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ABSTRACT Lipoid proteinosis is a rare genodermatosis characterized by hyaline deposition in the skin, mucosae, and visceral organs. This condition manifests with a wide range of muco-cutaneous and systemic features. Herein, we present a series of four cases diagnosed with lipoid proteinosis exhibiting distinct clinical features. All patients had characteristic moniliform blepharosis. One patient had neuropsychiatric features. Histopathological confirmation was obtained in two cases. Three of the patients received systemic retinoids and one patient was treated with erbium yttrium aluminum Garnett (Er: YAG) laser in addition for facial scars. Although the condition is generally benign, with a normal life expectancy, the cutaneous and systemic manifestations may often result in significant psychosocial morbidity. Management entails a multidisciplinary approach and presents a therapeutic challenge due to lack of effective treatment modalities.