A 30-year-old woman presented to the hospital with a morbilliform and pruritic generalized rash [], along with high-grade fever, fatigue, and malaise for 4 days. Physical examination and blood tests revealed enlarged cervical and inguinal nodes, leukocytosis (up to 66,800 white blood cell/mm3), and elevated levels of hepatic transaminases, lactate dehydrogenase, ß-2 microglobulin, and erythrocyte sedimentation rate. Serology results for hepatitis, syphilis, typhoid fever, and brucella were negative. The whole body computed tomography showed multiple neck, chest, and abdominal lymphadenopathy with splenomegaly suggestive of lymphoma []. A skin biopsy specimen revealed epidermotropism of atypical lymphoid cells, with CD30 positive staining as well as a dense perivascular and perifollicular lymphoid infiltrate []. An excisional biopsy of a 1.0 cm cervical node showed diffuse polymorphic lymphoid hyperplasia with effacement of the normal architecture with strong and diffuse staining for CD30 on immunohistochemistry, positive anti-leukocyte common antigen, and high proliferation index (Ki67 80%). All findings were consistent with lymphoma. However, osteomedullary biopsy showed no evidence of lymphoproliferative disorder, was absent for immunomarkers and high eosinophils proliferation rate. T-cell receptor rearrangement studies on the skin, lymph node, and bone marrow biopsies, failed to detect monoclonality. A detailed review of her past medical history revealed that she was started on lamotrigine 50 mg/day for somatization disorder 40 days prior to the onset of symptoms. Blood samples were positive for human herpesvirus 6 DNA. The diagnosis of DIHS/DRESS was made; lamotrigine was discontinued and treatment with oral corticosteroids (prednisolone, 60 mg/day) was started with rapid tapering. The patient made a full recovery with no organ deficit on follow-up visits at 1, 2, 4, and 6 months.
A 12 year old boy, with a known case of seizure disorder presented to the casualty with generalized maculopapular rash, fever and facial puffiness. The patient had been operated for gastric perforation when he was 3 days old following which the seizure episodes started. He was treated with phenobarbitone till the age of five. The drug was gradually withdrawn on the advice of a physician. Six months ago he suffered an episode of convulsion. He was started on sodium valproate and levetiracetam. One month back levetiracetam was stopped and lamotrigine was initiated. The patient was taking 1 tablet of 25mg daily for the first 15 days; later the dose was doubled. On the 18th day, patient presented with generalized maculopapular rash, itching and fever and on 20th day he developed colicky abdominal pain and vomiting followed by facial puffiness.\nOn examination, he was alert and oriented, with a temperature of 100°F and pulse was 114/ minutes. There was significant angioedema of his face with edematous lips and swollen ears, and an extensive maculo-papular rash on his entire trunk [], buttocks, extremities and face. The whole body had a red tinge. Examination showed yellow sclera, conjunctival congestion and crusting over his lips. He experienced no joint pain or neurological symptoms, and no abdominal or respiratory complaints. There was no history of any allergies. The birth history and immunization were uneventful. He had 3 siblings: one of them also had history of epileptic disorders for which she was on treatment.\nInvestigations revealed leukocytosis (15,960cells/cmm) with eosinophilia (19%) AEC=2600/cmm. Hemoglobin was 10.8gm%, platelet count 2.53lakhs/cmm; ESR and PCV were normal. Liver enzymes were raised with SGOT=905U/l, SGPT=495U/l, ALP=855U/l and GGT= 155U/l. Total bilirubin was 1.5mg% (direct 0.51mg% and indirect 0.99mg%). Blood urea and creatinine were normal. Routine examination of the urine was normal. Provisional diagnosis of DRESS was made based on the above findings.\nParental cortisteroids and systemic antihistamines were initiated. Lamotrigine was stopped. Within 5 days the rash subsided. Patient's general condition improved. On discharge patient was given oral steroids and antihistaminic's.