[A case of erythema exudativum multiforme associated with hydantoin treatment].
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Erythema multiforme (EM) is an immune-mediated disease categorized into EM minor and EM major, also called Stevens-Johnson syndrome. The presence of mucosal involvement differentiates erythema multiforme major from erythema multiforme minor. Many drugs and agents can induce Stevens-Johnson syndrome. We report a case of Stevens-Johnson syndrome associated with Mycoplasma pneumoniae infection. Lymphopenia with a significant decrease of CD4+ T cells in the blood and predominant CD4+ T cells in the skin vesicular fluid was found. The improvement of lymphopenia was associated with disease recovery. In a retrospective chart review of patients treated in our hospital over the past 3 years, we found that 5 patients with Stevens-Johnson syndrome all had lymphopenia (< 1.50 x 10(9)/L; average 0.99 x 10(9)/L), whereas 13 other patients with erythema multiforme minor demonstrated normal lymphocyte counts (average 3.13 x 10(9)/L), with the exception of one patient with herpes infection showing lymphopenia. These results suggested that an immunopathogenesis involving redistribution of CD4+ T cells might contribute to the development of Stevens-Johnson syndrome. Further studies to investigate the involvement of CD4+ T cells in Stevens-Johnson syndrome may implicate a specific strategy to prevent fatal Stevens-Johnson syndrome.
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A young male patient developed an erythema multiforme-like eruption following an accidental exposure to 1-chloromethylnaphthalene (1-CMN). In addition to the skin lesion, he suffered from liver involvement and tear insufficiency. Positive results of a patch test with 1-CMN and an in vitro lymphocyte transformation test suggested that direct exposure of the skin to chemical compounds was the probable cause of his symptoms.
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Infection with parvovirus B19 may result in a wide range of dermatologic manifestations. The specific skin findings include erythema infectiosum and papular purpuric "gloves-and-socks" syndrome. The nonspecific findings include reticular erythema, maculopapular eruptions, and petechiae and purpura, as well as other less frequently described findings. Associations with other dermatologic diseases, such as erythema multiforme and erythema nodosum, also have been described. A role in the pathogenesis of various collagen vascular disorders has been suggested and is under investigation. The diagnosis of infection rests on the typical clinical findings. Whenever parvovirus B19 infection is diagnosed, the physician must ensure that neither the patient nor his or her contacts is a member of certain vulnerable populations. In these populations, infection with parvovirus B19 may result in devastating complications. The vulnerable populations include those with hematologic disease, immunosuppressed patients, and pregnant women. Treatment of infection in the healthy immunocompetent individual is asymptomatic, and the acute infections typically resolve without complications.
Azathioprine is used in a variety of dermatological conditions. However, because of its side-effect profile, azathioprine is limited for use in patients with severe disease. An unpredictable, rare and potentially fatal side-effect of azathioprine is the development of a hypersensitivity reaction, often consisting of fever, hypotension and oliguria. We describe a 17-year-old patient with leucocytoclastic vasculitis who was placed on azathioprine; within 15 days of start of therapy, she developed a fever. Azathioprine was discontinued and an evaluation for sepsis was undertaken; all cultures were negative and the fever abated. Azathioprine was restarted 5 days later. After a single dose, fever, nausea and vomiting, diarrhoea, hypotension, tachycardia and oliguria developed and the patient was admitted to an intensive care unit. Azathioprine was discontinued and investigations revealed no sign of an infection. All the above signs and symptoms abated within 24 h and the patient was discharged from hospital in 7 days. A review of 28 case reports in the literature of azathioprine-induced hypersensitivity reactions suggest that most commonly a fever and gastrointestinal symptoms occurred on initial presentation. In addition, a maculopapular rash, urticaria, vasculitis, erythema multiforme or erythema nodosum may occur. Hepatotoxicity and nephritis have also been reported. The aetiology of the reaction is unknown but sudden onset of fever and hypotension suggests that this reaction may be due to cytokine or mediator release induced by azathioprine. As azathioprine is metabolized to 6-MP, rechallenges to both should be avoided in patients who experienced an azathioprine hypersensitivity-like reaction.
The two major cutaneous expressions of infective states are infections of the skin by viable organisms and immunological responses to nonviable microbial antigens or, in the case of molecular mimickry, their human analogues. These immunological responses are designated as cutaneous id reactions, and manifest a histomorphology similar to that seen at the primary infective site. This study presents the clinical and histological findings in 16 patients who developed skin eruptions associated with extracutaneous or systemic infections. There was a striking female predominance; patients ranged in age from 10 to 78 years. The majority of cases manifested skin lesions which clinically resembled Sweet's syndrome, erythema multiforme and/or erythema nodosum. Fever, arthralgia, oligoarthritis, mucosal ulcers of the mouth and/ or genital tract and uveitis were additional features in some cases. Isolated clinical presentations included a petechial rash in a stocking and glove distribution, papular dermatitis, a morbilliform eruption and annular erythema. Among the medical and family histories were atopy and stigmata associated with connective tissue disease (CTD). Two patients were ingesting drugs with known immune dysregulating properties. Skin biopsies showed focal lymphocytic interface dermatitis, a diffuse interstitial histiocytic infiltrate, and a mononuclear cell predominant vascular reaction which in some cases represented vasculitis by virtue of manifesting concomitant luminal or mural fibrin deposition. Eosinophils, eczematous alterations, and papillary dermal edema were identified in a minority of cases. All patients had evidence of a prior or concurrent infection, based on either positive IgM serology for specific microbes or cultures. Among the implicated pathogens were cytomegalovirus, parvovirus B19, streptococcus, mycoplasma, klebsiella, and Borrelia burgdorferi. All of these organisms are among those associated with reactive arthritis, a phenomenon that was seen in some cases. The histology suggested florid cell mediated immunity (CMI), which the authors attributed to the superantigen properties held by the aforesaid pathogens. Skin lesions and constitutional symptoms resolved quickly with antimicrobial therapy in 7 of 9 cases causally linked to bacteria. Spontaneous resolution occurred in 5 of 6 virally mediated eruptions. The other 4 patients were given topical steroids or prednisone; these included 1 patient with Borrelia burgdorferi infection and 1 patient with radiographic evidence of pneumonia who was never cultured, 1 patient with parvovirus B19 infection, and 1 patient with pneumococcal pneumonia and concomitant sarcoidosis. It is the authors' belief that the eruptions seen in these patients may in part reflect a genetic or iatrogenic predisposition to respond excessively to certain infectious triggers.