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Erythema multiforme majus (EMM), Stevens-Johnson syndrome (SJS), and Toxic epidermal necrolysis (TEN) were originally believed to be a spectrum of severe cutaneous adverse reactions. More recently it has been debated that EMM is a separate entity from SJS/TEN.1
EMM is found most often in young males. Herpes is the principle risk factor.1, 2 Multiple target lesions are present, affecting <10% of the body surface area (BSA). Rash is symmetric, with the distribution beginning acrally (dorsal surfaces of hands, feet, elbows, and knees).2, 3
SJS and TEN are believed to be severity variants of a single entity.1 SJS affects <10% BSA and TEN is severe, with >30% BSA involved. Drugs are the most common etiology for SJS/TEN.1 A flulike prodrome occurs 7-14 days before lesions. The initial rash is on the face, neck, chin, and central trunk. Mucosal involvement is extensive.2
Case Report
A 32 year old Asian female presented to a minute clinic with a 3 day history of “rash” on the dorsal surfaces of her hands and arms. She was given a Medrol dosepack and Benadryl. Day 7 she presented to the dermatology office with rash extending to her legs and trunk, covering 30% BSA. The distinct demarcation of areas involved versus non-involved was striking. Although this did not follow neural dermatomes, it did have a dermatomal-like pattern. The patient reported fever and cough 3 days before the onset of her rash. Her WBC count was 14.4K. Her HSV1 and mycoplasma pneumonia titers were elevated. Hepatitis panel was negative. Current medications were dyazide, zyrtec and ariane. She was placed on 70mg PO of prednisone qd, and cipro.
After 12 days of high dose steroids, the vesicles became bullae and coalesced. The patient was in constant pain, with WBC count remaining elevated. At this point, ~70% BSA was involved. Her lesions spared only her head, face, upper chest, groin and buttock regions. (Figures 1 and 2) The rash continued to be extremely well delineated, following dermatomal planes. Day 19 she returned to the office with oral lesions. IVIG antibody was initiated in an outpatient center at a dosage of 2 mg/kg over four hours.
One day following IVIG treatment, the lesions began to resolve and by day five most of the denuded skin was falling off. The reaction subsided completely within a couple of weeks.
Discussion
This case is difficult to classify. The rash initially presented on the acral surfaces, however the delineated, almost dermatomal appearance it evolved into was unique. Through its course, it continued to spare the head, neck, face and upper chest which are commonly affected areas with SJS/TEN. The high titer of HSV and M. Pneumonia, and the lack of commonly associated drugs support a diagnosis of EMM. However, the prodromal illness 3 days prior to onset of rash and the involvement of 70% BSA is more often found in SJS/TEN. This case is an interesting example of what we believe to be EMM in a very unique and conflicting presentation.
REFERENCES
1. Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schroder W, Roujeau JC. Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study. Arch Dermatol. Aug 2002;138(8):1019-1024.
2. Williams PM, Conklin RJ. Erythema multiforme: a review and contrast from Stevens-Johnson syndrome/toxic epidermal necrolysis. Dent Clin North Am. Jan 2005;49(1):67-76, viii.
3. Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. Ann Allergy Asthma Immunol. Apr 2005;94(4):419-436; quiz 436-418, 456.
4. Rzany B, Hering O, Mockenhaupt M, et al. Histopathological and epidemiological characteristics of patients with erythema exudativum multiforme major, Stevens-Johnson syndrome and toxic epidermal necrolysis. Br J Dermatol. Jul 1996;135(1):6-11.
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