Successful Treatment of Lipoid Proteinosis in a Young Adult with Isotretinoin

Lipoid proteinosis (LP) is an extremely rare autosomal recessive genodermatosis, characterized by persistent hoarseness and classical skin lesions. Amorphous hyaline depositions may affect any organ, but mainly the face extremities. These cause limitations in tongue movement, hence, difficulty speaking. Biopsies of lesions usually show massive deposits homogeneous, acidic, hyaline-like material at epidermal junctions superficial dermis that stains positive for periodic acid-Schiff stain (PAS-D). At present, there no specific treatment this disease, some studies have reported with acitretin etretinate results improvement. This paper reports on effectiveness isotretinoin first time disease.