Chronic bullous disease of childhood – A case report / Hronična bulozna bolest Kod Dece – Prikaz Slučaja

Abstract

Linear IgA bullous dermatosis is a chronic, acquired, autoimmune subepidermal vesiculobullous disease. Both children and adults are affected. It is characterized by direct immunofluorescence findings of linear immunoglobulins class A (IgA) deposits along the dermal-epidermal junction (basement membrane zone). In children, the disease is commonly referred to as chronic bullous disease of childhood and it mostly affects children between 2 and 5 years. The onset of the disease is acute; the first episode is the most severe, while recurrences tend to wax and wane in severity and last till puberty or even longer. Diaminodiphenylsulfone is the treatment of choice, although systemic corticosteroids are reported to be very effective as well. We report a 3-year-old boy with a vesiculobullous eruption which developed one week following administration of cephalexin for upper respiratory infection. He was referred to our Clinic from other health institutions as treatment failure for suspected strophulus or impetigo bullosus. On admission, the patient had fever and numerous vesiculobullous and erosive lesions distributed on the face and trunk. After immunohistological verification, the treatment with prednisone 25 mg/d was introduced, due to rapid progression of the disease and the fact that diaminodiphenylsulfone was not available. Improvement occurred after 2 weeks, so the dose was carefully tapered, taking into account the possibility of adrenal suppression. The medication was completely excluded within the next three months. No serious side effects were observed, except transitory hirsutism. The patient has had no relapses over the last 20 months of clinical follow-up.