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Acrodermatitis enteropathica-like eruption associated with combined nutritional deficiency.

We present here a case of acrodermatitis enteropathica-like eruption associated with essential free fatty acid and protein deficiencies as well as borderline zinc deficiency that occurred after Whipple's operation in a 31-yr-old woman. Her eruptions were improved not by zinc supplements alone, but her condition was improved by total parenteral nutrition including amino acids, albumin, lipid and zinc. Although we could not exactly decide which of the nutrients contributed the most to her manifestations, we inferred that all three elements in concert caused her dermatoses. This case shows that even though the patient's skin manifestations and laboratory results are suggestive of acrodermatitis enteropathica, the physicians should keep in mind the possibility that this disease can be associated with other nutritional deficiencies such as free fatty acid or protein deficiency.

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A case of chronic pancreatitis associated with liver infarction and acrodermatitis enteropathica.

Liver infarction and acrodermatitis enteropathica are rare complications of chronic pancreatitis. This report shows the case of a 56-year-old man who developed liver infarction due to portal vein thrombosis from chronic pancreatitis and acrodermatitis enteropathica during the course of his treatment. The rare combination of these complications in a patient with chronic pancreatitis has never previously been reported in the literature.

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[Acrodermatitis enteropathica: a case report].

Acrodermatitis enteropathica is a rare disease which is inherited autosomally and recessively. The reason for the illness is a smaller absorption of zinc. The onset of the illness is deceptive and clinical manifestations refers to the digestive system, skin, hair and nails. The report describes a case of a 6 month girl affected acrodermatitis enteropathica.

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[Zinc therapy in acrodermatitis enteropathica].

Report of two patients with acrodermatitis enteropathica, one of them 20 years old and with very severe symptoms of the disease. Oral therapy with zinc resulted in complete cure within two months. The theory about chelating of dietary zinc in patients with acrodermatitis enteropathica is discussed.

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Pemphigus vegetans presenting as acrodermatitis continua suppurativa.

The authors present a 51-year-old patient with clinical signs of acrodermatitis continua suppurativa of the toes. Since histological findings failed to unambiguously support the diagnosis of acrodermatitis continua suppurativa, immunohistology was performed. The results showed intercellular epidermal fluorescence. The subsequent clinical picture (vegetating formation), the repeated histological examination (suprabasal cleft and eosinophilia), as well as the data of immunohistological findings supported the diagnosis of pemphigus vegetans Hallopeau. The patient was treated successfully with a combination of cyclosporine-A and low-dose steroid therapy. The case is of special interest, because there is only a single publication about phalangeal and nail involvement in Hallopeau's type pemphigus vegetans in the dermatological literature according to our knowledge.

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Lethal acrodermatitis in bull terriers.

A lethal syndrome characterized clinically by growth retardation, progressive acrodermatitis, chronic pyoderma and paronychia, diarrhea, pneumonia, and abnormal behavior was observed in 17 related Bull Terrier pups. Median survival time was 7 months. Laboratory evaluation revealed non-degenerative neutrophilia, consistently low activities of serum alkaline phosphatase and alanine transaminase, and frequently, hypercholesterolemia. Lymphocyte blastogenic responses were decreased and there was dysgammaglobulinemia in pups in which quantitative studies of immunoglobulins were made. The mean of plasma zinc concentrations in 5 affected pups was significantly lower than the mean of age- and breed-matched controls. Pathologic findings included parakeratosis, hyperkeratosis, and superficial bacterial infections of the skin. There was severe reduction of lymphocytes in T-lymphocyte areas of lymphoid tissue. Bronchopneumonia and dilatation of the cerebral ventricles were found in most affected pups. Family studies indicated that the syndrome is inherited as an autosomal recessive trait. In spite of its similarities to lethal trait A46 in Black Pied Danish cattle and acrodermatitis enteropathica in man, oral or parenteral treatment with zinc failed to ameliorate the clinical signs of the syndrome.

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Hereditary zinc deficiency (Adema disease) in cattle, an animal parallel to acrodermatitis enteropathica.

Adema disease and acrodermatitis enteropathica, two parallel syndromes in calves of Friesian descent and in man, are described. Both are congenital zinc deficiency disorders with a lethal course if left untreated. Complete recovery follows oral zinc therapy. Symptoms and findings are set out in Table I. Diseased calves may serve as animal models for further studies on acrodermatitis enteropathica and the biological role of zinc.

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[Acrodermatitis enteropathica; zinc sulfate therapy].

We present the case of two siblings with acrodermatitis enteropathica. Zinc levels were low. Oral administration of 100 mg of zinc sulfate allowed total regression of all signs. When zinc therapy was interrupted, the manifestations reappeared and disappeared again when treatment was resumed. A defect of genetic origin in digestive zinc absorption is suggested. Mohnahan's advocated zinc treatment of acrodermatitis enteropathica in 1973; it is a non-toxic treatment, which is always active and be considered as a diagnostic test.

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[Acrodermatitis enteropathica: disparity between clinical features and zinc blood levels].

A new case of acrodermatitis enteropathica in a 2.5 month old infant is presented. Oral administration of zinc sulfate (10 mg/kg/day) improved in a few days clinical and analytical pattern. In 1.5 months treatment was forsaken, reappearing after 22 days digestive and cutaneous symptoms. Serum zinc was then rather high (175 mcg/dl). Disparity between serum zinc and clinical features, suggest that in acrodermatitis enteropathica there is a lack in transport, not only in the enterocyte, but also in other cells of the body. Discerning dose and treatment duration must be assessed by clinical response rather than zinc serum level.

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Acquired acrodermatitis enteropathica.

Acrodermatitis enteropathica has an hereditary, infantile form and an acquired form which usually develops secondary to total parenteral nutrition. Both forms are caused by a zinc deficiency. The use of a reduced serum alkaline phosphatase level as a marker for acrodermatitis enteropathica is presented in addition to a case of the acquired form of the disease.

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[Acrodermatitis enteropathica: a treatable disease today].

Acrodermatitis enteropathica was diagnosed in a girl at the age of 13 months. The clinical symptoms of the patient conformed to the known features of Acrodermatitis enteropathica. The gstrointestinal symptoms were not present. Previous to therapy the serum-zinc levels of the patient were markedly decreased. Following substitution with Zincaspartat the clinical condition improved and the serum-zinc levels returned to normal. The aetiology, pathogenesis, clinical course and the therapy of the disease are reported.

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Treatment of acrodermatitis enteropathica with zinc sulfate.

Control of a case of acrodermatitis enteropathica was achieved initially after three months of treatment with diiodohydroxyquin (Diodoquin). Reexacerbation of acrodermatitis enteropathica was noted two months after diiodohydroxyquin was discontinued. Treatment with zinc sulfate was substituted and resulted in dramatic and complete resolution of disease within 48 hours.

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[Adult papular acrodermatitis (Gianotti's disease). Report of 3 cases].

The authors report three cases of adult papular acrodermatitis with circulating HBs antigen. The eruption was followed by a benign icteric hepatitis which lasted from 30 to 45 days. In two cases, HBs antigen disappeared in a one month period, in one case the antigen has been present for more than three months. Direct immunofluorescence staining exhibits c3 deposits in the vessels of the dermal lesions, without any deposition of immunoglobulins or fibrinogen. We were unable to demonstrate the presence of HBs and " e " antigens in the skin lesions (using FITC conjugated specific antiserums). Serum protein concentrations of complement components C1q, C4,C3, C3PA were normal as measured by radial immunodiffusion. The percentage of circulating B and T cells was normal, as essayed by E-RFC, EAC-RFC and sIg. Thus, adult papular acrodermatitis, as well as the infantile form, does represent a sign of invasion of a benign viral hepatitis.

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Acrodermatitis chronica atrophicans in an 11-year-old girl.

We report a case of acrodermatitis chronica atrophicans in an 11-year-old girl living in an area endemic for Lyme borreliosis. The diagnosis was first made on the basis of clinical, histopathological and serological findings. Moreover, Borrelia burgdorferi-specific DNA was amplified from lesional skin by polymerase chain reaction. Intravenous treatment with ceftriaxone (2 g once daily) for 2 weeks was initiated. The skin changes clearly responded to the therapy, and Borrelia burgdorferi-specific gene segments were no longer detectable by polymerase chain reaction. This is the first report of molecular-proven acrodermatitis chronica atrophicans in childhood. The occurrence of this late skin manifestation of Lyme borreliosis in children is reviewed.

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Long-term prognosis in patients treated for erythema chronicum migrans and acrodermatitis chronica atrophicans.

OBJECTIVE: To determine whether Lyme borreliosis persisted or had recurred in patients treated for erythema chronicum migrans and acrodermatitis chronica atrophicans. DESIGN: Retrospective follow-up study. Mean time between treatment and follow-up study was 8.8 years (SD, 66.6 years). SETTING: Department of dermatology. PATIENTS: Patients (N = 52) treated from July 1964 to October 1992 for erythema chronicum migrans (ECM; n = 44) or acrodermatitis chronica atrophicans (ACA; n = 8). Fifty-two of the 56 successfully contacted and registered patients agreed to participate, for a response rate of 93%. MAIN OUTCOME MEASURES: Signs and symptoms of Lyme borreliosis; serum antibodies to Borrelia burgdorferi. RESULTS: The interval from the time of diagnosis to study entry was 0.8 to 28.7 years (mean, 8.2 years). No symptoms or signs of active Lyme borreliosis were observed in the 52 patients. Antibodies to B burgdorferi were found in the ECM group in 1 of the 23 patients who received a recommended treatment and 2 of the 21 patients who received other treatments; antibodies were found in the ACA group in all 5 adequately treated patients who received a recommended treatment and in 1 of the 3 patients who received other treatments. CONCLUSIONS: There was no association between serologic results and type of treatment or between serologic results and complaints or symptoms at the time of the study in either of the patient groups. The prognosis in most patients with Lyme borreliosis is excellent.

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Treatment of acrodermatitis enteropathica with zinc sulphate. Rerport of 3 cases.

The therapeutic effect of orally administered zinc in 3 patients affected with Acrodermatitis enteropathica is reported. Single daily doses of zinc resulted in rapid improvement of the general condition of these patients and in clinical remission within a week. Serum, urine and hair zinc levels as well as alkaline phosphatase, which were very low before treatment, returned to normal after therapy. This brief report confirms the efficacy of oral zinc in the treatment of Acrodermatitis enteropathica.

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