Lipoid proteinosis: Review of Indian cases

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.

Figure 1

(a) Showing string of beads appearance over eyelids (moniliform blepharosis). (b) Showing pox-like scarring on forehead

Figure 2

(a) Showing pox like scarring on legs. (b) Showing pox like scarring on forearms

Figure 3

Showing similar skin lesions on back and buttocks

Figure 4

(a) Showing thickened tongue with diffuse ulceration and slightly thickened uvula. (b) Showing thickened sublingual frenum with reduced tongue movement

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

(a) Orthopantomogram showing absence of all 2^nd premolars. Erupting all canine and 1^st premolars and 2^nd molars, and erupted left maxillary 1^st premolar with normal teeth structure. (b) Lateral cephalogram showing bean-shaped calcification in suprasellar region

Figure 7

Showing total regression of tongue lesion after 1 month