A 36-year-old woman presented at our institution (Gachon University Gil Hospital) with erythematous confluent maculopatches on her face, trunk, and palms; jaundice; fatigue; pruritus; and aggravation of skin lesions over the previous week. She had been previously diagnosed as having erythema multiforme 2 weeks earlier at a private dermatology clinic and had also been administered dapsone with oral antihistamines (mequitazine and cetirizine) and topical corticosteroid for 8 months.\nOn admission, her vital signs were a blood pressure of 110/70 mm Hg, heart rate of 78 beats/min, respiratory rate of 18 breaths/min, and body temperature of 38.8℃. She reported no night sweats, loss of weight, or arthralgia. A physical examination conducted at presentation revealed bilateral, multiple enlarged lymph nodes on both sides of the neck with splenomegaly. All of the nodes were non-tender, firm, and measured 0.5 cm to 1.0 cm in diameter. Laboratory tests results were as follows: Hb 11.2 g/dL, white blood cell 13,070/mm3 (segmented neutrophils 24%, lymphocytes 66.3%, and eosinophils 0.1%), platelets 245,000/mm3, total bilirubin 5.4 mg/dL, direct bilirubin 3.8 mg/dL, aspartate aminotransferase/alanine aminotransferase (AST/ALT) 233/366 U/L, lactate dehydrogenase 1,610 IU/L (normal range, 200 to 485 IU/L), and an international normalized ratio of 1.25. She was negative for hepatitis B surface antigen, anti-hepatitis C virus Ab, and anti-human immunodeficiency Ab, but positive for hepatitis B surface antibody; and positive for anti-nuclear Ab, but negative for rheumatoid factor, anti-neutrophil cytoplasmic Ab, anti-Ro (SS-A, B) Ab, and anti-smith Ab. Serology tests showed no evidence of active Epstein-Barr virus (EBV) infection and no bacterial growth was found during a blood culture study. There was no hypergammaglobulinemia.\nAn excisional biopsy of a 1.0 cm cervical node showed diffuse polymorphic lymphoid hyperplasia with effacement of the normal architecture and abundant high endothelial venules; the cellular infiltrate was polymorphic, with abundant transformed lymphocytes and plasma cells (). Sinuses were frequently recognizable, but reactive germinal centers were not found.\nImmunohistochemically, the majority of infiltrating atypical lymphocytes were CD3+ T-cells (), and some activated large cells were reactive for CD30 (). Follicular dendritic cell meshworks expressing CD21 were expanded (), and infiltrating atypical T-cells were negative for CD10, BCL-6, and EBV by in situ hybridization. For a definitive diagnosis, a molecular study on the rearrangement of the T-cell receptor (TCR)-gamma gene was performed and showed polyclonality (). An IgH gene rearrangement study also showed polyclonality.\nAn abdomino-pelvic computed tomography (CT) scan revealed splenomegaly (14 cm), an enlarged portocaval lymph node (1.7 cm), and gallbladder wall edema (). No evidence of lymphomatous involvement was found by chest CT or a bone marrow trephine biopsy.\nBecause aggravation of the skin eruption persisted and the liver function test remained abnormal, the possibility of DHS (exfoliative dermatitis, acute hepatitis, and multiple lymphadenopathy) was raised. The findings of a transjugular liver biopsy were compatible with drug-induced hepatitis manifesting as granulomatous inflammation (). No evidence of lymphomatous hepatic involvement was found. In view of her skin lesions, medication history, and age, we concluded that the cervical lymphadenopathy was actually pseudolymphoma. Accordingly, low dose prednisolone (30 mg/day) per os was started after transjugular liver biopsy to relieve the dapsone-induced skin lesions and hepatitis. Gradually, the skin lesions improved and the cervical lymph node enlargement regressed. Her total bilirubin, AST, and ALT values also normalized after 3 weeks of prednisolone, which was then tapered over a month. Follow-up CT scans conducted 3 and 6 months after presentation showed that the size of the cervical lymph nodes and spleen had normalized, and produced no other findings suggestive of lymphoma progression ().
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.