A 16 year old girl with fever and hepatitis.

A 16 year old girl presented with high grade fever, skin rash and cervical lymphadenopathy since 2 weeks. She was seen in another hospital where workup for hepatitis (Hepatitis A, B, C,and E), leptospirosis, malaria, and rickettsial disease was non-conclusive. Broad spectrum antibiotics were initiated (ceftriaxone, piperacillin-tazobactam, doxycycline, amikacin). However symptoms persisted.

At presentation, the patient was febrile and had tachycardia. Blood pressure was stable. A macular erythematous rash was seen over the trunk and limbs. No eschars were seen. Cervical non-tender lymph nodes were palpable. Respiratory examination was normal, and abdominal examination revealed right hypochondrial tenderness. Review of results from previous hospital showed anemian (Hb 9.2), a normal leucocyte and platelet count. The ALT/AST were 272/244 IU respectively with a serum bilirubin of 2.8 mg/dl. INR was 1.4. Serum creatinine was 0.9 mg/dl. Chest radiograph was normal. Blood cultures were sterile. HIV, EBV and CMV antibodies were negative.

The patient's mother described hypopigmented patches over the cheek and right shoulder since 3 years. A skin biopsy had revealed borderline tuberculoid leprosy ~1 month prior. Dapsone 100 mg/day was started and continued till ~1 week prior to presentation at this hospital.

Dapsone hypersensitivity syndrome was diagnosed. The patient was started on prednisolone 40 mg/day. Transient clinical improvement was noted, however symptoms recurred over the next 5 days with tachycardia, hypotension and worsening hepatic function. Imipenem-cilastatin was administered. Intravenous methyl prednisolone was started at 250 mg/day for 3 days followed by tapering doses, with dramatic clinical improvement and resolution of rash, lymphadenopathy and hepatic dysfunction. However hospital course was complicated by transfusion associated lung injury / ARDS requiring mechanical ventilation for 5 days.

Oral prednisolone was continued in tapering doses, with patient remaining free of symptoms.

Dapsone Hypersensitivity Syndrome