Stiff Skin Syndrome

A non-progressive autosomal dominant [MIM 184900] condition characterised by accumulation of hyaluronidase-digestible material and overexpression of extracellular matrix proteins
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Its strong association with diabetes has led to its name necrobiosis lipoidica diabeticorum), diabetic bullae, acquired perforating dermatosis (APD), granuloma annulare, oral lichen planus, vitiligo and stiff skin syndrome is not seen in our study.
Some of the cutaneous markers of diabetes mellitus like necrobiosis lipoidica diabeticorum, diabetic bullae, acquired perforating dermatosis and stiff skin syndrome is not seen in our study.
Winchester syndrome (OMIM 277950), systemic fibromatosis (OMIM 228550), stiff skin syndrome (OMIM 184900), lipoid proteinosis (OMIM 247100), and storage diseases including mucopolysacharoses, sphingolipidoses and mucilipidoses [Osterby et al.
Washington, Mar 20 (ANI): In a study, Johns Hopkins University School of Medicine researchers have shed light on a rare inherited disorder called stiff skin syndrome.
Instead we turned to a rare but inherited form of isolated skin fibrosis called stiff skin syndrome, hoping to gain a foothold regarding cellular mechanisms that might prove relevant to both conditions," says Dietz.
First, excess collagen is a hallmark feature of both stiff skin syndrome and scleroderma.
So Dietz's team examined patients with stiff skin syndrome and found them to have excessive amounts of fibrillin-1 in the skin.