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 16-year-old boy, known case of juvenile myoclonic epilepsy, presented to the neurology OPD with generalized maculopapular rash [], fever, and facial puffiness. He was on LTG 250 mg TDS, clobazam 10 mg OD, and valproate 200 mg (morning) 400 mg (evening) for 1 year. Subsequently, he developed erythematous rashes over the face, neck, trunk, arms, and legs followed by painful ulcers in the oral cavity and encrustations on the lips. History revealed that no such lesions occurred earlier. His neurological examinations, CT head and MRI, were found to be within normal limits. EEG was abnormal and suggestive of generalized epilepsy. Physical examination showed multiple round erythematous plaques on the face, neck, trunk, and extremities, some of which were tender. On oral examination, there was limited mouth opening and erythematous crusted areas on both the lips due to inflammation and ulceration. Based on the history and clinical presentation, a diagnosis of SJS was made. As the Pre-assumption was LTG, the drug was withdrawn immediately and the dose of clobazam was increased. The patient was admitted to dermatology ward and treated with cyclosporine and methylprednisolone and intravenous fluids. His condition improved during the next 2 weeks; he eventually recovered and continued follow-up on an outpatient basis.