Lipoid proteinosis with pseudomembranous conjunctivitis.

Abstract:

:Lipoid proteinosis is a rare autosomal recessive condition characterized by a diffuse mucocutaneous infiltration with histopathologic deposits that are positive for periodic acid-Schiff reagent and a sudanophil substance. We present a 9-year-old boy with lipoid proteinosis. His parents are siblings. He had classical manifestations, such as statural-ponderal delay, hoarseness, yellowish skin papules, atrophic scars, and moniliform blepharosis. A transmission deafness was also found. There were none of the intracranial calcifications that are usual at this age. A pseudomembranous conjunctivitis was surgically treated when he was 4 months old. This clinical manifestation has not hitherto been described in lipoid proteinosis.

journal_name

J Am Acad Dermatol

authors

Barthelemy H,Mauduit G,Kanitakis J,Cambazard F,Thivolet J

doi

10.1016/s0190-9622(86)70045-5

subject

Has Abstract

pub_date

1986-02-01 00:00:00

pages

367-71

issue

2 Pt 2

eissn

0190-9622

issn

1097-6787

pii

S0190-9622(86)70045-5

journal_volume

14

pub_type

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