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Letters to the Editor

Indian Pediatrics 1999; 36:98-99

Carbamazepine Hypersensitivity Syndrome


Carbamazepine hypersentative syndrome refers to a variety of manifestations consisting of pseudolymphoma syndrome, serum sickness, glandular fever, hypersensitivity vasculitis and necrotising granulomatous angitis(1). We describe a patient with a severe carbamazepine induced reaction with multisystem involvement.

A 13-year-old girl on carbamazepine therapy for 6 weeks for seizures. presented with fever, cough, dyspena and rash of 10 days duration. On examination she was toxic and had erythematous maculopapular rashes, conjunctivitis, generalized lymphadenopathy, hepatosplenomegaly, bilateral wheeze and crackles. Investigations revealed leukocytosis (36,500/cumm), eosinophilia, (9800/ cumm), anemia (8g/dl) with normal platelet counts. Liver function tests showed evidence of hepatitis with elevated levels of SGOT (2305 units), SGPT (665 units) and alkaline phosphatase (788 units), with normal bilirubin levels. Blood and urine cultures were sterile. Skiagram of the chest showed hyperinflated lungs and increased broncho- vascular markings. Serological tests consisting of Widal, MAT for leptospira, HbsAg, Paul Bunnel test, Rheumatoid factor and Antinuclear antibodies were negative. Bone marrow smears were normal.

Histopathological examination of axillary lymph node showed follicular hyperplasia with widening of paracortical areas due to infiltration by pale. foamy macrophages containing melanin pigment which was consistent with dermatopathic lymphadenopathy. She was treated with antibiotics for 10 days initially, but .was treated symptomatically afterwards with salbutamol and paracetamol only. Her temperature returned to normal levels 4 weeks after the onset of illness and all other symptoms and signs gradually regressed a week after that. There were thus, no clinical or laboratory findings to support a diagnosis of leukemia, lymphoma or infection.

A retrospective diagnosis of Carbamazepine hypersensitivity syndrome was made because of the manifestations of fever, rash, lymphadenopathy, hepatosplenomegaly, hepatitis and pulmonary involvement with eosinophilia and the spontaneous regression of illness. Dermatopathic lymphadenopathy (Lipomelanosis reticulosis) is a type of nodal hyperplasia that develops sec- c:1dary to various forms of generalized exfoliative dermatitis, inflammatorydermatoses such as psoriasis and neoplastic diseases such as mycosis fungoides(2). Only one case of Dermatopathic lymphadenopathy associated with Carbamazepine has been reported earlier(3). The other reported histopathological findings in lymph nodes in Carbamazepine hypersensitivity syndrome include lymphoid hyperplasia, lymphoma like picture., immunoblastic and angioimmunoblastic lymphadenopathy.

Carbamazepine hypersensitivity syndrome is a clinical diagnosis made by exclusion of infections, immunologic or neoplastic disorder or reaction to other drugs( 4) though confirmation by patch testing, lymphocyte transformation test and re-exposure to the drug has been reported. Our experience with this case emphasises the need to consider this diagnosis in any child receiving Carbamazepine presenting with fever, lymphadenopathy, rash and multisystem involvement.
 

S. Balasubrarnanian,
S. Shobhana,
L.N. Padrnasani,
M. Natarajan,
Rarnesh Rao,

Department of Pediatrics and Pathology,
Sir Ramachandra Medical College,
and Research Institute (Deemed University),
Porur, Chennai 600 116, India.

 

REFERENCES

1. De Vriese SP, Jan Phillipe, Van Renterghem M, Christa De Cuyper, Phillippe Hindryckx HH, Erve Mathys, et al. Carbamazepine hypersensitivity syndrome. Report of 4 cases and review of literature. Medicine (BaIt) 1995; 74: 144-151.

2. Gould E, Porto R, Albores. Dermatopathic lymphadenitis-The spectrum and significance of its morphological features. Arch Pathol Lab Med. 1988; 112: 1145-1150.

3. Severson GS, Harrington DS, Burnett DA, Linder J. Dermatopathic lymphadenopathy associated with carbamazepine. A case mimicking a lymphoid malignancy. Am J Med 1987; 83: 597-599.

4. Case records of the Massachusetts General Hospital (case 26-1,996). N Engl J Med 1996; 335: 577-584.

 

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