Pemphigus vulgaris in a patient with primary hypogammaglobulinemia: A case report

Pemphigus vulgaris in a patient with primary hypogammaglobulinemia: A case report [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2022; 56(2): 80-83 | DOI: 10.4274/turkderm.galenos.2022.81370

Pemphigus vulgaris in a patient with primary hypogammaglobulinemia: A case report

U�ur Hac� Musabak¹, Tuba Erdo�an¹, Mustafa Tunca²
¹Ba�kent University Faculty of Medicine, Department of Immunology and Allergy, Ankara, Turkey
²University of Health Sciences Turkey, G�lhane Faculty of Medicine, Department of Dermatology, Ankara, Turkey

Pemphigus vulgaris (PV) is a rare autoimmune disorder characterized by blisters on the mucous membranes and skin. Autoimmunity is an important complication developing in predominantly antibody deficiencies, which is a subgroup of primary immunodeficiencies (PID). Herein, we present a patient with PV who had primary antibody deficiency and whose disease relapsed during the maintenance period of conventional immunosuppressive treatments but progressed to remission following high-dose intravenous immunoglobulin therapy. Thus, we aimed to create awareness for the study of primary immunodeficiencies in rare autoimmune bullous diseases.

Keywords: Pemphigus vulgaris, primary antibody deficiency, primary immune deficiency

Primer hipogamaglobulinemili bir hastada pemfigus vulgaris: Bir olgu sunumu

U�ur Hac� Musabak¹, Tuba Erdo�an¹, Mustafa Tunca²
¹Ba�kent �niversitesi T�p Fak�ltesi, �mmunoloji ve Allerji Bilim Dal�, Ankara
²G�lhane T�p Fak�ltesi, Dermatoloji Ana Bilim Dal�, Ankara

Pemfigus vulgaris (PV), deri ve m�koz membranlarda b�llerin varl�� ile karakterize, nadir g�r�len otoimm�n bir hastal�kt�r. Otoimm�nite, �zellikle primer imm�n yetmezliklerin bir alt grubu olan antikor eksikliklerinde geli�en �nemli bir komplikasyondur. Burada, PV ile birlikte primer antikor eksikli�i olan ve geleneksel imm�nos�presif tedavilerin idame d�neminde n�kseden ancak y�ksek doz intraven�z imm�noglobulin tedavisi ile remisyona giren PV�li bir olgu sunulmaktad�r. Bu olgunun sunumu ile nadir g�r�len otoimm�n b�ll�z hastal�klarda primer imm�n yetmezliklerin ara�t�r�lmas� i�in fark�ndal��n geli�tirilmesi ama�lanm��t�r.

Anahtar Kelimeler: Pemfigus vulgaris, primer antikor eksikli�i, primer imm�n yetmezlik

U�ur Hac� Musabak, Tuba Erdo�an, Mustafa Tunca. Pemphigus vulgaris in a patient with primary hypogammaglobulinemia: A case report. Turkderm-Turk Arch Dermatol Venereol. 2022; 56(2): 80-83

Corresponding Author: Tuba Erdo�an, T�rkiye

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