Dapsone hypersensitivity syndrome: A rare life threatening complication of dapsone therapy
A 17-year-old male student was brought with complaints of high grade fever associated with chills and rigors, jaundice and itchy skin rash for 10 days. One month prior to current admission, he was started on dapsone 100 mg/day by a dermatologist for suspected lichen planus. He had discontinued the medication 10 days earlier when he developed the above complaints. On admission, he had high grade fever (103-104°F), deep icterus, palpable lymph nodes in the cervical and axillary regions but without palpable liver or spleen. He had a toxic look, was agitated and confused, with generalized skin erythema and extensive scaling - suggesting exfoliative dermatitis [Figures and ]. There was angular cheilitis with a crusted lesion over the lip, subconjunctival hemorrhage but oral mucosa and penile mucosa were normal []. Patient later developed shock (blood pressure: 70/40 mm Hg) and right internal jugular central venous catheter was placed. Noradrenaline was started as the hypotension did not respond to fluid challenge. Empirical broad spectrum antibiotic cover (meropenem and vancomycin) was given keeping the possibility of severe sepsis with septic shock, in view of breach in integrity of the skin.\nLaboratory evaluation revealed neutrophilic leucocytosis with eosinophilia (Hb: 12.3 g/dl, leucocytes: 12,700/μl, platelets: 2,10,000/μl, differential count: Neutrophils-78%, Lymphocytes-12%, Eosinophils-10%), hepatitis [bilirubin: 13.5 mg/dl (direct fraction:10.2 mg/dl), AST: 239 IU/l, ALT: 430 IU/l, alkaline phosphatase: 538 IU/l], hypoalbuminemia (2.8 g/dl), coagulopathy (prothrombin time: 26 s (control: 14 s), aPTT: 48 s (control: 28-34 s)), normal urine examination and renal function. Tests for malaria, leptospirosis, typhus fever, cytomegalovirus (CMV), Epstein Barr virus (EBV), ELISA for HIV-1 and 2, hepatitis A, B, E and C were negative. Antinuclear antibodies were negative. Repeated blood and skin swab cultures were sterile. Chest radiography and abdominal sonography were unremarkable.\nPatient's blood pressure improved and noradrenaline was stopped after 4 days of admission. He continued to have high grade fever despite being on antibiotics for 5 days. A diagnosis of dapsone systemic hypersensitivity syndrome was made based on history of dapsone intake, followed by fever, skin rash, eosinophilia, lymphadenopathy and hepatitis. He was started on oral prednisolone 50 mg/day (1 mg/kg) from day 6. He improved over next 10 days with gradual subsidence of fever, rash and hepatitis. Antibiotics were discontinued after 10 days and he was discharged on day 18 of admission. Prednisolone 1 mg/kg/day was continued for 1 month and then tapered off over next 6 weeks, with complete normalization of liver function and resolution of lymphadenopathy.