Lipoid proteinosis

A 21-year-old man presented with a complaint of hoarseness as well as lesio ns along the eyelids and on his knees, elbows, and fingers. Hoarseness had developed in childhood, followed by lesions along the eyelids and on the el bows and fingers. He had developed lesions on the knees, feet, scrotum, pen is, and axilla over the last 2 years. His parents were relatives, but nobod y in the family showed similar features. His physical examination was normal. On dermatologic examination, there wer e beaded papules along the eyelids and small, yellow-white infiltrations on the tongue, buccal mucosa, palate, scrotum, and penis (Fig. 1). His tongue was firm and its mobility was limited. He also had infiltration of the fre nulum (Fig. 2) and warty and hyperkeratotic papular and nodular lesions on the hands, knees, elbows (Fig. 3), and axilla. The laboratory findings and laryngoscopic examination were normal. Ophthalm ologic, neurologic, and psychiatric examinations were also normal. Electroe ncephalogram (EEG), electromyogram (EMG), craniography, and cranial compute d tomography (CT) scanning revealed no abnormality. The histopathologic examination of the skin biopsy specimens obtained from the axilla, elbow, and dorsum of the hand showed hyperkeratosis and periodi c acid-Schiff-positive (PAS(+)) staining hyaline material around the dermal capillaries and sweat glands, and confirmed the diagnosis of lipoid protei nosis.