Lipoid proteinosis [Urbach-Writhe disease]

In this report, we describe a 26-year-old man who presented with hyperpigmented firm papillomatous growths along the eye lids, back of the neck, axilla, lateral sides of the upper arms and pubic region. He had macroglossia with xerostomia. The pharyngeal mucosa including the posterior pharyngeal wall and tonsils showed infiltration by yellowish grayish material. The patient's voice was hoarse due to thickening of the vocal cords with narrowing of the laryngeal inlet. Both the parotid glands were enlarged, firm and had pneumoparatoid sensation on massaging. Biopsy of the skin lesions showed hyperkeratosis, acanthosis and papillomatosis. The dermis showed homogenous bundles of eosinophilic hyaline material with mantles of similar material surrounding the blood vessels, hair follicles and sweat glands. Parotid sialogram showed grade 3-4 sialectasia. These findings are consistent with "lipoid proteinosis" which is a rare disease with an autosomal recessive mode of inheritance. It usually presents at childhood. The late presentation of this disease in the patient described is unique and has not been reported previously

Hypopigmented macules in leprosy--a histopathological and histochemical study of melanocytes.

Study of the number of melanocytes and amount of pigmentation in hypopigmented lesions and adjacent normal areas in 20 leprosy patients showed no differences in these parameters. It appears that hypopigmentation in leprosy lesions could be caused by defective transfer of melanin into keratinocytes.