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Review
. 2022 Apr-Jun;26(2):236-241.
doi: 10.4103/jomfp.jomfp_249_21. Epub 2022 Jun 28.

Lipoid proteinosis: Review of Indian cases

Jigna S Shah  1 , Himali A Shah  1
Affiliations
Review

Lipoid proteinosis: Review of Indian cases

Jigna S Shah et al. J Oral Maxillofac Pathol. 2022 Apr-Jun.

Abstract

Lipoid proteinosis (LP) is a rare autosomal recessive disorder characterized by the deposition of amorphous hyaline material in the dermis and submucosal connective tissue. To date <500 cases of LP have been described and oral manifestations described in a very few reports. Indian cases are much less reported and reviewed. Hence, here review of 51 Indian LP cases along with a case of histologically proven LP in 12-year-old male patient with typical skin, ocular, laryngeal, oral and radiographic features is done. Cases from 1969 to 2021 were collected using keyword LP on google and google scholar and Indian cases were analyzed afterward. Review with case presentation regarding oral manifestations will help the oral physician to diagnose LP in early stage.

Keywords: Indian cases; lipoid proteinosis; oral manifestations; oral physician; review.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Showing string of beads appearance over eyelids (moniliform blepharosis). (b) Showing pox-like scarring on forehead
Figure 2
Figure 2
(a) Showing pox like scarring on legs. (b) Showing pox like scarring on forearms
Figure 3
Figure 3
Showing similar skin lesions on back and buttocks
Figure 4
Figure 4
(a) Showing thickened tongue with diffuse ulceration and slightly thickened uvula. (b) Showing thickened sublingual frenum with reduced tongue movement
Figure 5
Figure 5
(a) Showing Thickened lower labial mucosa with minute depressions and elevation giving pebbled appearance. (b) Showing crusting and fissuring seen on both lips
Figure 6
Figure 6
(a) Orthopantomogram showing absence of all 2nd premolars. Erupting all canine and 1st premolars and 2nd molars, and erupted left maxillary 1st premolar with normal teeth structure. (b) Lateral cephalogram showing bean-shaped calcification in suprasellar region
Figure 7
Figure 7
Showing total regression of tongue lesion after 1 month
See this image and copyright information in PMC

References

    1. Nayak S, Acharjya B. Lipoid proteinosis in a six-year-old child. Indian Dermatol Online J. 2012;3:25–7. - PMC - PubMed
    1. Hamada T, McLean WH, Ramsay M, Ashton GH, Nanda A, Jenkins T, et al. Lipoid proteinosis maps to 1q21 and is caused by mutations in the extracellular matrix protein 1 gene (ECM1) Hum Mol Genet. 2002;11:833–40. - PubMed
    1. Lee KC, Peters SM, Ko YC, Kunkle TC, Perrino MA, Yoon AJ, et al. Oral manifestations of lipoid proteinosis in a 10-year-old female: A case report and literature update. Oral Surg Oral Med Oral Pathol Oral Radiol. 2018;126:e228–32. - PubMed
    1. Ravi Prakash SM, Verma S, Sumalatha MN, Chattopadhyay S. Oral manifestations of lipoid proteinosis: A case report and literature review. Saudi Dent J. 2013;25:91–4. - PMC - PubMed
    1. Kachewar S, Singh H, Sasane AG, Bhadane S. Full blown case of lipoid proteinosis. Med J Armed Forces India. 2011;67:90–1. - PMC - PubMed