Lipoid proteinosis (Urbach-Wiethe disease) in two siblings

Rekha Thaddanee; Ajeet Kumar Khilnani; Purna Pandya; Mayank Chaturvedi

doi:10.4103/2229-5178.146168

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Year : 2014 | Volume : 5 | Issue : 6 | Page : 95-97

Lipoid proteinosis (Urbach-Wiethe disease) in two siblings

Rekha Thaddanee¹, Ajeet Kumar Khilnani², Purna Pandya³, Mayank Chaturvedi⁴
¹ Department of Pediatrics, Gujarat Medical Education and Research Society Medical College and Hospital, Dharpur, Patan, Gujarat, India
² Department of Ear, Nose and Throat, Gujarat Medical Education and Research Society Medical College and Hospital, Dharpur, Patan, Gujarat, India
³ Department of Dermatology, Gujarat Medical Education and Research Society Medical College and Hospital, Dharpur, Patan, Gujarat, India
⁴ Department of Dentistry, Gujarat Medical Education and Research Society Medical College and Hospital, Dharpur, Patan, Gujarat, India

Correspondence Address:
Dr. Rekha Thaddanee
Department of Pediatrics, Gujarat Medical Education and Research Society Medical College and Hospital, Dharpur, Patan - 384 265, Gujarat
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2229-5178.146168

Lipoid proteinosis is a very rare autosomal recessive disorder characterized by deposition of hyaline material in the skin and the upper aerodigestive tract. Hoarseness of voice occurs very early in life and airway obstruction may occur. Characteristic skin lesions include multiple brown atrophic scars over face and distal extremities, beaded papules over the margins of the eyelids and verrucous nodules over the friction bearing areas (elbows, knees). The overall prognosis is good. There is no definitive treatment.

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