IngentaConnect Clinicoepidemiological features and course of 43 cases of bullous...

Abstract:

Summary Background.

The clinicoepidemiological characteristics and course of bullous pemphigoid (BP) have not been described in populations from the Arabian Gulf. Hypothesis.

Ethnic and regional variations can influence the clinical behaviour and course of autoimmune diseases. Methods.

In this study, 43 patients with BP, registered in our autoimmune bullous diseases (ABD) clinic over a span of 14 years, were studied to determine the clinicoepidemiological features and course of the disease in our region. Results.

BP was observed to be the second commonest ABD in our clinic (27%), with a minimum estimated incidence of 2.6 cases/million/year among the referral population. The largest proportion (93%) of the patients was of Arab ethnicity and the female to male ratio was 5.1 :1. Mean ± SD age at diagnosis was 65.20 ± 18.80 years. Most of the patients (96%) had moderate to severe disease, and mucosal involvement was seen in 37% of the patients. Systemic steroids (prednisolone 20–60 mg daily) alone or in combination with azathioprine, intravenous immunoglobulins, tetracyclines, mycophenolate mofetil or dapsone were used to treat theses cases. At the last follow-up, 32% of patients were in complete remission and off treatment. The first-year mortality was 30%. Old age and poor general medical condition were the significant risk factors (P < 0.05) contributing to the mortality. Conclusion.

The study highlights the regional variations of BP and thus a need to uncover the ethnic, genetic and geographical influences, if any, responsible for these variations.

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Clinicoepidemiological features and course of 43 cases of bullous pemphigoid in Kuwait