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

Lipoid proteinosis (Urbach-Wiethe disease) in two siblings


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
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.146168

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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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