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. 2013 Apr;25(2):91-4.
doi: 10.1016/j.sdentj.2012.12.004. Epub 2013 Feb 20.

Oral manifestations of lipoid proteinosis: A case report and literature review

Affiliations

Oral manifestations of lipoid proteinosis: A case report and literature review

S M Ravi Prakash et al. Saudi Dent J. 2013 Apr.

Abstract

Lipoid proteinosis is an uncommon autosomal recessive metabolic disorder that presents in early life with hoarseness and pox-like acneiform scars involving the skin and mucous membranes. Previous studies have attributed the prevalence of lipoid proteinosis to consanguineous parents. This paper reports a classical case of lipoid proteinosis with oral manifestations but without a history of consanguinity.

Keywords: Acneiform scars; Consanguinity; Hoarseness; Lipoid proteinosis; Monoliform blepharosis.

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Figures

Figure 1
Figure 1
Photograph of the patient showing acneiform scars on the face with beaded papules on the margins of the eyelids.
Figure 2
Figure 2
Photograph showing lacerations on the upper and lower lip with cobblestone appearance at the dorsum and restricted protrusion of the tongue.
Figure 3
Figure 3
Photograph showing hyperkeratosis on the left buccal mucosa, giving a thickened and nodular appearance.
Figure 4
Figure 4
Photograph showing hyperkeratosis on the right buccal mucosa, giving a thickened and nodular appearance.
Figure 5
Figure 5
Photograph of the hematoxylin and eosin stained specimen showing acanthosis, hyperkeratosis, parakeratosis and koilocytosis with focal areas of hyaline eosinophillic material deposits (10× magnifications).
None

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