lipoid proteinosis: a case report urbach & wiethe disease

lipoid proteinosis (lp) is a rare autosomal recessive disease characterized by the deposition of an amorphous hyaline material in the skin, mucosa and viscera. the classic manifestation is onset in infancy with a hoarse cry due to laryngeal infiltration. skin and mucous changes develope, and the disease follows a slowly progressive course. in this case report, a 49 year-old man presented with a longstanding hoarseness since childhood, dysphagia and asymptomatic skin lesions. esophageal biopsy showed the deposition of homogenous eosinophilic hyaline-like material compatible with lp.