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Acrodermatitis enteropathica-like eruption during treatment of maple syrup urine disease: report of one case.

We describe a Chinese infant with maple syrup urine disease (MSUD) who had acrodermatitis enteropathica-like skin manifestations, edema, anemia, and diarrhea due to inadequate intake of branched-chain amino acids (BCAAs). A BCAA supplementation resulted in resolution of the eruption. This observation suggested the importance of monitoring plasma amino acids' levels for infants with metabolic disease on special formula.

Acrodermatitis↗

Serum concentrations of zinc and copper in bull terriers with lethal acrodermatitis and tail-chasing behavior.

OBJECTIVE: To establish similarities or differences in tissue concentrations of zinc, copper, and iron in Bull Terriers with lethal acrodermatitis (LAD) and tail-chasing behavior (TCB) and to confirm the suspicion that copper is involved in the etiopathogenesis of LAD. SAMPLES: Serum samples from 29 Bull Terriers (9 control dogs, 6 dogs with LAD, 14 dogs with TCB), and liver and kidney specimens from 2 dogs and 1 and 4 dogs with LAD or TCB, respectively. PROCEDURE: Serum, liver, and kidney mineral (zinc, copper, and iron) concentrations in Bull Terriers with LAD or TCB and in a group of control dogs were analyzed, using flame atomic absorption after wet ashing technique. RESULTS: Serum zinc and copper concentrations were lower (P < 0.05) in dogs with LAD, compared with values for control dogs and dogs with TCB. Liver zinc and copper concentrations were similar to serum values. Kidney zinc and copper concentrations were similar among the 3 groups. Serum, liver, and kidney iron concentrations had a wide range of variability within all 3 groups. CONCLUSION: Copper deficiency is associated with LAD. The primary cause of LAD may be copper deficiency, with zinc involved secondarily, or combined zinc and copper deficiencies. The role of ion deficiency in TCB was not clarified. CLINICAL RELEVANCE: Serum zinc and copper concentrations should be determined when LAD is suspected.

Acrodermatitis↗

Zinc therapy in acrodermatitis enteropathica.

An infant is described with acrodermatitis enteropathica, who initially presented with severe and intractable watery diarrhea. Diagnosis was established at the age of eleven weeks. Serum-zinc concentrations were extremely low and urinary zinc excretion was diminished. Eleven days after oral zinc supplement (100 mg elemental zinc per day), the skin lesions had healed. The high therapeutic doses of zinc required for healing are suggestive that zinc malabsorption is an important pathogenetical factor of this disease.

Acrodermatitis↗

Papular acrodermatitis of childhood related to poxvirus and parvovirus B19 infection.

Papular acrodermatitis of childhood (Gianotti-Crosti syndrome) is considered an unspecific cutaneous pattern related to an increasing number of infectious diseases. We report two cases of Gianotti-Crosti syndrome, one of which occurred in the setting of parvovirus B19 primary infection and the other followed poxvirus infection. Parvovirus B19 and poxvirus may represent new causative agents of Gianotti-Crosti syndrome.

Acrodermatitis↗

Acrodermatitis enteropathica. Successful zinc therapy.

Acrodermatitis enteropathica (AE) is a familial syndrome with a high incidence of morbidity and mortality. The pathogenesis is unknown. Diiodohydroxyquin and related preparations have been the mainstay of treatment for the past 20 years. Recent evidence indicates that zinc therapy given orally may be the most effective treatment. We will describe the case of a 21-year-old woman who had had AE since she was 3 months old. Successful results were obtained with zinc therapy alone.

Acrodermatitis↗

Detection of Borrelia afzelii, Borrelia burgdorferi sensu stricto, Borrelia garinii and group VS116 by PCR in skin biopsies of patients with erythema migrans and acrodermatitis chronica atrophicans.

OBJECTIVE: To evaluate the diagnostic performance of two polymerase chain reaction (PCR) procedures using skin biopsies of 20 erythema migrans (EM) and 24 acrodermatitis chronica atrophicans (ACA) patients. METHODS: One assay amplified a fragment of the outer surface protein (Osp) A gene. The second method amplified the spacer region between the 5S and 23S rRNA genes; hybridization of this fragment allowed identification of Borrelia burgdorferi sensu lato species. RESULTS: Among EM patients, both assays detected Borrelia DNA in 15 samples. Among ACA patients, the ospA PCR detected 15 positives and 10 samples were positive by 5S-23S PCR. In 19 samples one species was detected, 15 skin biopsies contained Borrelia afzelii, and Borrelia garinii was found in two patients. Group VS116 was detected in two EM patients, and therefore this group has pathogenic potential. Mixed infections of B. afzelii and B. garinii, group VS116 or B. burgdorferi sensu stricto were found in three EM and three ACA patients. CONCLUSIONS: Diagnosis of EM and ACA by PCR is useful and knowledge of the presence of species may be used to predict the course of disease or the need for further antibiotics.

Journal Article↗

Zinc dependency as a cause of chronic diarrhea in variant acrodermatitis enteropathica.

Two siblings with chronic diarrhea, growth failure, mood changes, and occasional cheilosis responded repeatedly to treatment with either pharmacologic doses of zinc or pancreatin (Viokase), and their symptoms were exacerbated after withdrawal of therapy. Pancreatic exocrine deficiency was ruled out in both cases. Proteolytic activity was 20% of normal in one infant tested. Plasma zinc concentration was normal. Plasma picolinic acid concentration was low in these two patients and in one previously reported patient (mean 3.6, normal 12.4 +/- 3.3 mumoles/liter). This is a characteristic shared with acrodermatitis enteropathica. The response to Viokase may be due to its content of picolinic acid and/or zinc or the correction of a deficiency of carboxypeptidase, a zinc-requiring enzyme.

Child, Preschool↗