STIFF SKIN SYNDROME - A CASE-REPORT AND REVIEW OF THE LITERATURE

Stiff skin syndrome (SSS) is a rare disorder characterized by stony-ha rd skin, limited joint mobility and mild hypertrichosis. We present a severe case in a 4-year-old boy. In addition to the clinical and histo logical features of SSS, our patient also had muscle and bone involvem ent along with pyloric stenosis, gastro-esophageal reflux, inguinal he rnias and atopic dermatitis. We highlight the complexity of this case and the diagnostic approach to patients with this disorder, We also re view and summarize the 24 cases previously reported in the literature.