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Erythema multiforme in children. Response to treatment with systemic corticosteroids.

It is generally accepted that the correct treatment for patients with severe erythema multiforme is systemic corticosteroids. This paper is a review of thirty-two paediatric patients with severe erythema multiforme (Stevens-Johnson syndrome) who were treated with either large doses of systemic corticosteroids or supportive care only. Those patients treated with steroids did not recover sooner than those treated in other fashions and the steroid treated group had a significant incidence of medical complications. This retrospective study proves nothing but it does suggest that treatment of patients with the Stevens-Johnson syndrome with systemic corticosteroids may be associated with significant side effects and prolonged recovery.

Adolescent

Kawasaki's disease appearing as erythema multiforme.

In 1967, Kawasaki reported an acute, febrile, mucocutaneous condition accompanied by swelling of cervical lymph nodes that affected infants and young children in Japan. He called it mucocutaneous lymph node syndrome. We report here a case of Kawasaki's disease with characteristic findings that demonstrated a typical erythema multiforme rash. With the increased number of reported cases of Kawasaki's disease in the United States, it becomes increasingly important to differentiate the exanthem of Kawasaki's disease from early erythema multiforme.

Diagnosis, Differential

Erythema multiforme. Review of twenty-six cases.

A survey of twenty-six cases of erythema multiforme was undertaken. All patients had oral lesions and seventeen had skin involvement, mainly on the hands. The average age was 32 years, and female patients predominated in the group. Nearly one third gave a history of a severe emotional disturbance prior to the attack, four patients developed lesions after penicillin therapy, and two presented initially with acute primary herpes simples stomatitis. Skin and mucosal biopsies were undertaken in some patients, and it was concluded that no pathognomonic features were identifiable; a subepithelial bulla, however, could occasionally help in the differential diagnosis. Other laboratory investigations, including a complete blood count, determination of herpesimplex titer, and urinalysis, were not helpful, except that the erythrocyte sedimentation rate was raised in sever cases. Possible etiologic factors were discussed in relation to the survey. It was thought that the cause is still obescure except in drug-induced cases and that the diagnosis is essentially a clinical one, since various laboratory investigations cannot be shown to produce consistent pathognomonic findings.

Adolescent

Detection of HSV-specific DNA in biopsy tissue of patients with erythema multiforme by polymerase chain reaction.

Formalin-fixed paraffin-embedded skin biopsies of lesions of erythema multiforme (EM) from 32 patients and 13 controls were examined for the presence of herpes simplex virus (HSV) by polymerase chain reaction (PCR) and for histological findings by direct immunofluorescence and staining with haematoxylin and eosin. HSV-specific DNA was detected in 23 (72%) patients. A history of recurrent skin rash was present in 59% of the PCR-positive cases, while 55% had had suspected HSV infections. Only two PCR-positive specimens were found in patients without a history of recurrent rash and/or previous oral lesions. One biopsy was positive for HSV by conventional cell cultures. There was no significant difference in histology between HSV-related and HSV-negative cases of EM. In the 13 control specimens [bullous pemphigoid (3), dermatitis herpetiformis (2), lichen planus (1), aphthous ulcer (1), fixed-drug eruption (1), varicella-zoster (1), hypereosinophilic syndrome (1), photocontact dermatitis (1), contact dermatitis (1), and cellulitis (1)], no HSV-DNA was detected.

Adult

A case of unusual SLE related syndrome characterized by erythema multiforme, angioneurotic edema, marked hypocomplementemia, and Clq precipitins of the low molecular weight type.

Our patient and those of Agnello et al. had identical clinical symptoms such as erythema multiforme, arthralgias and angioneurotic edema and both differed from systemic lupus erthematosus in several important points, i.e., in spite of marked hypocomplementemia the nephropathy is not prominent and it needs high-dose steroids to eliminate the clinical and serological abnormalities. In addition to the features reported by Agnello et al. we found increased viral antibody titers in our patient's sera.

Adult

Erythema nodosum and erythema multiforme associated with milker's nodules.

An epidemic of 44 cases of milker's nodules was recorded in the Tampere Central Hospital catchment area in Finland during the autumn of 1974. Exanthema or erythema multiforme-like secondary eruptions were seen in 10 cases. One female patient is reported in detail because of the simultaneous occurrence of erythema nodosum and erythema multiforme. The virological diagnosis was confirmed by electron microscopy.

Adult

The staphylococcal scalded-skin syndrome versus erythema multiforme.

This article reports a case of scalded-skin syndrome in a young child. Characterized by a generalized exfoliative dermatitis resembling scalded skin, the disease may involve the oral mucosa and must be differentiated from Stevens-Johnson syndrome (erythema multiforme). Since the etiology is specific, the disease can be treated with antibiotics with dramatic results.

Administration, Oral

Detection of viral DNA within skin of healed recurrent herpes simplex infection and erythema multiforme lesions.

The polymerase chain reaction (PCR) was used to detect HSV DNA in genomic DNA extracted from skin biopsies obtained from healed skin of five patients with hyperpigmented macules following recurrent cutaneous HSV infections and from eight patients with HSV-associated erythema multiforme (EM). A 92-bp HSV-1 DNA fragment was found in all the skin biopsies from the site of recurrent HSV infection and in five of eight (62%) biopsies from the EM patients. Virus DNA was not found in tissues distant from the site of HSV recurrence or from a patient without a history of HSV infection. These findings confirm the presence of HSV in healed skin from the site of recurrent HSV disease and are consistent with the concept that HSV is involved in EM pathogenesis.

Adolescent

T lymphocytes expressing HECA-452 epitope are present in cutaneous acute graft-versus-host disease and erythema multiforme, but not in acute graft-versus-host disease in gut organs.

Lymphocytes in formalin-fixed skin biopsies from patients with cutaneous acute graft-versus-host disease (aGVHD) were studied with HECA-452 (an antibody recognizing lymphocytes with skin-homing properties) and a panel of antibodies recognizing pan-B (L26 [CD20]), pan-T (L60 [CD43] and A6 [CD45RA]), and T-helper subset (OPD4) antigens in paraffin sections. Biopsies from patients with erythema multiforme (EM) were similarly studied for comparison. In both conditions, T lymphocytes stained by OPD4 were predominantly confined to the dermis, whereas those stained by HECA-452 were concentrated in the epidermis; however, there was considerable variation between cases, and overlap between findings in the dermis and epidermis. Lymphocytes similarly studied in paraffin sections of liver, salivary gland, and gut affected by aGVHD were essentially unreactive with HECA-452, although they were largely stained by pan-T markers and showed some comparable reactivity with OPD4. The findings suggest that aGVHD of the skin is mediated by a different set of lymphocytes than in gut organs, and may have a similar immunologic mechanism to EM.

Acute Disease

Abnormal root development, probably due to erythema multiforme (Stevens-Johnson syndrome).

A case report is presented of a patient in whom nearly all teeth of the permanent dentition still present had short roots, while some teeth had never been formed at all. It may be concluded from the typical differences in length between the roots of the various teeth that this condition must be due to complete cessation of the growth of the teeth at the age of 7 or 8 years. Since the patient suffered a fulminant attack of erythema multiforme (STEVENS-JOHNSON syndrome) at this age, and since no other possible explanation of the short roots has been found, it is concluded that this clinical condition may have been the reason for the short root anomaly found. Damage or even destruction of the epithelial root sheath during the disease may be assumed to be the direct cause of the failure of full root development.

Adult