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

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