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Erythema elevatum diutinum in a patient with acquired immunodeficiency syndrome. Another clinical simulator of Kaposi's sarcoma.

Several types of vasculitis have been described in patients with human immunodeficiency virus infection. Erythema elevatum diutinum is a rare variant of cutaneous leukocytoclastic vasculitis which, with the exception of the case reported herein, has been described only once in human immunodeficiency virus-infected patients. Our male patient, a longtime intravenous drug abuser, had cutaneous lesions, closely resembling Kaposi's sarcoma, on the extensor surfaces of the lower extremities. Cutaneous biopsy specimens, however, demonstrated leukocytoclastic vasculitis with fibrinoid necrosis of the vessel walls and areas of basophilic degeneration of collagen bundles in early lesions, whereas late lesions showed dense diffuse fibrosis with proliferation of dermal spindle cells and some foci of residual leukocytoclastic vasculitis. Oral therapy with dapsone resulted in marked clearing of the cutaneous lesions within few days. This case raises the necessity of histologic confirmation for all cases of suspected Kaposi's sarcoma in patients with acquired immunodeficiency syndrome. We discuss the possible pathogenesis of leukocytoclastic vasculitis in human immunodeficiency virus-infected patients.

Acquired Immunodeficiency Syndrome↗

[Rickettsia conorii: a new cause of erythema nodosum].

A case of 26 year old male with an infection caused by Rickettsia conorii (RC) associated to erythema nodosum (EN) is presented. The patient had EN together with a high temperature and toxic symptoms 2 weeks after camping in the mountains. The diagnosis was made by ascertaining IgM titers positive to RC by indirect immunofluorescence (IIF). The disease disappeared 24 hours after the initiation of treatment with doxiciclin. Based on this observation, we consider RC to be an etiologic agent which should be taken into account in the differential diagnosis of EN.

Adult↗

[Erythema nodosum and pregnancy. Report of a case].

Erythema nodosum is a rare disease, it appears frequently in association with infectious disease and with some drugs administration. A case associated with pregnancy is presented. The importance of hormonal behavior in its genesis, is considered. We conclude that the transitional hormone state in women is a very important factor in it's appearance. Estrogens play an important roll in the immunological response.

Adult↗

[Urticarial erythema with neutrophilic infiltration--correlation of cutaneous vascular changes with clinical severity].

Twenty-seven cases of urticarial erythema with predominantly neutrophilic infiltration in the upper dermis were examined clinically, histologically and serologically. Their condition persisted longer than common urticaria, with transitory high fever and arthralgia being noted frequently. Based on histological examination results, the patients were divided into three groups. Ten patients with histological findings of leukocytoclastic vasculitis were diagnosed as urticarial vasculitis which was accompanied in 7 cases by systemic lupus erythematosus, Sjögren syndrome or viral hepatitis. This group frequently showed hypocomplementaemia and multiple organ involvement such as hepatitis and nephritis. An immunofluorescence study demonstrated immunoglobulin and/or complement components to be deposited in the vessel walls of upper dermis, thus implicating type III allergy in the pathogenesis. The second group consisted eight patients with moderate infiltration of neutrophils toward the vascular walls though vasculitic changes were not apparent. Systemic lupus erythematosus and Sjögren syndrome were noted in 5 of these patients but multiple organ involvement was relatively quite infrequent. Antihistamines and even systemic corticosteroids failed to have any effect in the majority of the patients on these two groups. The remaining nine patients constituting the third group showed neither neutrophilic infiltration toward vessel walls nor vascular damage and there was no multiple organ involvement. Bacterial infection of upper respiratory tract appeared to possibly be a trigger in most of these patients for whom antibiotics were effective as treatment. In conclusion, histological examination is particularly important for cases such as the present cases for accurate diagnosis and deciding appropriate treatment.

Erythema↗

[Erythema nodosum: a study of 160 cases].

We have evaluated 160 patients diagnosed of erythema nodosum (EN) on the basis of clinical and/or histopathological criteria to assess their etiology and their major epidemiological, clinical and laboratory features in our area. Mean age was 37.5 years and 86% of patients were females. The etiology could be determined in 67.5% of cases: sarcoidosis (20.6%), drugs (18%), streptococcal infection (16.3%), tuberculosis (6.3%), pregnancy (1.9%), Crohn's disease (1.3%), Behçet's disease (1.3%), lymphoma (0.6%), adenocarcinoma (0.6%), and toxoplasmosis (0.6%). 32.5% of cases were idiopathic. We found significant differences between the different etiological factors regarding mean age, fever, joint disease, ESR, leukocytosis and serum proteins. We compare our results with those from other series and we emphasize the need for a systematic workup in all patients with EN to facilitate their etiological diagnosis.

Adult↗

[HLA antigens in 11 cases of patients with recurrent erythema and anti-SS-B antibody].

HLA antigens were studied in 11 cases of patients with recurrent erythema and anti-SS-B antibody. Among DR antigens positive associations with DR5, DRw6 (including DRw13), and DRw52 were obtained. These data demonstrated that 11 cases of this report are immunogenetically closely related to each other and appear to be related to Sjögren's/lupus erythematosus overlap patients.

Antibodies, Antinuclear↗

[Erythema multiforme induced by the combination of phenytoin and cerebral irradiation].

The authors report the case of erythema multiforme characterized by its cephalic onset and its rise after association phenytoin and cranial irradiation, for glioblastoma with epileptic fits. Eight similar cases are observed in literature with the same timing. Benign outcome is not always the rule because 2 patients died. This type of reaction being not forseeing, and outcome very varying, we suggest to avoid phenytoin in patients having a cerebral tumor revealed by epilepsy and needing a cranial irradiation.

Brain↗

[The fifth disease--erythema infectiosum. Parvovirus infection in a family group].

The causative agent of Erythema Infectiosum (EI), also known as the fifth disease, has been shown to be Human Parvovirus B19. This virus may also cause foetal damage, resulting in spontaneous abortion, hydrops foetalis or stillbirth. Three cases of Human Parvovirus infection in a family are presented. The child has a classical rash. The father had a rash and joint-symptoms, which is common in adults. The mother had a subclinical infection, which is seen in the majority of EI cases. As the woman was pregnant, ultrasonic tests were carried out frequently to detect possible hydrops foetalis. This pregnancy resulted in a healthy full term infant weighing 3,610 g.

Adult↗

[Erythema migrans arciforme et palpabile (T-cell pseudolymphoma)].

A 65-year-old man with recurrent erythema migrans arciforme et palpabile (EMAP) is reported. EMAP is characterized as a T-cell pseudolymphoma, the cell populations of which are composed of T-helper- and T-suppressor cells in a ratio of 2:1. EMAP can therefore not be differentiated immunohistologically from the lymphocytic infiltration of Jessner-Kanof.

Aged↗

[Postherpetic erythema exsudativum multiforme with concomitant exacerbation of psoriasis vulgaris].

Herpes simplex virus (HSV) infection is a well-known trigger mechanism for both erythema multiforme (EM) and psoriasis. While HSV-related EM and psoriasis are not uncommon, simultaneous development of both disorders following HSV infection is relatively rare. We report on a patient who simultaneously developed both EM and a guttate psoriasis within 16 days of an HSV infection. The major differences in the pathophysiological mechanisms by which these dermatoses are triggered are discussed.

Biopsy↗

Erythema nodosum mimicking soft tissue sarcoma.

A case of painful localised swelling of the arm is described. This had the clinical features of a soft tissue sarcoma. However, histological analysis of a biopsy specimen revealed a cellular picture identical to that of erythema nodosum.

Diagnosis, Differential↗

[Aspergillus colonization of a pulmonary sequestration. Apropos of a case associated with erythema nodosum].

We describe a case of interlobar pulmonary sequestration diagnosed at thoracotomy in which there were a number of unusual features at presentation: an opacity with multiple cavities, positive aspergillus precipitins, erythema nodosum and a haemothorax complicating a trans-thoracic needle biopsy. Although it is unusual to find such a constellation of symptoms and signs they could be explained in a logical fashion.

Aspergillosis↗

[HLA pattern in patients with post-herpetic erythema exsudativum multiforme].

33 patients suffering from postherpetic erythema multiforme (PEM) were investigated with special reference to histocompatibility typing. In comparison with a control group (n = 54), we found strikingly high levels of HLA-DR1 and HLA-DR4 as well as a significantly elevated level of HLA-Cw3 in our patients (p less than 0.01).

Erythema Multiforme↗

[Role of herpes simplex virus in the development of exudative erythema multiforme].

The paper presents analysis of current knowledge on etiology and immunopathogenesis of multiform exudative erythema (MEE). Among a variety of pathogenetic actions of herpes simplex on immune system are those relevant to MEE onset. These variants are dealt with in detail. The view on MEE as resultant from herpes simplex infection promises appearance of new prospective modes of etiotropic therapy.

Adult↗

[Determination of antibodies against Borrelia burgdorferi in patients with morphea, lichen sclerosus et atrophicus and erythema chronicum migrans].

Several cutaneous entities described in Europe as Chronic Migrans Erythema (CME), Mild Cutis Lymphadenosis (MCL) and Chronic Atrophyc Acrodermatitis (CAA) constitute clinical manifestations of a Borrellia Burgdorferi. The presence of clinical and hystologic lesions similar to those of liquen esclerosus and atrophyc (LEA) and localized esclerodermia (morphea) in patients with CAA has driven to several authors to demonstrate the aethiologic participation of B. Burgdorferi in patients carrying those cutaneous lesions with contradictory results. A serologic study with indirect immunofluorescence (IIF) and FIAX test was performed in 16 patients (9 with morphea, 6 with LEA and 1 with CME) in order to evaluate the role of this microorganism in our environment. Five reactive sera were obtained of which only one presented IgG antibodies titrated at 1/256. The IgM antibodies by IIF and IgG by FIAX test turned out to be negative. A specific relationship between B. Burgdorferi and the studied entities could not be established.

Adolescent↗

The incidence of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. A population-based study with particular reference to reactions caused by drugs among outpatients.

We carried out a study to estimate the incidence of erythema multiforme (EM), Stevens Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) requiring hospitalization and to determine which drug therapies were associated with these reactions. We reviewed the clinical records of all patients who were hospitalized with these discharge diagnoses at Group Health Cooperative (GHC) of Puget Sound, Seattle, Wash, from 1972 through 1986. During this 14-year period, an average of about 260,000 persons, with demographic characteristics similar to those of the general population, received their care from GHC, and there were about 25,000 admissions to hospitals per year at the GHC hospitals. Based on International Classification of Diseases-Adapted coding, a total of 61 suspect cases of EM, SJS, or TEN were identified from the computerized hospital discharge file. Based on record review and the application of a uniform set of diagnostic criteria, a total of 37 patients (61%) were classified as having EM, SJS, or TEN. Of these, 16 cases (43%) were attributed to drugs administered to these patients prior to hospitalization. The overall incidence of hospitalization for EM, SJS, or TEN due to all causes was 4.2 per 10(6) person-years. The incidence of TEN alone due to all causes was 0.5 per 10(6) person-years. The incidence of EM, SJS, or TEN associated with drug use were 7.0, 1.8, and 9.0 per 10(6) person-years, respectively, for persons younger than 20 years of age, 20 to 64 years of age, and 65 years of age and older. Drug therapies with reaction rates in excess of 1 per 100,000 exposed individuals include phenobarbital (20 per 100,000), nitrofurantoin (7 per 100,000), sulfamethoxazole and trimethoprim, and ampicillin (both 3 per 100,000), and amoxicillin (2 per 100,000). Overall, our data suggest that cases of EM, SJS, and TEN sufficiently severe to require hospitalization are infrequent among outpatients using well-established drug therapies. A continuing challenge is the evaluation of these severe cutaneous reactions that are associated with newly marketed or less frequently prescribed drug therapies.

Adolescent↗

In situ and in vitro characterization of the cellular immune response in erythema nodosum leprosum.

We sought to evaluate cell-mediated immune responses in erythema nodosum leprosum (ENL), a reactional state occurring in lepromatous leprosy. Skin biopsies from patients with leprosy were studied with monoclonal antibodies against T lymphocyte antigenic determinants, interleukin 2 (IL 2), and IL 2 receptors (Tac) by using immunoperoxidase staining of frozen sections. Peripheral blood lymphocytes from 18 ENL patients were tested in vitro for lepromin-induced suppression of Con A stimulation. Serial studies of seven lepromatous patients who developed ENL during the course of the study showed increases in both the Leu-3a:Leu-2a ratio and the number of IL 2-positive cells. IL 2-positive cells comprised 0.3% of the cells in all of the ENL lesions studied as compared with the 0.03% found in nonreactional lepromatous lesions (P less than 0.001). Lepromin-induced suppression of the Con A response, present in nonreactional lepromatous patients, significantly decreased in patients developing the ENL reaction, but returned after recovery from ENL. These changes in tissues and peripheral blood suggest that the pathogenesis of ENL is related to cell-mediated immune processes. Despite these immunologic changes, however, ENL patients do not recover antigen-specific skin tests or eliminate Mycobacterium leprae.

Antibodies, Monoclonal↗