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.
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.