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Lipoid proteinosis: a rare encounter in dental office.

Deshpande P, Guledgud MV, Patil K, Hegde U, Sahni A, Huchanahalli Sheshanna S - Case Rep Dent (2015)

Bottom Line: Lipoid proteinosis is a sporadic congenital metabolic disorder which is characterized by deposition of hyaline material in dermis, submucosal connective tissue, and various internal organs.It has an extremely low prevalence rate with less than 300 cases reported so far.This progressive disease has a vast spectrum of manifestations ranging from asymptomatic lesions to fatal seizures and respiratory obstruction making timely diagnosis of this rare disorder an imperative task for oral health care practitioners.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine & Radiology, JSS Dental College and Hospital, JSS University, Mysore, Karnataka 570015, India.

ABSTRACT
Lipoid proteinosis is a sporadic congenital metabolic disorder which is characterized by deposition of hyaline material in dermis, submucosal connective tissue, and various internal organs. It has an extremely low prevalence rate with less than 300 cases reported so far. This progressive disease has a vast spectrum of manifestations ranging from asymptomatic lesions to fatal seizures and respiratory obstruction making timely diagnosis of this rare disorder an imperative task for oral health care practitioners. We report a case of characteristic oral manifestations of lipoid proteinosis in a 28-year-old male patient along with a review of relevant prevailing literature.

No MeSH data available.


Related in: MedlinePlus

Waxy beaded papules on the margins of the eyelids.
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fig1: Waxy beaded papules on the margins of the eyelids.

Mentions: On general physical examination he was found to be moderately built and nourished. There was presence of waxy beaded papules on the margins of both eyelids (Figure 1) and areas of hyperkeratosis on the dorsal surface of the hands.


Lipoid proteinosis: a rare encounter in dental office.

Deshpande P, Guledgud MV, Patil K, Hegde U, Sahni A, Huchanahalli Sheshanna S - Case Rep Dent (2015)

Waxy beaded papules on the margins of the eyelids.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4383431&req=5

fig1: Waxy beaded papules on the margins of the eyelids.
Mentions: On general physical examination he was found to be moderately built and nourished. There was presence of waxy beaded papules on the margins of both eyelids (Figure 1) and areas of hyperkeratosis on the dorsal surface of the hands.

Bottom Line: Lipoid proteinosis is a sporadic congenital metabolic disorder which is characterized by deposition of hyaline material in dermis, submucosal connective tissue, and various internal organs.It has an extremely low prevalence rate with less than 300 cases reported so far.This progressive disease has a vast spectrum of manifestations ranging from asymptomatic lesions to fatal seizures and respiratory obstruction making timely diagnosis of this rare disorder an imperative task for oral health care practitioners.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine & Radiology, JSS Dental College and Hospital, JSS University, Mysore, Karnataka 570015, India.

ABSTRACT
Lipoid proteinosis is a sporadic congenital metabolic disorder which is characterized by deposition of hyaline material in dermis, submucosal connective tissue, and various internal organs. It has an extremely low prevalence rate with less than 300 cases reported so far. This progressive disease has a vast spectrum of manifestations ranging from asymptomatic lesions to fatal seizures and respiratory obstruction making timely diagnosis of this rare disorder an imperative task for oral health care practitioners. We report a case of characteristic oral manifestations of lipoid proteinosis in a 28-year-old male patient along with a review of relevant prevailing literature.

No MeSH data available.


Related in: MedlinePlus