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[Leser-Trélat syndrome, erythema anulare centrifugum Darier, breast cancer and basaliomas in a patient].

A 46 year old woman had suffered from Erythema anulare centrifugum Darier (EAC) since 20 years. During a rush of the erythema numerous seborrhoic keratoses appeared (Leser-Trélat sign/LTS). Clinical examination revealed a ductular carcinoma of the breast. During the last two years, both types of lesions increased although the tumor had been treated. In addition, the patient developed several basal cell epitheliomas at forehead and chest.

Breast Neoplasms↗

[Lyme arthritis (erythema migrans arthritis)].

In the light of an observation of a patient with Lyme arthritis living near the Swiss-German border, the authors describe "Lyme or erythema migrans disease". The clinical findings include erythema chronicum migrans, meningoradiculitis and arthritis, and, occasionally, carditis. Also, it has recently become possible to describe this entity serologically, by detection of antibodies against the etiologically responsible spirochetes, which are usually transmitted by tick bites. This diagnostic procedure can also be expected to provide a better overview of the incidence of Lyme arthritis in our region.

Antibodies, Bacterial↗

[Fixed pigmented erythema caused by levamisole administration].

INTRODUCTION: Description of a case of fixed drug eruption induced by levamisole. OBSERVATION: Fixed toxic erythema with pigmentation was observed in a patient taking levamisole as an adjuvant to 5 fluorouracil chemotherapy for cancer of the colon. The imputability of levamisole was highly likely. Epicutaneous skin tests were negative. DISCUSSION: Several types of skin eruptions have been described after intake of levamisole. It was highly likely that levamisole was implicated here and fixed pigmented erythema should be added to the list.

Adenocarcinoma, Mucinous↗

[A case of complete remission of metastatic skin carcinoma (erythema type) from advanced gastric cancer by CDDP administration].

A 75-year-old female with skin metastasis from advanced gastric cancer (portion A, tub2, ss beta, n2, P0H0, Borrmann 3-type) was successfully treated with CDDP at a dosage of 25 mg/body every week or two. She had undergone R2 curative subtotal gastrectomy in October 1986, and had orally taken tegafur 300 mg/day as postoperative adjuvant chemotherapy. Three years after the operation, she had skin erythema at chest and neck. A pathological examination revealed that the skin erythema was a metastasis of gastric cancer. After administration of 5-FU and MMC, the skin lesion diminished in size, but in November of 1990, it expanded again. Administration of 5-FU, MMC and epirubicin was not effective. The skin lesion expanded extensively and changed to nodular type. CDDP was intermittently administered from August 1991 to December 1991, 25 mg/body div. every week or two; the total volume of CDDP reached 225 mg. The metastatic skin carcinoma disappeared and a complete response was obtained. CEA, which had been high (190 ng/ml), regained the normal level. Thrombocytopenia and liver dysfunction developed as side effects, but reduced after cessation of CDDP administration. The patient has been well for 3 years and 6 months since the onset of the skin metastasis.

Adenocarcinoma↗

Potassium iodide in erythema nodosum and other erythematous dermatoses.

Potassium iodide therapy has a history of more than 150 years. It has been tried in many diseases in the past. However, with the development of modern medications indications for potassium iodide therapy are very limited. It is well known that potassium iodide is the drug of choice for sporotrichosis. Subacute nodular migratory panniculitis and erythema nodosum have also been treated successfully with this drug.

Erythema Multiforme↗

Necrolytic migratory erythema. Distinctive dermatosis of the glucagonoma syndrome.

Glucagon-secreting tumors of the pancreatic islets (glucagonomas) produce a distinctive syndrome in which weight loss, diabetes mellitus, anemia,and prominent mucocutaneous findings occur. The cutaneous component-necrolytic migratory erythema--may be polymorphous, but most commonly manifests as erosions and crusts of the groin, perineum, buttocks, distal part of the extremities, and central area of the face. Alternatively, scaly papules and plaques may predominate in these areas. The eruption may resemble such dermatoses as pemphigus foliaceus, acrodermatitis enteropathica, chronic mucocutaneous candidiasis, psoriasis, and severe seborrheic dermatitis. Two patients with chronic, previously undiagnosed dermatoses had necrolytic migratory erythemia, which led to the discovery of glucagonomas present in each. In one patient surgical resection of the tumor resulted in total clearing of the rash within 48 hours. Awareness of this distinctive entity may lead to early diagnosis and, possibly, cure.

Adenoma, Islet Cell↗

Sweet's syndrome and erythema nodosum: the simultaneous occurrence of 2 reactive dermatoses.

BACKGROUND: The simultaneous occurrence of Sweet's syndrome (SS) and erythema nodosum (EN) in 1 patient is rare. Our review of the literature revealed only 11 biopsy-proved cases in which the 2 reactive dermatoses occurred together. None were associated with an underlying malignant neoplasm. OBSERVATIONS: We report a biopsy-proved case of SS and EN occurring simultaneously in a patient with an underlying malignant neoplasm (specifically, acute myelogenous leukemia). We also report another biopsy-proved case of SS and EN occurring simultaneously in a patient with underlying Crohn's disease. CONCLUSIONS: The simultaneous occurrence of SS and EN in 1 patient is rarely reported. Both disorders are reactive dermatoses that share many overlapping features. Although individually distinctive, SS and EN are also part of a growing continuum of reactive dermatoses. Our expanded understanding of the similarities and simultaneous manifestation of SS and EN may help us in the future to identify a common underlying mechanism of pathogenesis.

Adult↗

Solitary erythema migrans in Georgia and South Carolina.

OBJECTIVE: To evaluate the incidence of Borrelia burgdorferi infection in humans with erythema migrans (EM) in 2 southeastern states. DESIGN: Prospective case series. SETTING: Family medicine practice at academic center. PATIENTS: Twenty-three patients with solitary EM lesions meeting Centers for Disease Control and Prevention (CDC) criteria for Lyme disease. INTERVENTIONS: Patients underwent clinical and serologic evaluation for evidence of B burgdorferi infection. All lesions underwent photography, biopsy, culture and histopathologic and polymerase chain reaction analysis for B burgdorferi infection. Patients were treated with doxycycline hyclate and followed up clinically and serologically. MAIN OUTCOME MEASURES: Disappearance of EM lesions and associated clinical symptoms in response to antibiotic therapy; short-term and follow-up serologic assays for diagnostic antibody; growth of spirochetes from tissue biopsy specimens in Barbour-Stoenner-Kelly II media; special histopathologic stains of tissue for spirochetes; and polymerase chain reaction assays of tissue biopsy specimens for established DNA sequences of B burgdorferi. RESULTS: The EM lesions ranged from 5 to 20 cm (average, 9.6 cm). Five patients (22%) had mild systemic symptoms. All lesions and associated symptoms resolved with antibiotic therapy. Overall, 7 patients (30%) had some evidence of B burgdorferi infection. Cultures from 1 patient (4%) yielded spirochetes, characterized as Borrelia garinii, a European strain not known to occur in the United States; 3 patients (13%) demonstrated spirochetallike forms on special histologic stains; 5 patients (22%) had positive polymerase chain reaction findings with primers for flagellin DNA sequences; and 2 patients (9%) were seropositive for B burgdorferi infection using recommended 2-step CDC methods. No late clinical sequelae were observed after treatment. CONCLUSIONS: The EM lesions we observed are consistent with early Lyme disease occurring elsewhere, but laboratory confirmation of B burgdorferi infection is lacking in at least 16 cases (70%) analyzed using available methods. Genetically variable strains of B burgdorferi, alternative Borrelia species, or novel, uncharacterized infectious agents may account for most of the observed EM lesions.

Adult↗

Necrolytic acral erythema: a patient from the United States successfully treated with oral zinc.

BACKGROUND: Recently, necrolytic acral erythema (NAE) has been described as a cutaneous marker for hepatitis C virus (HCV) infection. Only 2 cases have been reported in the United States. Successful remission has been induced only with interferon therapy with or without ribavirin. OBSERVATIONS: We describe a 46-year-old, HCV-positive African American woman with well-defined, dusky, erythematous plaques on the dorsa of the feet, Achilles tendons, legs, knees, and elbows. Histologic examination revealed confluent upper epidermal necrosis, acanthosis, papillomatosis, and superficial and deep perivascular inflammation. She was diagnosed as having NAE. We induced successful disease remission with oral zinc administration. This is the third NAE case reported in the United States and the first report of disease remission with oral zinc therapy alone. CONCLUSIONS: Since its initial description in Egypt, more cases of NAE are being reported in the United States. Increased awareness of this entity is crucial. Oral zinc might represent a less toxic alternative therapeutic option for patients with NAE.

Acrodermatitis↗

Clinical relevance of different IgG and IgM serum antibody responses to Borrelia burgdorferi after antibiotic therapy for erythema migrans: long-term follow-up study of 113 patients.

OBJECTIVES: To investigate the kinetics of anti-Borrelia burgdorferi antibodies for a minimum of 1 year after antibiotic therapy in patients with erythema migrans (EM) and to correlate antibody titer kinetics with clinical variables. DESIGN: Retrospective study of serial anti-B burgdorferi antibodies in correlation to clinical variables. SETTING: University-based hospital. PATIENTS: One hundred thirteen patients with EM. INTERVENTIONS: Pretreatment and a median of 4 consecutive posttreatment serum samples from median follow-up of more than 400 days were simultaneously investigated for anti-B burgdorferi IgG and IgM antibodies. Semiquantitative titers were plotted to identify different groups of antibody kinetics. Individual patients were then stratified to those groups according to their antibody development. A statistical comparison of clinical and therapy-related characteristics among the serologic groups was performed. RESULTS: Anti-B burgdorferi IgG and IgM antibody titers developed in 3 distinct courses: persistent positivity across follow-up (IgG: 12 patients, 11%; IgM: 14, 12%), persistent negativity (IgG: 63, 56%; IgM: 47, 42%), and decrease of a positive pretreatment titer to a negative titer approximately 5 months after therapy (IgG: 34, 30%; IgM: 49, 43%). Statistics revealed significant correlations only between persistent positive IgG titers and long disease duration or large EM lesions before therapy. CONCLUSIONS: Long duration or large size of EM before therapy correlates with persistence of a positive anti- B burgdorferi IgG antibody titer after therapy. Serologic profiles do not depend on the type or duration of therapy or the clinical course thereafter. Thus, antibody testing in the follow-up of patients with EM is inappropriate for the assessment of therapeutic response.

Adolescent↗

Association of herpes simplex virus-induced erythema multiforme with the human leukocyte antigen DQw3.

We studied the genetic markers in human leukocyte antigen (HLA) region HLA-A, -B, -C, -DR, -DQ, C2, Bf, C4A, and C4B of 31 patients with erythema multiforme (EM) and their families. In contrast to the complement allotypes, which showed no deviation from the distribution in the normal population, two HLA class II antigens occurred in much higher frequency in patients with EM. The frequency of HLA-DQw3 (77.4%) increased with high significance compared with normal Caucasian control individuals (41.2%). An even stronger DQw3 association was found in the patient group with postherpetic EM (88.8%; relative risk, 9.41). Interestingly, all patients suffering from frequently recurrent EM were found to have the DQw3 allele (relative risk 44.2). The previously reported association to HLA-B15 was also seen and may be due to a linkage disequilibrium with the HLA-DR4 allele (66.6% in recurrent EM). Our data provide further evidence that classic recurrent EM is related to herpes simplex virus infection and should be regarded as a distinct entity within the enigmatic EM syndrome. DQw3 may serve as a helpful marker for distinguishing this entity from other diseases with EM-like lesions.

Adolescent↗

Clinicopathologic correlation in erythema multiforme and Stevens-Johnson syndrome.

BACKGROUND AND DESIGN: To confirm the recent hypothesis that the spectrum of severe erythema multiforme (EM) is actually composed of two different disorders, we retrospectively studied 38 such cases, particularly in regard to their histopathologic features. Based on photographs and a recent proposal, the cases were classified as EM major when the eruption consisted of typical or raised atypical target lesions located on the extremities and/or the face or as Stevens-Johnson syndrome when the eruption consisted of flat atypical target lesions or purpuric macules that were widespread or distributed on the trunk. The cases were also assessed for causal agent. A biopsy specimen was obtained in each case. Several histologic parameters were analyzed (and scored) without clinical data and correlated to the clinical pattern. These parameters were first studied in a global assessment and then in a detailed evaluation. RESULTS: The global assessment showed two different histological patterns: (1) a predominantly inflammatory pattern characterized by a lichenoid infiltrate and epidermal necrosis that mainly affected the basal layer; and (2) a predominantly necrotic pattern in which major epidermal necrosis and minimal inflammatory infiltration were found. The former pattern was associated with EM major, the latter with Stevens-Johnson syndrome (P < .001) and with drug-related cause (P < .001). The detailed evaluation showed also less epidermal necrosis, and more dermal inflammation, and more exocytosis in EM major. Conversely, there was more epidermal necrosis, less dermal inflammation, and less exocytosis in Stevens-Johnson syndrome. The difference was statistically significant for the inflammation and exocytosis. CONCLUSIONS: This study suggests that the two different symptomatologies in the spectrum of severe EM correlate with two different patterns of histopathologic changes. A prospective multicentered study should be conducted to definitively characterize these entities.

Erythema Multiforme↗

Herpes simplex virus-associated erythema multiforme in prepubertal children.

OBJECTIVE: To examine clinical associations, evolution of the condition, and response to treatment of erythema multiforme (EM) in prepubertal children. DESIGN: A retrospective case series evaluation of children younger than 13 years with EM. SETTING: Ambulatory care university hospital. PATIENTS: Referral patients from pediatricians serving a population of 3.2 million. INTERVENTIONS: Results of treatment of each EM episode with topical acyclovir or oral acyclovir at a dose of 25 mg/kg per day and 6-month prophylaxis with oral acyclovir at a dose of 20 mg/kg per day were evaluated. OUTCOMES: Age at EM onset, preceding illness, and number and duration of episodes during a 3-year period were recorded. RESULTS: Twelve children (7 boys and 5 girls) in whom herpes simplex virus (HSV)-associated EM developed were evaluated. Preceding lesions were herpes labialis in 8 children and herpes facialis in 2 children. Two children had no obvious HSV lesion. The mean age at onset of disease was 8.1 years, and the mean time from the preceding HSV to the onset of skin lesions was 3.9 days (range, 0-11 days). Episodes of EM lasted a mean of 10.6 days. In 9 children, the EM was recurrent, with a mean of 2.6 episodes per year. All 12 children, including those with negative viral cultures for HSV or no HSV history had HSV detected in their target lesions by polymerase chain reaction amplification of DNA obtained from skin biopsy specimens. Six of 12 children were treated with oral acyclovir at a dose of 25 mg/kg per day for 1 or more individual episodes, without reduction in the episode. Three children underwent 6-month prophylaxis with oral acyclovir at a dose of 20 mg/kg per day and remained disease free during treatment. After discontinuation of the prophylactic treatment with acyclovir, 1 child relapsed at 4 months. The other 2 children had no further episodes during a 3-year period. CONCLUSIONS: The HSV-associated EM is a recurrent disease that can be precipitated by sun exposure and does not progress to Stevens-Johnson syndrome. Childhood HSV-associated EM may be unresponsive to treatment with oral steroids or oral or topical acyclovir. Frequent recurrences of EM may be abrogated by prophylactic treatment with acyclovir.

Acyclovir↗

Conjunctivitis and skin erythema. Outbreak caused by a damaged high-intensity lamp.

Eighty-one members of girls' basketball teams were exposed to ultraviolet light while sitting in the bleachers of a school gymnasium. A mercury high-intensity discharge lamp lighting the bleacher area had a hole in its outer envelope that allowed the emission of ultraviolet light. Sixty-nine (85%) of the 81 girls were affected; 49 (71%) had symptoms of conjunctivitis and 63 (91%) had symptoms of erythema.

Accidents, Home↗

Erythema and conjunctivitis. Outbreak caused by inadvertent exposure to ultraviolet light.

During August to November 1977, fifty-eight episodes of erythema or conjunctivitis or both occurred among nursing-service employees at a large state hospital. Epidemiologic investigation implicated an ultraviolet-light-producing germicidal bulb in a nursing station's desk lamp as the causative agent. Removal of the lamp from the nursing station controlled the outbreak. Analysis of the spectral irradiance from the bulb suggested that the National Institute of Occupational Safety and Health exposure standards were exceeded in five to 20 minutes, depending on the individual's distance from the lamp.

Antisepsis↗

Academy rash. A probable epidemic of erythema infectiosum ('fifth disease').

Over a few weeks, 69 children (13%) enrolled in a private school manifested an erythematous and migratory rash, most commonly on the extremities, along with fever or other systemic or respiratory symptoms. Fever lasted for a mean of four days, and rash, 11 days. Cases appeared in every grade from kindergarten through ninth, and the incidence peaked in the fifth through seventh grades. More boarding students were affected than day students. Secondary cases in households of affected children and among adults from the school were noted. Eighty-eight attempts at virus isolation were unsuccessful. The illnesses were consistent with erythema infectiosum, a disease of unknown etiology, which has occurred in epidemic form in schools and also in sporadic cases.

Adolescent↗

Erythema nodosum: etiologic and predictive factors in a defined population.

OBJECTIVE: To examine the frequency and features of erythema nodosum (EN), establish disease associations, and identify the optimal set of predictors for the occurrence of secondary EN. METHODS: We performed a retrospective study of an unselected population of patients 14 years and older with biopsy-proven EN diagnosed at a referral hospital between 1988 and 1997. Patients were classified as having either idiopathic EN or EN secondary to other diseases if the skin nodules occurred in the context of a well-defined disease, or if there was a precipitating event in close temporal proximity to the onset of EN. RESULTS: One hundred six patients (82 women) were diagnosed as having biopsy-proven EN. At the time of diagnosis, no precipitating events or underlying diseases were identified in 36.8% of patients. Sarcoidosis and nonstreptococcal upper respiratory tract infections (URI) were the most common conditions associated with secondary EN. Only 1 of 35 patients with an initial diagnosis of idiopathic EN and a followup of at least 1 year was finally diagnosed as having secondary EN. The best predictive model of secondary EN included an abnormal results on a chest radiograph, a previous history of nonstreptococcal URI, and a significant change in antistreptolysin O (ASO) titer in 2 consecutive determinations performed in a 2-4-week interval. Also, the presence of peripheral synovitis, a positive tuberculin skin test, and a history of diarrhea suggested the presence of secondary EN. This model showed high sensitivity and specificity. CONCLUSION: Idiopathic EN is common. A basic procedure including careful medical history-taking, a physical examination for peripheral synovitis, 2 consecutive ASO determinations, a tuberculin skin test, and chest radiography may be sufficient to diagnose EN.

Biopsy↗

Occurrence of erythema chronicum migrans and Lyme disease among children in two noncontiguous Connecticut counties.

In May-November 1977 erythema chronicum migrans of Lyme arthritis occurred in at least 12 children in New London County, Connecticut, east of the original 3-town epidemic focus. The attack rate (0.15 cases per 1,000 persons under 18) was considerably lower than within the focus itself but similar to the rate in towns west of the focus. No cases were identified in Litchfield County in northwest Connecticut. Observed rates of Lyme disease varied markedly within Connecticut in 1977.

Adolescent↗