Journal of the American Academy of Dermatology

Braddock et al.

10. 11. 12. 13.

14. 15.

16.

simulating lymphosarcoma induced by diphenylhydantoin sodium. JAMA 196l;176:491-3. Charlesworth EN. Phenytoin-induced pseudolymphoma syndrome. Arch Dermatol 1977;113:47%80. Choovivathanavanich P, Wallance EM, Scaglione PR. Pseudolymphoma induced by diphenylhydantoin. J Pediatr 1970;76:621-3. Oates RK, Tonge RE. Phenytoin and the pseudolymphoma syndrome. Med J Aust 1971;2:371-3. Rosenthal CH, Noguera CA, Coppola A, et al. Pseudotymphoma with mycosis fungoides manifestations, hyperresponsiveness to diphenylhydantoin and lymphocyte disregulation. Cancer 1982;49:2305-14. Wolf R, Kahane E, Sandbank M. Mycosis fungoides-like lesions associated with phenytoin therapy. Arch Dermatol 1985;121:1181-2. Rijlaarsdam U, Scheffer E, Meijer CJLM, et al. Mycosis fungoides-like lesions associated with phenytoin and carbamazepine therapy. J AM ACAODERMATOL1991;24:21620. Adams JD. Localized cutaneous pseudolymphoma associ-

17. 18. 19. 20. 21. 22.

ated with phenytoin therapy: a case report. Australas J Dermatol 1981;22:28-9. Isobel T, Hormatsu T, Fujita T, et al. Adult T-celt [ymphoma following diphenylhydantoin therapy. Acta Haematol Japonica (Kyoto) 1980;43:711-4. Li F, Willard DR, Goodman R, et al. Malignant lymphoma after diphenylhydantoin (Dilantin) therapy. Cancer 1975;36:1359-62. Gams RA, Neal JA, Conrad FG. Hydantoin-induced pseudo-pseudolymphoma. Ann Intern Med 1968;69:55768. Rubinstein I, Langevita P, Shiabi G. Isolated malignant lymphoma of the jejunum and long-term diphenylhydantoin therapy. Oncology 1985;42:104-6. Hood AF, Kwan TH, Burnes DC, et al. Primer of dermatopathology. Boston: Little, Brown and Company, 1984:171, 178. Waldmann TA, Davis MM, Bongiovanni KF, et at. Rearrangements of genes for the antigen receptor on T-cells as markers of fineage and clonality in human lymphoid neoplasms. N Engl J Med 1985;313:776-83.

Toxic epidermal necrolysis in early infancy M e g i n C. Scully, M D a and Ilona J. Frieden, M D a,b San Francisco, California Toxic epidermal necrolysis ( T E N ) is a life-threatening bullous dermatosis characterized by the sudden onset of full-thickness epidermal necrosis. T E N is a disease of both children and adults, but T E N in early infancy is a rare event; only two well-documented cases in infants less than 6 months of age have been reported. We report a third case of a 6-week-old infant with Escherichia coli sepsis who received ampicillin and other antibiotics and subsequently developed TEN. Despite the withdrawal of ampicillin and aggressive systemic and wound care, the infant died. The infants in the other two reported cases also died, which suggests that T E N in early infancy has an extremely poor prognosis. (J AM ACAD DERMaTOL 1992;27: 340-4.) Toxic e p i d e r m a l necrolysis ( T E N ) is a life-threatening bullous dermatosis c h a r a c t e r i z e d b y the sudden onset o f extensive e p i d e r m a l necrosis. Mucous m e m b r a n e s are f r e q u e n t l y involved, and systemic toxicity is severe. T E N occurs most frequently as a result of drugs, l, 2 b u t it has also been reported in association with a n u m b e r o f conditions including graft-versus-host disease ( G V H D ) , 3,4 infection, s and malignancy. 6 T h e pathogenesis of T E N has yet

to be determined. M a n y accept an immune-mediated mechanism 7, 8; others believe that T E N is mediated by toxic metabolites. 9, l0 T E N is a disease of both adults and children, 11, 12 but T E N in early infancy is a rare event, with only two biopsy-confirmed cases involving infants less than 6 months of age. 13, 14 W e report a third case of a 6-week-old infant with Escherichia coli sepsis and renal insufficiency who subsequently developed TEN.

From the Departments of Dermatologya and Pediatrics? School of Medicine, Universityof California,San Francisco, Presentedat the FifteenthAnnual Meetingof the Societyfor Pediatric Dermatology,San Diego, Calif., Aug. 9-11, 1991. Reprints not available. 16/4/M471

CASE R E P O R T

340

The infant, a product of a normal pregnancy and delivery, was apparently well until 2 weeks of age, when he developed E. cob sepsis and shock. After initial treatment with intravenous anapicillin and gentamicin, a diagnostic

Volume 27 Number 2, Part 2 August 1992

Toxic epidermal necrolysis in early infancy 341

Erythrode~rnic,bullae Trauma~fblisters Urine~

Blood+ E.coli

MEDICATIONS

Abscess+ E.coli

Expl~ed

Ampicillin Gentarnicin

WNNN

Cefotaxime Vancomycin

m[]m

Ceftazidime Clindamycin 0

,,&,

I

14

I

21

i

28

I

35

I

42

i

49

,I

56

DAYS OF LIFE Fig. 1. Medications received by patient (y axis) and days of life on which they were administered (x axis).

Fig. 2. Infant at 37 days of age with erythema, erosions, and blisters over hand and forearm. workup revealed bilateral hydronephrosis with decreased creatinine clearance, hydroureter, and reflux resulting from posterior urethral valves. A Foley catheter was placed, peritoneal dialysis was begun, and definitive surgery was planned. The patient received a variety of antibiotics including vancomycin, clindamycin, and cefotaxime (Fig. 1). His hospitalization was complicated by a perinephric abscess that developed at 42 days of age and required surgical drainage and a nephrostomy tube. Ab-

scess cultures grew E. coil At 47 days of age he was noted to have skin fragility at tape sites. Within a 2-hour period he developed erythema and fluid-filled blisters over his hands, wrists, and feet (Fig. 2). Findings of a frozen section of a scissor-snip biopsy specimen of the bulla roof showed extensive epidermal necrosis (Fig. 3). The blister roof contained nearly the entire thickness of the epidermis, thus confirming the diagnosis of TEN and differentiating it from staphylococ-

342

Journal of the American Academy of Dermatology

Scully and Frieden

,,,'~',,,,*,,

,

. . . .

,~.~,

.

9..

'Y',.'

, .~ J, 9 ,

,,,,,,, . ' .

,p~,~,~,

,,, t~.

~

~

~,~ .

'~ , / ,

~

'

,"' "'~,q, ....

.

.

.

~,

'

.