Limits...
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: 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.

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

Hematoxylin and eosin stained sections (40x) revealed stratified squamous parakeratinized hyperplastic epithelium with presence of fibers, fibroblasts, blood vessels, and numerous eosinophilic amorphous aggregates in the connective tissue.
© Copyright Policy - open-access
Related In: Results  -  Collection


getmorefigures.php?uid=PMC4383431&req=5

fig5: Hematoxylin and eosin stained sections (40x) revealed stratified squamous parakeratinized hyperplastic epithelium with presence of fibers, fibroblasts, blood vessels, and numerous eosinophilic amorphous aggregates in the connective tissue.

Mentions: Complete hemogram and thyroid profile values were found to be within the normal range. An incisional biopsy from the buccal mucosa and ventral surface of the tongue was performed. Similar histological findings and staining patterns were observed in the sections from both sites. Hematoxylin and eosin stained sections revealed stratified squamous parakeratinized epithelium and it was hyperplastic in nature. The tongue specimen showed spongiosis of epithelial cells in few areas. The connective tissue showed hyalinized fibrous stroma with fibers, fibroblasts, blood vessels, and proliferating endothelial cells. Numerous eosinophilic amorphous aggregates were seen in the connective tissue (Figure 5). Similar type of deposit was also found around the blood vessels and the proliferating endothelial cells. Special staining with Congo red and crystal violet was found to be negative which ruled out the presence of amyloid tissue (Figure 6). Periodic acid Schiff staining (PAS) was found to be positive, which showed deposition of pink hyaline PAS-positive material in the connective tissue and around the blood vessels (Figure 7).


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)

Hematoxylin and eosin stained sections (40x) revealed stratified squamous parakeratinized hyperplastic epithelium with presence of fibers, fibroblasts, blood vessels, and numerous eosinophilic amorphous aggregates in the connective tissue.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig5: Hematoxylin and eosin stained sections (40x) revealed stratified squamous parakeratinized hyperplastic epithelium with presence of fibers, fibroblasts, blood vessels, and numerous eosinophilic amorphous aggregates in the connective tissue.
Mentions: Complete hemogram and thyroid profile values were found to be within the normal range. An incisional biopsy from the buccal mucosa and ventral surface of the tongue was performed. Similar histological findings and staining patterns were observed in the sections from both sites. Hematoxylin and eosin stained sections revealed stratified squamous parakeratinized epithelium and it was hyperplastic in nature. The tongue specimen showed spongiosis of epithelial cells in few areas. The connective tissue showed hyalinized fibrous stroma with fibers, fibroblasts, blood vessels, and proliferating endothelial cells. Numerous eosinophilic amorphous aggregates were seen in the connective tissue (Figure 5). Similar type of deposit was also found around the blood vessels and the proliferating endothelial cells. Special staining with Congo red and crystal violet was found to be negative which ruled out the presence of amyloid tissue (Figure 6). Periodic acid Schiff staining (PAS) was found to be positive, which showed deposition of pink hyaline PAS-positive material in the connective tissue and around the blood vessels (Figure 7).

Bottom Line: 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.

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