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[Diarrhea, erythema nodosum, arthralgia].

Following antibiotic treatment of febrile tonsillitis, a 20-year old man developed watery diarrhea during military service. He was admitted to the infirmary by the medical officer. During the last year the patient had traveled to Spain. The history of recent food intake was not remarkable. The clinical investigation revealed a slightly tender liver. A markedly elevated ESR, a consecutively developing erythema nodosum on both lower legs and arthralgias opened a broad spectrum of differential diagnoses that are discussed here. Stool cultures grew salmonella typhimurium. The clinical picture, the treatment and possible complications of this salmonellosis are discussed.

Adult↗

Primary and secondary erythema migrans in central Wisconsin.

BACKGROUND AND DESIGN: We report a series of 28 consecutive patients with erythema migrans (EM) who underwent skin biopsies for culture of Borrelia burgdorferi. Culture results, Lyme serologic findings, and clinical features in patients with only primary EM are compared with those in patients with secondary EM. RESULTS: Culture preparations of skin specimens obtained from six of 12 patients with only primary EM, and from 14 of 16 patients with secondary EM were positive for B burgdorferi. Seven patients with only primary EM were initially seronegative, and only one patient had an annular lesion. A central crusted punctum was present in five of six primary EM lesions that were not culture positive, but in none of nine culture-positive primary EM lesions. Patients with secondary EM were all seropositive and had less cutaneous and more constitutional symptoms than patients with only primary EM. Three patients with secondary EM had abnormal liver enzyme profiles, one patient had complete heart block, and one patient had myocarditis simulating infarction. Less than one third of all patients recalled a tick bite. An isomorphic nonresponse was seen in skin previously involved with secondary EM in one patient who had a drug exanthem from amoxicillin. CONCLUSIONS: Borrelia burgdorferi can be reliably cultured from skin biopsy specimens of secondary EM. Culture preparation aids definitive diagnosis of Lyme disease in patients with only primary EM who often lack constitutional symptoms, have nondiagnostic lesions, or are seronegative.

Administration, Oral↗

Enalapril-associated erythema and vasculitis.

Our pharmacologic armamentarium is expanding at a tremendous pace, and requires that we as clinicians maintain a certain level of knowledge of the drugs that we use, as well as their metabolism, and how they may function, both positively and negatively, in a given patient. Certain drugs, such as the angiotensin-converting enzyme inhibitors, interfere with the natural deactivation of certain vasoactive substances normally present in the body. Since many of these systems are functionally interrelated (eg, angiotensin-converting enzyme and kininase II), we may see cutaneous side effects when these drugs are administered. Enalapril is an angiotensin-converting enzyme inhibitor that promoted a cutaneous eruption in a patient with renal failure secondary to diabetes. We suspect that our patient's vasculitis and maculopapular erythema is included in a large number of polymorphic eruptions that can be seen with angiotensin-converting enzyme inhibitors, and review the operative pathogenetic mechanism. Many of these side effects are dose related, and can be controlled or eliminated by lowering the dosage of angiotensin-converting enzyme inhibitor, depending upon the clinical circumstances.

Adult↗

[Darier's erythema annulare centrifugum of neonatal onset with a 15 years' follow-up. Efficacy of interferon and role of cytokines].

INTRODUCTION: Darier's erythema annulare centrifugum (EAC) is a disease of unknown etiology. Some observations of EAC in infancy suggest a better prognosis for early EA. The observations presented herein gives different information regarding prognosis and pathogenesis. OBSERVATION: A child presents with typical lesions of EAC from early infancy up to the age of 15 years. Laboratory data are normal. Similar lesions were locally reproduced by intradermal injection by candidine but antifungal drugs were unsuccessful. Many antiinflammatory treatments failed to improve the disease. Because of the improvement during hyperthermic episodes, a treatment by subcutaneous interferon alpha (2 millions U 3 times a week) was given for six months with a dramatic success (with seric increase of TNF alpha and IL2) DISCUSSION: The long term duration of EAC suggests that EA of infancy may represent the early beginning of Darier's EAC. The efficacy of interferon suggests that cytokines are involved in the pathogenesis of EAC.

Cytokines↗

Study on clinical picture of erythema migrans.

Clinical picture of erythema migrans (EM) was evaluated in 83 patients from north-eastern Poland. Patients were in mean age of 42,8. Most of patients (71%) were women. Tick bite remembered 64% patients. Mean incubation period of EM was 33 days. The mean diameter of the skin lesions was 14.8 cm. They persisted for 2-270 (mean-54) days. Lesions were located mostly on the skin of lower extremities. The largest lesions were found on trunk and the smallest on lower extremities. EM of diameter exceeding 20 cm were observed in patients with longer history of the disease. Specific antibodies were found in 53% of cases and were present more frequently (78%) in patients with shorter incubation period (< 30 days).

Adolescent↗

Erythema multiforme.

The diagnosis of erythema multiforme was established on the basis of physical examination, biopsy specimen evaluation, and the patient's medication history. Use of a triamcinolone mouthrinse and discontinuation of Triphasil-28 contraceptive led to resolution of the lesions.

Adult↗

Erythema elevatum diutinum. Response to dapsone.

A patient with a 20-year history is presented as a classic example of erythema elevatum diutinum, with a dramatic response to dapsone therapy, and with interesting features of improvement during pregnancies and occurrence of giant cell tumors of soft tissue, neither of these latter features having been previously reported.

Adult↗

["Circumscribed defibrillator erythema": a differential diagnostic and therapeutic problem in differential occult defibrillator infection diagnosis].

We report about three out of 452 patients (pts) (< 1 %) with circumscribed erythema (E) after implantation of an automatic cardioverter defibrillator (ICD). ICD-E occurred in two pts after pulse generator replacements and in one pt after initial ICD implantation. There were no clinical signs of inflammation and laboratory and technical finding were completely normal. Allergic reactions could be ruled out in all pts. Local or systemic drug treatment did not influence the ICD-E. During a follow-up of 28, 10 or 9 months, the ICD-E is still present in two patients and much improved in one pt without any treatment. We did not observe an infection in any of the pts. Our data show that the ICD-E occur rarely after ICD implantation and no special treatment is necessary if an infection or allergic reaction has been excluded. Nevertheless, it is sometimes difficult to exclude in those pts an atypical infection.

Adult↗

[Necrolytic migrating erythema without glucagonoma].

INTRODUCTION: The glucagonoma syndrome is an uncommon but well-known entity associating erythema necroticans migrans (ENM) with glucagonoma. CASE REPORT: A 43-year-old man with a past history of alcoholic cirrhosis and ascitis was hospitalized for skin disorders which had developed over the past 4 months. Centrifugal erythematous skin lesions were observed, some with non-turgid bullae and marginal desquamation, others with an erosive center. Lesions first appeared on the hands then diffused widely without involving the periorifical areas, folds and lower limbs. Laboratory results revealed an anemia, hypovitaminosis K, cholestatic liver failure, a beta-gamma block and low zinc levels. Histology study of the skin biopsy demonstrated a << Neapolitan trench >> image suggestive of ENM. A paraneoplasic syndrome and pancreas tumor were not found. Despite supplementation with zinc, amino acids and vitamins, the patient died from his liver disease. DISCUSSION: There have been 4 cases of ENM reported in the literature, all in cirrhosis patients, two of which had low zinc levels. Our case is thus the third with cirrhosis and low zinc. Bazex's syndrome, acrodermatitis enteropathica, annular chronic lupus erythematosis and annular superficial pemphigus were eliminated as possible diagnoses. The failure of zinc and amino acid supplementation would favor the secondary nature of the zinc deficiency and the predominant role of cirrhosis in this skin disease. The possible role of essential fatty acids in ENM is raised.

Adult↗

Persistent erythema and pruritus, with a confluent histiocytic skin infiltrate, following the use of a hydroxyethylstarch plasma expander.

A 40-year-old woman developed a persistent pruritus with erythema of the head and upper trunk, which started within 2 weeks of a hydroxyethylstarch (HES) infusion and was still present 2 years later. Histological examination demonstrated a striking dermal infiltrate of KP1-positive foamy macrophages which had electron-lucent vacuoles. The timing of the onset, the body site distribution, the exclusion of other possibilities and an appropriate history of high dosage HES infusion, suggested that this was the cause of the eruption. Staining with periodic acid-Schiff (PAS) surprisingly was negative but we could not stain HES in vitro with this histochemical stain, and we postulate that the unique extent of the infiltrate and the negative PAS stain in our patient may indicate an impaired ability to degrade this chemical. This may be an important factor in the poorly understood pathogenesis of persistent pruritus induced by HES.

Adult↗

Successful treatment of a patient with octreotide-resistant necrolytic migratory erythema.

We report a patient with the glucagonoma syndrome and octreotide-resistant necrolytic migratory erythema (NME). The NME responded on two occasions to an intravenous infusion of essential fatty acids (EFA) and amino acids (AA). A deficit of serum EFA prior to treatment was corrected following the infusion, whilst plasma AA were low before and after treatment. These findings indicate that NME, in the glucagonoma syndrome, may respond to correction of the EFA deficit, and that NME may be a disease of EFA deficiency.

Amino Acids↗

Unusual erythema elevatum diutinum with fibrohistiocytic proliferation.

A 78-year-old woman with a history of symmetrical erythematous plaques on the arms, and a monoclonal gammopathy, developed a strange striped reticulate papular dermatosis with central atrophy. Histological examination was compatible with a very late stage of erythema elevatum diutinum (EED), showing a fibrohistiocytic proliferation with areas of granulation tissue. This fibrosis may result from the chronic dermal injury of leucocytoclastic vasculitis and is sometimes the predominant histology of EED. Investigations for underlying haematological anomalies, such as paraproteinaemia and infection with human immunodeficiency virus, must be performed. Dapsone is ineffective once the fibrous nodules have appeared.

Aged↗

Erythema toxicum neonatorum.

Erythema toxicum neonatorum is a common finding of unknown etiology affecting healthy newborn children, particularly those born at term. It is seen in the first days of life, but rarely at birth. Infants with it often have sterile papulopustules containing a predominance of eosinophils. It is important to distinguish from other cutaneous eruptions of neonates, some of which are serious.

Age of Onset↗

[Usefulness of polymerase chain reaction for the diagnosis of Bazin erythema induratum].

BACKGROUND: Erythema induratum of Bazin (BEI), is included in the group of cutaneous granulomatous lobulillar panniculitis. The aethiopathogenic association between EI and tuberculosis can not rely on the clinicohistological features of these panniculitis and M. tuberculosis has never been isolated from BEI lesions. Detection of the mycobacterial DNA by PCR on cutaneous biopsy samples would allow to confirm this association. PATIENTS AND METHODS: Fourteen patients with clinical BEI were chosen retrospectively. Seventeen lesional biopsy samples were obtained, stained with the Kinyoun carbolfuchsin acid-fast technique and haematoxylin and eosin and tested by PCR. A fragment of the IS6110 insertion sequence specific of M. tuberculosis was amplified and confirmed by digestion with Sal I restriction endonuclease. The efficiency of the procedure, the presence of inhibitory substances and the preservation of DNA were checked by PCR of the beta-actin gene. RESULTS: M. tuberculosis DNA was detected in 12 of the 17 samples tested (70.5%) which corresponded to 10 of the 14 patients (71.4%). According to beta-actin PCR results, the rate of extracted DNA was inadequate on four of the five negative biopsies. CONCLUSIONS: The results of these series suggest the probable involvement of M. tuberculosis on the BEI pathogenesis and give support to the usefulness of the PCR in the diagnosis of this pathology concerning the need of specific treatment.

Adult↗

[Facial erythema and pancreatic glucagonoma].

Glucagonoma is a rare pancreatic tumor, that gives a characteristic clinical syndrome. In this report we describe the case of a 27 year old woman whose only clinical manifestations were a facial erythema and a palpable abdominal mass. Surgical removal of the tumor resulted in elimination of the clinical symptoms. Immunohistochemical findings were consistent with pancreatic glucagonoma.

Adult↗

Detection of Mycobacterium tuberculosis DNA in lobular granulomatous panniculitis (erythema induratum-nodular vasculitis).

OBJECTIVE: To determine, using polymerase chain reaction (PCR) amplification, if Mycobacterium tuberculosis complex DNA is present in the skin biopsy specimens of lobular granulomatous panniculitis. DESIGN: A retrospective descriptive study. SETTING: A university-based hospital. PATIENTS: From the 65 patients included in the study, we examined 72 paraffin-embedded skin biopsy specimens with a histologic diagnosis of erythema induratum or nodular vasculitis. The biopsy specimens were from the histopathological archives of the Departments of Dermatology and Pathology of the Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, from 1976 to 1994. Twenty-two biopsy specimens were excluded from the final analysis because we could not amplify the internal control. MAIN OUTCOME MEASURES: Detection of a 123-base pair fragment of the IS6110 insertion sequence specific for M tuberculosis complex. RESULTS: The results of PCR amplification were positive for M tuberculosis complex DNA in 77% of the skin biopsy specimens. No significant difference could be detected with respect to the age of the patients, ulceration of the nodules, reactivity to purified protein derivative, abnormal results of a chest x-ray examination, personal and family history of tuberculosis, and PCR results. The presence and degree of necrosis on histologic examination were significantly higher in the PCR-positive group (P = .04). None of the following variables were associated with PCR results: presence of vasculitis, degree of granulomatous infiltrates, number of giant cells, and presence of well-organized granulomas. CONCLUSIONS: The DNA of M tuberculosis can be detected in a considerable number of skin biopsy specimens of lobular granulomatous panniculitis. None of the clinical and histologic variables evaluated could accurately predict the results of PCR amplification.

Adult↗

Erythema elevatum diutinum in association with coeliac disease.

Erythema elevatum diutinum (EED) has been described in association with several immunological or infectious diseases. We describe a female patient who presented with clinically and histologically typical EED in whom previously undiagnosed coeliac disease was found. Appearance of EED lesions was preceded by widespread joint pains. In extensive laboratory tests, the only abnormal findings were an elevated erythrocyte sedimentation rate (ESR) and decreased haemoglobin and folic acid levels. Later, IgA and IgG type antireticulin and antigliadin antibodies were detected. Serum total IgA was elevated but no paraproteinaemia was found. In lesional skin, granular deposits of IgA and C3 were seen at the dermo-epidermal junction. A duodenal biopsy revealed total villous atrophy. Dapsone treatment was partly effective but complete healing of the EED lesions was achieved only after the introduction of a strict gluten-free diet. The patient has now remained symptom-free on the diet for 1.5 years.

Celiac Disease↗

Erythema multiforme-like manifestations and arthritis in a 3-year-old child with leukocytoclastic vasculitis.

A 3-year-old boy with erythema multiforme-like manifestations and severe articular involvement is reported. Because of the unusual onset of the cutaneous lesion a skin biopsy was performed, revealing the typical features of a leukocytoclastic vasculitis. A direct immunofluorescent study revealed C3 and IgM deposition within the wall and around the small vessels. The clinical and immunopathological findings in this patient were similar to those reported for the acute hemorrhagic edema of infancy (AHE) described in children younger than 2 years of age. The present case supports the hypothesis that AHE is a distinctive leukocytoclastic vasculitis of childhood, irrespective of the age at onset.

Arthritis↗