Involvement of three mucous membranes in herpes-induced recurrent erythema multiforme

Abstract

To the Editor: Classically, herpes-induced erythema multiforme (EM) appears as cutaneous target lesions with none or one mucous membrane involved. We report a case of recurrent EM manifested by severe, multiple mucous membrane involvement. A 10-year-old boy was admitted to the hospital with a 12-day history of persistent lip and mouth ulcers and intermittent fever. He was treated with a short course of cephalexin, which was discontinued after 5 days. In the past, he had two short-limited episodes of herpes labialis, the last one being 3 months before his most recent presentation. Physical examination revealed an ill-appearing, febrile boy with edema and crusting of the lips. He had extensive areas of ulceration on the anterior mouth and tongue with relative sparing of the gingiva. Similar lesions were noted at the urethral meatus. He had bilateral, nonpurulent conjunctival erythema. On his extremities, he had scattered, raised edematous papules and a few targetoid lesions with central bullae. The rest of the examination was unremarkable. Polymerase chain reactions (PCR) for Mycoplasma pneumoniae and Herpes simplex viruses (HSV) were negative. He was clinically diagnosed with EM major and treated empirically with acyclovir for 10 days and oral steroids for 3 weeks. Six months later, he developed one small ulcer on his lower lip and was started on oral acyclovir. Ten days later he was noted to have a fever, extensive oral ulcerations, and one genital ulcer. His eye examination revealed blepharitis and mild conjunctival erythema. No skin lesions were noted. He had evidence of IgG HSV antibodies. He required treatment with oral steroids because of the severity and extent of the oral lesions. Four weeks after discharge, while still on low-dose oral steroids, he developed a new vesicle on his upper lip. Electron microscopy and PCR from the lesion revealed HSV-1. He was started on acyclovir for 7 days and continued on a prophylactic dose (200 mg orally twice a day) for 1 year. Oral steroids were weaned slowly over a few months. He had no new episodes of HSV infection or EM at 1-year follow-up. EM is a postinfection hypersensitivity reaction characterised by acrally-distributed target lesions or raised edematous papules associated with one or no mucosal lesions.1.Huff J.C. Weston W.L. Tonnesen M.G. Erythema multiforme: a critical review of characteristics, diagnostic criteria, and causes.J Am Acad Dermatol. 1983; 8: 763-775Abstract Full Text PDF PubMed Scopus (403) Google Scholar, 2.Ayangco L. Rogers III, R.S. Oral manifestations of erythema multiforme.Dermatol Clin. 2003; 21: 195-205Abstract Full Text Full Text PDF PubMed Scopus (129) Google Scholar, 3.Fabbri P. Panconesi E. Erythema multiforme (“minus” and “maius”) and drug intake.Clin Dermatol. 1993; 11: 479-489Abstract Full Text PDF PubMed Scopus (24) Google Scholar The most common infectious agent responsible for this classical description is HSV (63% of cases).1.Huff J.C. Weston W.L. Tonnesen M.G. Erythema multiforme: a critical review of characteristics, diagnostic criteria, and causes.J Am Acad Dermatol. 1983; 8: 763-775Abstract Full Text PDF PubMed Scopus (403) Google Scholar Recurrent EM is most frequently caused by reactivation of herpes infection. In 70% of patients there is a temporal relationship between HSV infection and recurrent EM (2-17 days).4.Schofield J.K. Tatnall F.M. Leigh I.M. Recurrent erythema multiforme: clinical features and treatment in a large series of patients.Br J Dermatol. 1993; 128: 542-545Crossref PubMed Scopus (139) Google Scholar If present, mouth lesions are the most common mucosal manifestations of recurrent EM (69%) and consist of primarily anterior mouth involvement.4.Schofield J.K. Tatnall F.M. Leigh I.M. Recurrent erythema multiforme: clinical features and treatment in a large series of patients.Br J Dermatol. 1993; 128: 542-545Crossref PubMed Scopus (139) Google Scholar We are reporting a case of herpes-induced recurrent EM in which mucosal lesions were more extensive (3 mucous membranes) and more severe than previously reported. In addition, one of the episodes was characterised by mucosal lesions alone. We felt that HSV was the trigger in all of the episodes (4/4 Assier criteria).1.Huff J.C. Weston W.L. Tonnesen M.G. Erythema multiforme: a critical review of characteristics, diagnostic criteria, and causes.J Am Acad Dermatol. 1983; 8: 763-775Abstract Full Text PDF PubMed Scopus (403) Google Scholar Moreover, no further episodes of HSV infection or EM were noted on acyclovir prophylaxis, at 12-month follow-up visit. Our case highlights the fact that the extent and the severity of the mucosal lesions are not helpful in making etiological assumptions in patients with EM.