Naslov
IgA pemphigus. Case report
(IgA Pemphigus. Case report)

Autori
Marinović, Branka ; Pašić, Aida ; Lipozenčić, Jasna ; Gotovac-Mustač, Mirjana

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Journal of Autoimmunity / Prof. Yehuda Shoenfeld - London : Delhi : Academic Press, 1999

Skup
2nd International Congress on Autoimmunity

Mjesto i datum
Tel Aviv, Izrael, 07.03.1999. - 11.03.1999

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
benign type of pemphigus; IgA antibody disease; autoimmunie blistering disease
(benign type of pemphigus; IgA antibody disease; autoimmune blistering disease)

Sažetak
IgA pemphigus is a relatively newly described, rare, autoimmune-mediated blistering disease characterized by the presence of specific IgA autoantibodies directed to desmosomal proteins of epidermis. We report on a 77-year old female patient who presented with erythematous patches on her forehead, scalp, skin of the trunki, proximal parts of extremities and in the intertriginous areas (axillary and inguinally). The involved parts of the body were partly covered with yellowish crusts and partly wet. Intensive pruritus was present. When admitted to our department, the following tests were performed to establish the diagnosis; in histopathology tere was an intraepidermal blister located in the upper epidermis with acantholytic cells within separation. Lymphocytitc spongiosis was alsos present. On direct immunofluorescence of periblistered skin, IgA and to a lesser extent IgG and C3 were detected. Indirect immunofluorescence was negative. Other laboratory tests were within the normal range. Therapy with prednisolone in a dose of 1 mg/kg body weight daily was introduced. After 4 weeks of therapy, satisfactory improvement of skin changes was achieved and pruritus reduced. Then azathioprin ws introduced as adjuvant therapy, along with corticosteroid dose reduction. We conclude that IgA pemphigus is a relatively benign type of pemphigus disease with specific histopathologic and immunofluorescent findings, and with some clinical course specificities.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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