Challenging treatment of linear IgA dermatosis in a patient with dapsone-induced hypersensitivity syndrome

Abstrakt

We report a case of a 44-year-old woman, who was admitted to dermatovenereology department with a history of severe pruritic erythematous lesions for several months. Clinical picture consisted of erythematous and focally urticarial patches and plaques, with grouped vesicles in figurate arrangement resembling a cluster of jewels located on her scalp, trunk and extremities.

Direct immunofluorescence confirmed diagnosis of linear IgA bullous dermatosis and the patient started treatment with oral dapsone in gradually increasing doses to 150 mg daily. Skin lesions healed promptly, nevertheless the patient developed dyspnea and acral cyanosis manifesting methemoglobinemia, on that account dose had to be lowered to 100 mg.

Three weeks after dapsone introduction, the patient presented with malaise, high fever, morbilliform confluent rash, facial edema, cervical lymphadenopathy and painful oral mucosal lesions. Laboratory screening revealed elevated liver enzymes and serum bilirubin.

Dapsone-induced hypersensitivity syndrome was highly suspected, therefore dapsone was stopped and the patient was treated by oral corticosteroids with slow tapering. Soon after dapsone withdrawal vesicular lesions on her extremities relapsed, consequently treatment with 1500 mg sulfasalazine was added into combination.

To date, the patient is successfully treated with 1000 mg sulfasalazine along with low-dose prednisone, the blistering disorder resolved completely leaving milia and hyperpigmentation. The authors recommend sulfasalazine as beneficial treatment option in patients with linear IgA disease.