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Stevens-Johnson syndrome and toxic epidermal necrolysis are severity variants of the same disease which differs from erythema multiforme.

A new classification, based on the pattern and distribution of cutaneous lesions, separates erythema multiforme major from Stevens-Johnson syndrome. A retrospective re-classification of 76 cases supported the validity of that separation by demonstrating differing causes and pathology. Another prospective international case-control study found differing demographic characteristics and risk factors between erythema multiforme major on the one hand and Stevens-Johnson syndrome or toxic epidermal necrolysis on the other. Erythema multiforme major was mainly related to Herpes virus infection, while Stevens-Johnson syndrome and toxic epidermal necrolysis were associated with drug reactions.

Diagnosis, Differential↗

Bacterial endotoxin (lipopolysaccharide) as a cause of erythema multiforme.

The classic iris lesions in a patient with erythema multiforme bullosum were reproduced grossly as well as microscopically by the intradermal injection of a variety of heat-killed, Gram-negative bacteria, as well as their common endotoxin, lipopolysaccharide W. In vitro exposure of the patient's blood to these antigens induced specific fibrin microclots characteristic of a hypersensitivity state. It is possible that some cases of erythema multiforme associated with a variety of respiratory, gastrointestinal, or urinary tract infections may represent a single specific delayed sensitivity reaction to the bacterial endotoxin, lipopolysaccharide.

Antigens, Bacterial↗

Erythema multiforme. Clinical characteristics and natural history in fifty patients.

Erythema multiforme is a chronic mucocutaneous inflammatory disease with a variable recurrent pattern. Thirty female and twenty male patients, ranging in age from 11 to 75 years, were studied. Twelve patients had oral lesions only, nineteen had oral and lip changes, and nineteen had oral, lip, and skin involvements. Most of the patients had a noncyclical pattern of attacks which required systemic corticosteroid therapy. When the disease attacks electively were not treated, healing would vary between 2 and 24 weeks. This study confirmed the idiopathic nature and extremely variable features of erythema multiforme. Frequency, location, duration, and severity of attacks did not shed any clues as to trigger mechanisms (etiology), persons who might be predisposed to such attacks, treatment responses, or prognosis.

Adolescent↗

Erythema multiforme due to griseofulvin.

Three patients are reported who developed erythema multiforme during treatment with griseofulvin. To our knowledge there have been no previously documented reports of this drug causing erythema multiforme in either the English or American literature.

Adult↗

Erythema multiforme in childhood and early infancy.

Erythema multiforme is said to be rare in childhood and especially in early infancy. Three infants, none older than 1 year, were seen with this condition; all showed typical clinical features. In one of the three infants, no distinctive etiological factor(s) could be found. One was suffering from congenital hepatitis (cause unknown) and another from a staphylococcal infection.

Amoxicillin↗

[11 cases of erythema multiforme. The etiological correlations and therapy].

Erythema multiforme (EM) is characterised by a polymorphous eruptive complex which many involve the cutis and oral, genital and conjunctival mucous. Its etiopathogenesis is unclear: it is thought to be associated with various viral or bacterial infective agents (herpes virus, Coxsackie virus, mycoplasmas, etc.), numerous drugs, physical therapy, systemic pathologies of various types. The authors report their experience in relation to 11 patients suffering from EM; the clinical characteristics of each case are reported, together with the course of disease, and the diagnostic and therapeutic protocol adopted. The discussion examines the possible cause of disease: in 5 patients the etiology was traced back to viral infections (4 herpes and 1 coxsackie) and to the administration of drugs in the remaining cases (4 in relation to anti-phlogistic agents and 2 regarding antibiotics).

Adolescent↗

An immunopathological study of herpes-associated erythema multiforme.

Any pathogenetic mechanism proposed for erythema multiforme (EM) must account for the prominent mononuclear cell infiltrate in the skin lesions. The purpose of this study was to characterize immunopathologically, with monoclonal antibodies to human leukocyte antigens, the inflammatory cells in early target lesions of recurrent herpes-associated EM. Cryostat sections of snap-frozen skin biopsies were studied by the avidin-biotin immunoperoxidase technique with use of the following monoclonal antibodies: anti-HLA-DR, anti-Leu M5, anti-Leu 4 + 5b, anti-Leu 3a + 3b, anti-Leu 2a, anti-Leu 14, and anti-Leu 6. The dermal mononuclear inflammatory infiltrate in the EM biopsies consisted of monocyte-macrophages and T-lymphocytes, with both helper and suppressor T cells present. Both the dermal inflammatory infiltrate and the overlying keratinocytes were strongly HLA-DR positive. No definite alteration of Langerhans cell number or distribution was noted. These findings are consistent with the characteristics seen in cell-mediated immune reactions in the skin and point to this as a likely immune mechanism for the tissue damage of EM.

Adult↗

Epstein-Barr virus-related persistent erythema multiforme in chronic fatigue syndrome.

BACKGROUND: Erythema multiforme (EM) has been rarely reported in Epstein-Barr virus (EBV)-associated diseases; this includes patients with chronic fatigue syndrome who have chronic or recurrent and disabling illness and an abnormal antibody reactivity to EBV. We describe a patient fulfilling the chronic fatigue syndrome diagnostic criteria who had developed an unusually persistent EM resistant to corticosteroids therapy. The EBV DNA was studied in skin EM lesions, throat washings, peripheral mononuclear cells, and plasma. The EBV antigens were studied in skin EM lesions and in mononuclear cells. The patient was followed up to 2 years. OBSERVATIONS: The patient had abnormal titers of antibodies against various EBV antigens and by immunofluorescence she disclosed the EBV nuclear antigen and the viral capsid antigen in the blood vessels of the affected skin. The dot blot hybridization assay detected viral DNA in throat washings and mononuclear cells, but not in plasma. The presence of the viral genomic content in lesional skin is suggested by the autoradiographic signal and by the difference from appropriate control specimens. Skin lesions and constitutional symptoms cleared after acyclovir sodium therapy and recurred after discontinuation of this therapy. CONCLUSIONS: This is the first EM case in which evidence of the EBV causal role has been provided. The association with chronic fatigue syndrome suggests the EBV role in selected cases of this syndrome.

Adult↗

Vaginal stenosis due to bullous erythema multiforme (Stevens-Johnson syndrome). Case report.

Bullous erythema multiforme is a disorder in which oral and genital erosions, ocular inflammation and cutaneous bullae may follow a viral or bacterial infection or the ingestion of certain drugs, such as sulphonamides. Unpleasant sequelae have been reported including residual scarring, and death may occasionally result from infection or renal failure; severe scarring can cause blindness and infrequently the genital tract is involved. We report a patient who developed complete vaginal stenosis following bullous erythema multiforme in pregnancy.

Adult↗

Disseminated granuloma annulare following erythema multiforme minor.

A 44-year-old woman presented with erythema multiforme minor followed by disseminated granuloma annulare 4 weeks later. The patient was not taking any medication and had no history of herpes simplex infection. Involvement of a delayed-type hypersensitivity reaction in the pathogenesis of these two well known disorders, as suggested by immunological investigations, may explain their concurrence in our patient. The substitution of the erythema multiforme minor lesions by an eruption of disseminated granuloma annulare at the same sites suggests the possibility of a Koebner phenomenon or an isotopic response.

Adult↗

Skin reactions to radiotherapy--a spectrum resembling erythema multiforme: case report and review of the literature.

Eruptions resembling erythema multiforme associated with radiotherapy are rare. Although several case reports are cited in the older literature, only recently has this phenomenon been described in patients receiving external beam radiation therapy both with and without contributory concurrent medications. The eruption typically begins within the radiation port and then generalizes; it is usually self-limited, but serious and fatal outcomes are reported. We review the literature and report a case of an eruption resembling erythema multiforme associated with external beam irradiation and 5-fluorouracil.

Adenocarcinoma↗

Erythema multiforme secondary to herpes simplex infection: a case report.

BACKGROUND: Erythema multiforme (EM) is a complex disease that may have cutaneous and/or mucosal involvement. The severity may range from mild to severe and potentially life threatening. The literature cites many factors including viruses, infections, and medications as causes. This report documents a patient who developed EM secondary to a herpes simplex viral (HSV) infection. METHODS: Two weeks following an eruption of herpes labialis, a 20-year-old white female patient developed acutely painful oral and labial ulcers accompanied by target skin lesions. A diagnosis of erythema multiforme (EM) was made. The patient was treated with antivirals, analgesics, and symptomatic therapy. RESULTS: Nine days after the onset of symptoms, the oral and cutaneous lesions had started to heal and the patient no longer required pain medication. CONCLUSIONS: Although the etiology of EM is still often unknown, infections with herpes simplex virus have been implicated as a possible precipitating factor. This case illustrates the association of the occurrence of EM with an HSV infection.

Acyclovir↗

Erythema multiforme and urticaria. Eruptions induced by chemically related ophthalmic anticholinergic agents.

Erythema multiforme developed in an 80-year-old man following the use of scopolamine hydrobromide ophthalmic drops. The erythema multiforme cleared when the medication was discontinued and recurred on challenge. Later, he was given tropicamide, an anticholinergic ophthalmic preparation that, like scopolamine, has a tropic acid residue. Within 15 minutes an immediate hypersensitivity reaction with generalized urticaria developed in the patient.

Aged↗

Erythema multiforme and Stevens-Johnson syndrome in patients receiving cranial irradiation and phenytoin.

In 15 months we encountered eight patients with intracranial tumors who developed erythema multiforme (EM) or erythema multiforme bullosa (Stevens-Johnson syndrome). All occurred shortly after use of phenytoin (DPH) and brain radiation therapy (WBRT). The clinical picture differed from the classic form of EM in that the erythema began on the scalp and spread to the extremities, progressing in three cases to extensive bullous formation. There were no cases of EM among patients who received either DPH or radiotherapy alone. The combination of DPH, WBRT, and tapering of steroids seems to predispose to EM. The pathogenesis of the disorder is probably immunologic. In the absence of seizures, anticonvulsants should not be given routinely to patients with brain tumors. When anticonvulsants are necessary in patients scheduled for WBRT, DPH may not be the drug of choice.

Adult↗

Human orf infection complicated by erythema multiforme.

A 35-year-old woman developed an orf infection of the fingers after contact with an infected goat. Following the primary orf infection, erythema multiforme developed and was controlled with prednisone. The association between erythema multiforme and orf is discussed; also, it is noted that orf is another viral condition that should be added to the agents that can initiate erythema multiforme.

Adult↗

[Human orf and erythema multiforme].

BACKGROUND: Orf is a disease of sheep due to a parapoxvirus. Transmission in man is possible, and is generally benign, self-limited condition. Although, rare complications have been mentioned. We report a case of Orf with erythema multiforme in a young girl. CASE REPORT: A 13-year-old girl presented to our department with an erythema multiform. The cutaneous examination revealed painless erythematous nodule of her right index finger developed 15 days after a contact with a lamb. The diagnosis of Orf complicated by erythema multiforme was made on the basis of her typical history. Lesions heal with symptomatic therapy in 15 days. DISCUSSION: Orf presents in sheeps as a pustular dermatitis or a vesicular oral mucosal lesions, and in man as a single lesion on the site of infection. This lesion generally heals without complications, although secondary infection is not uncommon. Twenty one cases of erythema multiforme have been recorded following infection with the Orf virus. In typical cases, the diagnosis is established by a history of contact with infected animals and the appearance of the lesion. Electron microscopic examination can be helpful sometimes. Lesions regress in 4 to 5 weeks without specific treatment.

Child↗

Recurrent erythema multiforme: a possible role of foodstuffs.

Seven patients are described, who presented with a history of recurrent episodes of erythema multiforme and who were subsequently found to be sensitive, by patch testing, to benzoic acid. Advice on avoidance of benzoic acid resulted in resolution of attacks in four patients, but three patients who were not able to adhere to an exclusion diet continued to have symptoms. These findings implicate benzoic acid, a food preservative, as an aetiological factor in erythema multiforme. It is proposed that patch testing to food substances should be carried out on patients who present with recurrent erythema multiforme, since avoidance of potential allergens may lead to resolution of symptoms.

Adolescent↗