Successful Treatment of Lipoid Proteinosis in a Young Adult with Isotretinoin
نویسندگان
چکیده
Lipoid proteinosis (LP) is an extremely rare autosomal recessive genodermatosis, characterized by persistent hoarseness and classical skin lesions. Amorphous hyaline depositions may affect any organ, but mainly the face extremities. These cause limitations in tongue movement, hence, difficulty speaking. Biopsies of lesions usually show massive deposits homogeneous, acidic, hyaline-like material at epidermal junctions superficial dermis that stains positive for periodic acid-Schiff stain (PAS-D). At present, there no specific treatment this disease, some studies have reported with acitretin etretinate results improvement. This paper reports on effectiveness isotretinoin first time disease.
منابع مشابه
Acitretin Treatment for Lipoid Proteinosis
Lipoid proteinosis (LP) is a rare, autosomal-recessive disease characterized by the hoarseness and widespread cutaneous scarring, more prominent on sun-exposed areas. Yellow-white plaques can be seen on oral mucosa and on the skin among depressed scars. Histological evaluation of the affected sites shows accumulation of hyaline-like material in dermis and disruption of basement membrane. Althou...
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Lipoid proteinosis or Urbach-Wiethe disease is a rare autosomal recessive disorder. It is characterized by progressive deposition of hyaline substance in the mucous membranes, skin and internal organs. It is characterized by papular and nodular lesions on the face, elbows, knees and hands. The tongue is firm and hoarseness may be present at birth. We aim to describe one case of this disease in ...
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A case of lipoid proteinosis presented with multiple papular lesions on the face and hoarseness of voice from early infancy. Five sibs of the patient were affected and one had died early in infancy, probably because of laryngeal obstruction.
متن کاملLipoid Proteinosis.
The case of a 37-year-old woman with lipoid proteinosis is presented. The first symptoms of hoarseness of voice occurred in puberty. Additional symptoms included beaded eyelid papules, macroglossia with yellowish papules, yellowish deposits of soft palate, popular eruption in the axillary area and tuberous nodules on the elbows.
متن کاملLipoid proteinosis.
Lipoid proteinosis is a rare, autosomal recessive disorder that presents in early infancy with hoarseness, followed by pox-like and acneiform scars, along with infiltration and thickening of the skin and certain mucous membranes. Histological and ultrastructural examination reveals widespread deposition of hyaline-like material and disruption/reduplication of basement membrane around blood vess...
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ژورنال
عنوان ژورنال: Journal of pharmaceutical research international
سال: 2023
ISSN: ['2456-9119']
DOI: https://doi.org/10.9734/jpri/2023/v35i107351