A 16-year-old female student presented with absence seizures for 2 years of age and no significant past or family history of epilepsy or allergic disorder. The computerized tomography (CT) head and magnetic resonance imaging (MRI) of the brain were found to be normal; electroencephalography (EEG) was abnormal with diffuse cerebral dysfunction. She had been treated with clobazam 10 mg/day OD, for 1.5 years, but was still experiencing seizures about one to two in a day; therefore, LTG was added, starting with 25 mg a day, with weekly escalation, up to a dose of 100 mg/day. After 2 weeks of adding LTG, she developed fever, headaches, chills, and rash all over the body; the patient presented to the dermatology outpatient department (OPD) after 1 week of onset of the symptoms.\nOn examination, maculopapular lesions involving the genitals associated with itching were seen, which then spread to the trunk and then extremities in the next 3 days []. They became tender, and the erythematous, partially blanchable lesions took on a dusky hue over the trunk, upper limb, lower limb, and progressed to involve the face and lips (crusted plaques). No involvement of the oral cavity was noted nor were there any vesicular lesions. On the basis of history and clinical examination, a diagnosis of TEN was made and LTG was discontinued immediately and admitted under the dermatology department. LTG was stopped, and she was started on levetiracetam 500 mg twice a day, continued on clobazam 10 mg/day, intravenous steroids, fluids, and antibiotics. With medications and supportive care, the patient improved without any further complications.
An 18-year-old African American female with a history of hyperthyroidism presented to our facility with fever, chills, body aches, significant cervical lymphadenopathy, facial edema, and a progressive (eventually generalized) macular morbilliform rash. She reported that elevated transaminases had been noted during a recent visit with her endocrinologist. Her CBC included a WBC count of 8,600 mm3, hemoglobin of 12.4 g/dl (MCV 77.8 fL), and platelet count of 261,000 mm3. Ferritin was markedly elevated (1229.6 ng/mL); serum iron, TIBC, and iron saturation were decreased, suggestive of anemia of chronic disease. AST was elevated to 127 units/mL, and ALT was elevated to 239 units/mL. Hemoglobin electrophoresis revealed normal adult hemoglobin. A rapid HIV test was nonreactive. PCR testing of peripheral blood was negative for EBV and HHV-6. Multiple blood cultures were negative.\nAbdominal ultrasound showed splenomegaly and enlarged porta hepatis lymph nodes. A PET scan revealed diffuse hypermetabolic lymphadenopathy involving cervical, supraclavicular, axillary, pelvic, and inguinal nodes, as well as findings consistent with malignant infiltration of the bilateral kidneys and spleen (). Although the possibility of a drug reaction had been in the differential diagnosis prior to the imaging studies, the degree and extent of the imaging abnormalities raised clinical concern for a malignant process.\nDue to the concern for malignancy, axillary lymph node and bone marrow biopsies were performed. Examination of the bone marrow showed that it was appropriately cellular for age (∼80%), with maturing trilineage hematopoiesis, polyclonal plasmacytosis, and eosinophilia. Scattered small T-cell aggregates were present. Flow cytometric immunophenotyping did not detect any abnormal lymphoid populations. No evidence of malignancy was identified.\nHistologic examination of the lymph node revealed mostly preserved, but significantly distorted, nodal architecture with expansion of the paracortex by a mixed infiltrate of small lymphocytes, eosinophils, histiocytes, plasma cells, and scattered large atypical lymphoid cells, including occasional Reed–Sternberg-like cells. Secondary follicles were largely absent (Figures and ). A few apoptotic bodies and pigment-containing histiocytes were identified.\nImmunohistochemical stains for CD20, PAX5, CD79a, OCT2, and BOB-1 highlighted the B-cell population in the cortex that was largely confined to primary follicles. CD23 highlighted irregularly expanded follicular dendritic cell meshworks. CD3 highlighted numerous T cells in the paracortex and interfollicular areas. CD15 highlighted granulocytes. CD30 highlighted scattered large immunoblasts, including rare Reed–Sternberg-like cells (); no sheets of positive cells were seen. MUM1 was positive in plasma cells, predominantly in the medullary cords and sinuses. ALK immunostaining and EBV in situ hybridization (EBER) were negative.\nThe lymph node findings, including architectural distortion, expanded follicular dendritic cell meshworks, eosinophilic infiltrate, and proliferation of large CD30-positive lymphoid cells raised concern for a malignant process such as T-cell lymphoma or classic Hodgkin's lymphoma; however, the morphologic and immunophenotypic features were not entirely typical for those diagnoses. Molecular analysis of the lymph node did not detect any monoclonal IGH or IGK gene rearrangement or T-cell receptor gene rearrangement.\nTwo days after the lymph node biopsy was performed, the patient's WBC count had risen to 24,100/mm3. Examination of the peripheral blood smear revealed neutrophilia, atypical lymphocytes, and mild relative eosinophilia, with an increased absolute eosinophil count of 1,400/mm3. On further review of the patient's history, it was noted that the patient had received a course of minocycline to treat folliculitis, beginning approximately five weeks prior to admission and ending four days prior to admission.\nAfter consideration of all of the available information, a diagnosis of DIHS was made. The minocycline was discontinued permanently. Following treatment with prednisone, the patient's rash, leukocytosis, and lymphadenopathy gradually resolved.