Sclerodactyly
| Sclerodactyly | |
|---|---|
| Specialty | Dermatology  |
Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes that yields a characteristic claw-like appearance and spindle shape of the affected digits, and renders them immobile or of limited mobility.[1][2] The thickened, discolored patches of skin are called morphea, and may involve connective tissue below the skin, as well as muscle and other tissues.[3] Sclerodactyly is often preceded by months or even years by Raynaud's phenomenon when it is part of systemic scleroderma.[citation needed]
The term "sclerodactyly" comes from Greek skleros 'hard' and daktylos 'digit'.
It is generally associated with systemic scleroderma and mixed connective tissue disease, and auto-immune disorders.
Sclerodactyly is one component of the limited cutaneous form of systemic sclerosis (lcSSc), also known as CREST syndrome (CREST is an acronym that stands for calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.)[4] Sclerodactyly is also one component of Huriez Syndrome, along with palmoplantar keratoderma and skin cancer.[5] Sclerodactyly sometimes arises as a complication of the microvascular changes seen in diabetes mellitus, and is in this case referred to as diabetic sclerodactyly.[6][7][8]
Treatment of sclerodactyly is by physical therapy, phototherapy, surgery, topical corticosteroids or vitamin D analogues, and systemic immunosuppressive drugs when the condition is part of systemic scleroderma[citation needed]. Localized treatment won't halt systemic disease, but can restore function and cosmetic aspects of the affected digits. The mild to moderate proximal interphalangeal (PIP) joint flexion and extension contractures and stiff distal interphalangeal (DIP) joints in slight flexion often seen in sclerodactyly can be addressed somewhat with physical therapy.[9] In a few cases when immune involvement isn't apparent (in these cases environmental causes are suspected), the condition may gradually clear up by itself If the trigger is avoided[citation needed]. In other cases, early treatment while the disease is in the inflammatory stage is much more likely to be successful than on established lesions[citation needed].
See also
[edit]References
[edit]- ^ "Sclerodactyly: MedlinePlus Medical Encyclopedia Image". medlineplus.gov. Retrieved 2022-08-12.
- ^ "Systemic sclerosis | DermNet NZ". dermnetnz.org. Retrieved 2022-08-12.
- ^ Fett, Nicole M. (2013-09-01). "Morphea (Localized Scleroderma)". JAMA Dermatology. 149 (9): 1124. doi:10.1001/jamadermatol.2013.5079. ISSN 2168-6068.
- ^ "CREST syndrome: MedlinePlus Medical Encyclopedia Image". medlineplus.gov. Retrieved 2022-08-12.
- ^ Patrizi, A.; Di Lernia, V.; Patrone, P. (May 1992). "Palmoplantar keratoderma with sclerodactyly (Huriez syndrome)". Journal of the American Academy of Dermatology. 26 (5 Pt 2): 855–857. doi:10.1016/0190-9622(92)70121-u. ISSN 0190-9622. PMID 1613149.
- ^ Gruson, Lisa Moed; Franks, Andrew (2005-12-30). "Scleredema and diabetic sclerodactyly". Dermatology Online Journal. 11 (4): 3. doi:10.5070/D32165S0J0. ISSN 1087-2108. PMID 16403375.
- ^ Tüzün, B.; Tüzün, Y.; Dinççağ, N.; Minareci, O.; Oztürk, S.; Yilmaz, M. T.; Satman, I.; Yazici, H. (February 1995). "Diabetic sclerodactyly". Diabetes Research and Clinical Practice. 27 (2): 153–157. doi:10.1016/0168-8227(94)01023-s. ISSN 0168-8227. PMID 7607054.
- ^ Wielosz Kurowska Suszek Majdan (2017). "Coexistence of diabetes mellitus type 1 with diffuse systemic sclerosis – case report and literature review". Reumatologia. 55 (2): 104–107. doi:10.5114/reum.2017.67607. PMC 5442303. PMID 28539684.
- ^ Skirven, Terri (2021). Rehabilitation of the Hand and Upper Extremity (7th ed.). Elsevier.
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