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Peristomal Localized Pemphigoid: Is It an Underdiagnosed Condition with a Good Prognosis?
Can Baykal1, Elizaveta Melnicova Ayas, Tuğba Atcı
1Can Baykal, MD, is Professor, Department of Dermatology and Venereology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey; Elizaveta Melnicova Ayas, MD, is Resident; Department of Dermatology and Venereology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey; Tuğba Atcı, MD, is Assistant Professor; Department of Dermatology and Venereology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey; Şule Öztürk Sarı, MD, is Associate Professor, Department of Pathology; Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey; and Nesimi Büyükbabani, MD, is Professor, Department of Pathology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey and Koç Medical Faculty, Istanbul,; and Rıfkiye Küçükoğlu, MD, is Professor, Department of Dermatology and Venereology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
Objective:
To evaluate the rate of peristomal involvement in a large series of bullous pemphigoid (BP) patients and to focus on the clinical features and the course of the disease of these patients.
Methods:
Bullous pemphigoid patients were investigated retrospectively regarding the rate of peristomal involvement, and the data of these patients, including demographics, diagnosis leading to stoma placement, stoma type and stoma duration, treatment, and follow-up were evaluated in detail. In addition, the authors' results were compared with literature data.
Results:
The authors observed peristomal involvement in four cases (1.5%) (male/female: 1) among a cohort of 270 consecutive BP patients. The mean age at BP diagnosis was 69.5 ± 6.8 (63-78) years. Peristomal lesions occurred 4.7 ± 3.8 (1-10) years following stoma placement. Although three patients presented with BP lesions only on peristomal localization (colostomy limited), the fourth patient developed generalized pemphigoid lesions for 6 months followed by only peristomal involvement for 3 years. One patient was lost to follow-up. Topical corticosteroids (n = 3), systemic corticosteroids (n = 2), and dapsone (n = 2) were the treatment of choice in three patients, providing a complete response in a mean follow-up period of 4.7 ± 4 years without any mortality.
Conclusions:
The high rate of peristomal BP patients in this series raised the possibility of misdiagnosis of this specific condition as irritant dermatosis. Peristomal lesions may be isolated as an initial presentation of BP or represent the recurrence. Recognition of peristomal BP showing a favorable prognosis compared with classical BP is important.
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