Defne Özkoca¹
Savaş Yaylı²

¹Koç University, Faculty of Medicine, Department of Dermatology, İstanbul, Türkiye
²Koç University, Faculty of Medicine, Department of Dermatology, İstanbul, Türkiye

Özkoca D, Yaylı S. Bullous Skin Disorders. In: Kutlubay Z, editor. Guidelines in Dermatology. 1^st ed. Ankara: Türkiye Klinikleri; 2025. p.55-61.

ABSTRACT

The diagnosis and management of relatively common autoimmune blistering diseases are discussed in several international guidelines. In this review, we summarized the diagnosis and management of pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, bullous pemphigoid, mucous membrane pemphigoid and dermatitis herpetiformis guidelines initiated by the European Academy of Dermatology and Venereology (EADV) Bullous Group. Pemphigus vulgaris and pemphigus foliaceus are autoimmune blistering diseases with distinct clinical manifestations. Pemphigus vulgaris primarily affects the oral mucosa, followed by cutaneous lesions, presenting as flaccid bullae. Pemphigus foliaceus is characterized by cutaneous lesions, particularly in seborrheic areas. Diagnosis involves clinical presentation, histopathology, and immunofluorescence, along with the detection of specific antibodies. Treatment aims to control the disease and manage side effects of long-term immunosuppression, with corticosteroids and rituximab being the most used, depending on the disease severity. Monitoring for the complications and relapses is essential, with regular follow-ups until remission is achieved. Paraneoplastic pemphigus (PNP), now called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune condition linked to malignancies, particularly lymphoproliferative diseases. It presents with severe mucositis and polymorphic skin lesions. Treatment focuses on managing the underlying malignancy along with systemic corticosteroids, corticosteroid-sparing agents, and, if needed, B-cell depleting agents. Multidisciplinary management is crucial for optimal care. Bullous pemphigoid (BP), presents with bullae on erythematous skin and rarely mucosal involvement. Treatment focuses on reducing symptoms, with topical or systemic corticosteroids as first-line therapies. Regular monitoring is necessary, especially in elderly patients with comorbidities, to adjust treatment and prevent relapses. Mucous membrane pemphigoid (MMP) primarily affects mucosal surfaces, with oral lesions being the most common. Severe cases can involve ocular, genital, and other mucosal areas. Diagnosis is based on clinical findings and detection of specific antibodies. Treatment includes dapsone, methotrexate, or corticosteroids, with a focus on improving oral hygiene and preventing complications. Dermatitis herpetiformis (DH) is a pruritic, gluten-induced skin disease, often associated with celiac disease. The treatment involves a strict gluten-free diet and dapsone for severe cases. Regular monitoring is required due to potential side effects of dapsone, particularly hemolytic anemia.

Keywords: Bullous; Mucosa; Pemphigus; Pemfigoid; Treatment