Pachydermodactyly, mimicker of rheumatoid hands, presents in a patient with Tuberous Sclerosis | journal=Pakistan Journal of Medical Sciences | volume=39 | issue=2 | date=2023-01-25 | pages=624–627 | issn=1681-715X | doi=10.12669/pjms.39.2.6631 | pmid=36950423 | pmc=10025706 }} It has also been demonstrated that intralesional corticosteroids can sometimes reduce swelling.{{Cn|date=May 2024}} Intralesional triamcinolone injections have also been reported to alleviate symptoms. Pachydermodactyly patients frequently have surgery to improve the appearance of a digit.{{cite journal | last1=Kato | first1=Naoki | last2=Niitsu | first2=Mamoru | last3=Kawabe | first3=Tetsuya | last4=Arai | first4=Eiichi | last5=Fukumoto | first5=Keizo | title=Surgical Management of Unilateral Soft Tissue Swelling around the Proximal Interphalangeal Joint in an Adolescent: A Case Report of Pachydermodactyly | journal=Case Reports in Orthopedics | publisher=Hindawi Limited | volume=2015 | year=2015 | issn=2090-6749 | doi=10.1155/2015/242078 | doi-access=free | pages=1–4| pmid=26688765 | pmc=4672109 }}

References

External links

[https://dermnetnz.org/topics/pachydermodactyly DermNet]
[https://www.visualdx.com/visualdx/diagnosis/pachydermodactyly?diagnosisId=53242&moduleId=101 VisualDx]

{{Medical resources

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Category:Dermal and subcutaneous growths