Acquired cutis laxa: an unusual skin manifestation of systemic amyloidosis

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Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis in a patient with multiple myeloma*

A 57-year-old woman presented with periorbital ecchymoses, laxity in skin folds, polyneuropathy and bilateral carpal tunnel syndrome. A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain, consistent with cutis laxa. The diagnosis of primary systemic amyloidosis was made by the presence of amyloid material in the eyelid using hist...

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Cutis Laxa Skin Fibroblast Strain

Skin fibroblasts from two cases of autosomal recessive cutis laxa (CL), having insignificant elastin production and mRNA levels, were challenged with transforming growth factor beta-i (TGF-fi1). Elastin production was brought from undetectable values to amounts typical of normal human skin fibroblasts in a dose-dependent fashion. Basic fibroblast growth factor (100 ng/ml) alone or in combinatio...

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Biomechanical Properties of the Skin in Cutis Laxa

Cutis laxa (CL) is a heterogeneous group of disorders characterized by loose, redundant, inelastic or prematurely wrinkled skin (Berk et al., 2012; Uitto et al., 2013). Several inherited forms of CL have been identified (Urban and Davis, 2013), with 9 causative genes known to date (ALDH18A1, ATP6V0A2, ATP7A, EFEMP2/FBLN4, ELN, FBLN5, LTBP4, PYCR1, RIN2). A shared feature of all types of inherit...

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Cutis laxa autosomal recessive type II or wrinkly skin syndrome?

1. Gutte R, Khopkar U. Predominant palmoplantar lichen planus: A diagnostic challenge. Indian J Dermatol 2014;59:343‐7. 2. Pavitran K, Karunakaram M, Palit A, Raghunatha S. On disorders of keratinisation. In: Valia RG, Valia AR, editors. IADVL Textbook of Dermatology. 3rd ed. Mumbai, India: Bhalani Publishers; 2008. p. 995‐1069. 3. Gutte RM. Unilateral acrosyringeal lichen planus of palm. India...

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Acquired Localized Cutis Laxa due to Increased Elastin Turnover

Cutis laxa is a rare disease characterized by abnormal skin wrinkling and laxity, due to decreased elastin synthesis or structural extracellular matrix defects. We have explored elastin metabolism in a case of adult onset cutis laxa localized to the upper body of a woman. For this purpose, we obtained skin biopsies from affected and unaffected skin areas of the patient and analyzed these with m...

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ژورنال

عنوان ژورنال: Galle Medical Journal

سال: 2012

ISSN: 1391-7072

DOI: 10.4038/gmj.v17i1.4362