Pemphigus vulgaris in a patient with primary hypogammaglobulinemia: A case report Primer hipogamaglobulinemili bir hastada pemfigus vulgaris: Bir olgu sunumu
Turkderm Turkish Archives of Dermatology and Venereology, cilt.56, sa.2, ss.80-83, 2022 (Scopus, TRDizin)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 56 Sayı: 2
- Basım Tarihi: 2022
- Doi Numarası: 10.4274/turkderm.galenos.2022.81370
- Dergi Adı: Turkderm Turkish Archives of Dermatology and Venereology
- Derginin Tarandığı İndeksler: Scopus, TR DİZİN (ULAKBİM)
- Sayfa Sayıları: ss.80-83
- Anahtar Kelimeler: Pemphigus vulgaris, primary antibody deficiency, primary immune deficiency
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
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.