Definition: Dupuytren’s disease is a superficial fibromatosis of the hand. It is a nodular, cytologically bland fibroproliferative process that involves the palmar aponeuroses.
Epidemiology
Peak age 60-70 years
Rare under 20
May be bilateral
Predominantly seen in patients of northwest European origin
In some populations may affect as many as 20% of males and 4% of females
Familial predisposition
Familial form: Generally appears to be autosomal dominant
May be associated with other fibromatoses
- Plantar fibromatosis in 10-20% of cases
- Penile fibromatosis in 1% of cases
Clinical synopsis
May cause contractures
With or without pain
Knuckle pads
Clinical associations
Epilepsy
Diabetes
Alcohol abuse and cirrhosis
Smoking
Manual labor
Localization
Most cases palmar
Rare cases involve dorsum of hand or wrist
Multinodular
Most often involves palm
May rarely involve dorsum of hand or wrist
Involves aponeurosis and rarely overlying dermis
No infiltration of deeper tissues
Synopsis
Grossly forms multiple nodules 1 cm or less
Infrequently forms a single dominant nodule
Cellularity ranges from low to high
Older lesions are densely collagenous
Bland spindle cells
Nuclei small and dark to moderate sized and vesicular
Scant cytoplasm
Mitotic figures may be frequent but not atypical
Small foci of perivascular hemorrhage and hemosiderin may be present
Metaplastic cartilage and bone are seen rarely
Necrosis very rare
Immunochemistry
Actin: Variable positivity
Desmin: Variable positivity
S-100: negative
Beta-catenin: 50% positive
Differential diagnosis
calcifying aponeurotic fibroma
extra-abdominal desmoid fibromatosis
adult-type fibrosarcoma
synovial sarcoma
epithelioid sarcoma
inclusion body fibromatosis (infantile fibromatosis)
Prognosis
Never metastasizes
May regress with age
Recurrences do occur but are not destructive
CGH
No gene copy number changes in Dupuytren’s contracture by array comparative genomic hybridization (18474290).
Credits
See also
fibromatoses (fibromatosis)
Book sections
Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 30, 2001
Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone, World Health Organization Classification of Tumours 2002
Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors, 4th edition, 2001
References
Kaur S, Forsman M, Ryhänen J, Knuutila S, Larramendy ML. No gene copy number changes in Dupuytren’s contracture by array comparative genomic hybridization. Cancer Genet Cytogenet. 2008 May;183(1):6-8. PMID: 18474290
Gudmundsson KG, Arngrimsson R, Sigfusson N, Bjornsson A, Jonsson T. Epidemiology of Dupuytren’s disease: clinical, serological, and social assessment. The Reykjavik Study. J Clin Epidemiol 2000 Mar 1;53(3):291-6
Ushijima M, Tsuneyoshi M, Enjoji M. Dupuytren type fibromatoses. A clinicopathologic study of 62 cases. Acta Pathol Jpn 1984 Sep;34(5):991-1001
Iwasaki H, Muller H, Stutte HJ, Brennscheidt U. Palmar fibromatosis (Dupuytren’s contracture). Ultrastructural and enzyme histochemical studies of 43 cases. Virchows Arch A Pathol Anat Histopathol 1984;405(1):41-53
Montgomery E, Lee JH, Abraham SC, Wu TT. Superficial fibromatoses are genetically distinct from deep fibromatoses. Mod Pathol 2001 Jul;14(7):695-701
Mikkelsen OA. Dupuytren’s disease—initial symptoms, age of onset and spontaneous course. Hand 1977 Feb;9(1):11-5
Allen PW. The fibromatoses: a clinicopathologic classification based on 140 cases. Am J Surg Pathol. 1977 Sep;1(3):255-70.