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PubMed2003

Immunoglobulin levels in Bull terriers suffering from lethal acrodermatitis.

An enzyme-linked immunosorbent assay, developed by one the authors (Huang, 1993) was used to measure immunoglobulin levels in previously stored plasma samples from eight dogs suffering from lethal acrodermatitis (LAD) and 10 normal Bull terriers. The aim of the study was to document plasma immunoglobulin levels and to determine if dogs suffering from LAD had low IgA levels. The results showed that dogs suffering from LAD had normal IgG and IgM levels but significantly lower IgA levels than the control group. This finding may in part explain the frequent occurrence of microbial infections recorded in dogs suffering from LAD.

Acrodermatitis↗
PubMed2006

Identification of a unique splice site variant in SLC39A4 in bovine hereditary zinc deficiency, lethal trait A46: An animal model of acrodermatitis enteropathica.

Lethal trait A46, also known as bovine hereditary zinc deficiency, Adema disease, and hereditary parakeratosis, is an autosomal recessive disorder first described in 1964, with a clinical presentation similar to that of acrodermatitis enteropathica (AE) in humans. The molecular basis of the defect has not been previously identified. Recently, the basic defect in AE was found to lie in SLC39A4. We report the characterization of the bovine ortholog of SLC39A4 and identification of a unique splice site variant within this gene in affected animals. The mutation leads to exon skipping, leaving the coding region in frame. The gene product is predicted to lack two critical motifs, which lie in adjacent transmembrane domains implicated in the formation of a pore responsible for the transport of zinc. While further functional studies are warranted, this unique variant is likely to be responsible for the impaired zinc absorption in this disease.

Acrodermatitis↗
PubMed1976

An epidemic of infantile papular acrodermatitis (Gianotti's disease) in Japan associated with hepatitis-B surface antigen subtype ayw.

An epidemic of infantile papular acrodermatitis (I.P.A.) (Gianotti's disease) occurred in Matsuyama City, in south-east Japan in 1974-75. Patients ages ranged from less than one year to eight years. Hepatitis-B surface antigen (HBsAg) was detected by an immune adherence haemagglutination method in the serum samples of 48 of the 54 patients tested. HBsAg subtypes were determined by a haemagglutination-inhibition method. ayw antigens were identified in 42 patients and adr antigens in 3; it was not possible to determine subtypes in the remaining 3 patients because antigen titres were too low. Since subtype ayw and I.P.A. are extremely rare in Japan, the association of the disease with HBsAg subtype ayw is regarded as being most significant.

Acrodermatitis↗
PubMed1986

Clinical manifestations of acrodermatitis chronica atrophicans in 50 Swedish patients.

A study was made of 50 consecutive patients with untreated acrodermatitis chronica atrophicans (ACA). In all patients elevated anti-spirochetal antibody titers were found at indirect immunofluorescence and enzyme-linked immunosorbent assays, and histologically biopsies exhibited a dermal lymphocytic infiltrate with a moderate to rich admixture of plasma cells and telangiectases. Nine patients had a history consistent with spontaneously healing erythema chronicum migrans Afzelius (ECMA) on the extremity on which, after 0.5-8 years, ACA lesions developed. Eight patients had a history indicating previous cranial nerve involvement and nine had had periods of severe pains in the cervical or lumbar region. Two patients had developed ECMA, facial palsy and ACA in chronological order. In 15 patients radiographic abnormalities of joints and/or bone tissue were found. Besides ACA lesions, lichen sclerosus et atrophicus- or scleroderma-like lesions were found in six patients. The inflammatory ACA lesions were sometimes discrete and had been overlooked. Joint deformities, sclerotic lesions, diffuse edema or pain were the cardinal symptoms in some patients. The findings indicate that clinical recognition of ACA may be difficult and that a combination of clinical, histopathologic and serologic findings may be necessary to establish the diagnosis. The results are consistent with the concept that ACA is a late manifestation of infection by the same spirochete as causes ECMA and Bannwarth's syndrome.

Acrodermatitis↗
PubMed1999

Joint and bone involvement in Dutch patients with Lyme borreliosis presenting with acrodermatitis chronica atrophicans.

We report on the radiological abnormalities of bones and joints in Dutch patients suffering from Lyme borreliosis presenting with acrodermatitis chronica atrophicans (A.C.A.). In a highly endemic area of the Netherlands rheumatic complaints were mentioned by 26 out of 60 patients suffering from a late stage of Lyme disease. Radiological findings in our group of patients were subluxation of the toe joint and periostitis of the bones of the lower limb.

Acrodermatitis↗
PubMed2001

Normal proliferative responses of peripheral blood mononuclear cells to streptococcal preparation OK-432 in patients with pustulosis palmaris et plantaris constitute a distinct feature from the reduced responses observed in those with psoriasis vulgaris, pustular psoriasis, and acrodermatitis continua of Hallopeau.

It was previously reported that peripheral blood mononuclear cells (PBMC) from the patients with psoriasis vulgaris (PV) showed a reduced proliferative response in vitro to the stimulation of a lyophilized preparation of penicillin-treated low virulence Su-strain of Streptococcus pyogenes group 3, OK-432. In this study, at first it was examined whether OK-432 acts as a superantigen. By analyzing the usage of Vbeta T-cell receptor (TCR) of proliferating T cells stimulated with OK-432, it was found that OK-432 stimulated preferentially Vbeta2 TCR-bearing T cells. Next, to find differences in in vitro responses of PBMC among various types of sterile pustular dermatoses such as pustulosis palmaris et plantaris (PPP), acrodermatitis continua of Hallopeau (AC), and generalized pustular psoriasis (GPP), the proliferative responses of PBMC obtained from these patients under the stimulation of OK-432 were compared. When the PBMC was stimulated with interleukin (IL)-2, no significant difference was found in their proliferative responses among those obtained from the patients with these sterile pustular dermatoses, PV or healthy controls. However, like those from PV patients, PBMC from AC and GPP patients showed significantly smaller responses to OK-432 than those from the healthy controls. In contrast, the proliferative responses of PBMC from the patients with PPP to OK-432 was comparable to those from healthy controls. These results, in addition to its unique clinical and histopathological characteristics, suggest that PPP has a different pathogenetic background from that underlying PV, AC, or GPP.

Acrodermatitis↗
PubMed2002

Peripheral neuropathy in acrodermatitis chronica atrophicans - effect of treatment.

Forty-seven patients with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA) and with objective neurological and/or neurophysiological findings were followed up after antibiotic treatment with dermatological, serological, neurological and neurophysiological controls. Despite a good therapeutic effect on ACA lesions, specific antibody values and symptoms of irritative nerve lesions, the objective neurological and neurophysiological findings of nerve deficit remained unchanged. There was no progress of neuropathy findings during the follow-up time. Our interpretation of the results is that the remaining neuropathy signs after treatment of ACA are neurological sequelae and not manifestations of persisting Borrelia infection.

Acrodermatitis↗
PubMed2002

Identification of SLC39A4, a gene involved in acrodermatitis enteropathica.

We have characterized the human gene SLC39A4, which encodes a protein with features characteristic of a ZIP zinc transporter. The chromosomal location and expression of SLC39A4, together with mutational analysis of eight families affected with acrodermatitis enteropathica, suggest that SLC39A4 is centrally involved in the pathogenesis of this condition.

Acrodermatitis↗
PubMed1979

A defect in zinc uptake by jejunal biopsies in acrodermatitis enteropathica.

1. In a system in vitro, 65Zn accumulation by jejunal mucosal biopsies from patients with acrodermatitis enteropathica was found to be markedly reduced compared with controls. 2. We suggest that defective uptake of zinc by enterocytes is the primary abnormality responsible for the zinc deficiency underlying this disorder.

Acrodermatitis↗
PubMed2001

Treatment of acrodermatitis continua of Hallopeau with oral propylthiouracil and methotrexate.

A 71-year-old man with acrodermatitis continua of Hallopeau was treated successfully with a combination of oral propylthiouracil and methotrexate. After 14 weeks, he developed acute pancytopenia, an uncommon idiosyncratic side-effect of propylthiouracil, and presented with a life-threatening methicillin-resistant Staphylococcus aureus pneumonia. This illustrates the potential value and associated risks of propylthiouracil in the management of this difficult condition.

Acrodermatitis↗
PubMedDate not supplied

Acrodermatitis enteropathica: case report and review of the literature.

Acrodermatitis enteropathica (AE) is a rare hereditary disorder caused by impaired absorption of zinc from the gastrointestinal tract. It is characterized by acral and periorificial dermatitis, alopecia, and diarrhea. Symptoms usually begin on weaning from breast or formula feeding. We report a full-term, 21-month-old boy with typical skin lesions and decreased plasma zinc level (12 micro g/dl). The patient was given zinc sulfate 40 mg/day and at the end of 1 month his condition had improved significantly. After reviewing the literature we emphasize the important role of zinc in human metabolism and the difference between AE and acquired zinc deficiencies.

Acrodermatitis↗
PubMed1976

[Successful zinc treatment of a severe case of acrodermatitis enteropathica (author's tteropathica].

A 24-year-old patient treated with hydroxychinoline preparations without cure for acrodermatitis enteropathica since he was nine years old was given zinc oxide orally, at first 50 mg three times, later twice daily. Zinc concentrations in blood plasma, whole blood and sputum were serially determined. At the start of treatment they were significantly below that in four healthy controls. One week after starting zinc therapy the blood level had returned to normal and with it the changes in skin, hair and nails had regressed, as had the emotional disturbances.

Acrodermatitis↗
PubMed1998

Acrodermatitis chronica atrophicans: histopathologic findings and clinical correlations in 111 cases.

We studied 111 consecutive, untreated and serologically confirmed patients with acrodermatitis chronica atrophicans. Emphasis was on the histopathologic patterns of erythematous and fibrous lesions, and on an assay used to correlate histopathologic findings with such clinical features as fibrous nodules, ulnar bands and the pain reaction allodynia. There was a significant correlation between allodynia and signs of marked inflammation, but not between allodynia and neural and perineural cell infiltrates or fibrosis. Moreover, there was no significant correlation between serum IgG titers to Borrelia and the density of inflammatory cell infiltrates or the proportion of plasma cells in tissue. Histopathologic examination did not reveal any important differences between fibrous nodules, ulnar bands and sclerodermatous lesions. The histopathologic pattern is not diagnostic per se, but characteristic enough to alert the experienced pathologist.

Acrodermatitis↗
PubMed2001

Homozygosity mapping places the acrodermatitis enteropathica gene on chromosomal region 8q24.3.

Acrodermatitis enteropathica (AE) is a rare autosomal recessive pediatric disease characterized by dermatitis, diarrhea, alopecia, and growth failure. The disease results from insufficient uptake of zinc by the intestine and can be fatal unless the diet is supplemented with zinc. To map the gene responsible for AE, a genomewide screen was performed on 17 individuals, including 4 affected individuals, in a consanguineous Jordanian family. Three markers-D8S373, D10S212, and D6S1021-had a pattern consistent with tight linkage to a recessive disease: one allele in the affected sibs and multiple alleles in unaffected sibs and parents. Two-point parametric linkage analysis using FASTLINK identified one region, D8S373, with a maximum LOD score >1.5 (1.94 at D8S373: recombination fraction.001). Twelve additional markers flanking D8S373 were used to genotype the extended family, to fine-map the AE gene. All five affected individuals-including one who was not genotyped in the genomewide screen-were found to be homozygous for a common haplotype, spanning approximately 3.5 cM, defined by markers D8S1713 and D8S2334 on chromosomal region 8q24.3. To support these mapping data, seven consanguineous Egyptian families with eight patients with AE were genotyped using these markers, and six patients from five families were found to be homozygous in this region. Multipoint analysis with all consanguineous families, by Mapmaker/Homoz, resulted in a maximum LOD score of 3.89 between D8S1713 and D8S373. Sliding three-point analysis resulted in a maximum LOD score of 5.16 between markers D8S1727 and D8S1744.

Acrodermatitis↗
PubMed2002

A novel member of a zinc transporter family is defective in acrodermatitis enteropathica.

The rare inherited condition acrodermatitis enteropathica (AE) results from a defect in the absorption of dietary zinc. Recently, we used homozygosity mapping in consanguineous Middle Eastern kindreds to localize the AE gene to an approximately 3.5-cM region on 8q24. In this article, we identify a gene, SLC39A4, located in the candidate region and, in patients with AE, document mutations that likely lead to the disease. The gene encodes a histidine-rich protein, which we refer to as "hZIP4," which is a member of a large family of transmembrane proteins, some of which are known to serve as zinc-uptake proteins. We show that Slc39A4 is abundantly expressed in mouse enterocytes and that the protein resides in the apical membrane of these cells. These findings suggest that the hZIP4 transporter is responsible for intestinal absorption of zinc.

Acrodermatitis↗
PubMed2004

Acrodermatitis enteropathica mutations affect transport activity, localization and zinc-responsive trafficking of the mouse ZIP4 zinc transporter.

The Zip4 protein is involved in dietary zinc uptake from the intestinal lumen. The human ZIP4 gene (SLC39A4) was identified because of its association with acrodermatitis enteropathica (AE), a genetic disorder of zinc absorption. To date, several SLC39A4 mutations have been identified in AE patients. To investigate the effects of these mutations on function of the Zip4 transporter, we introduced six AE-associated missense mutations into the orthologous mouse ZIP4 gene for functional expression in cultured cells. All mutations decreased 65Zn uptake activity of mZip4, thereby providing a causal link to AE. The mutants fell into two groups based on their phenotypic effects. Several alleles (G340D, L382P, G384R, G643R) failed to localize on the cell surface at high levels. These defects were attributable to misfolding and/or mislocalization in the secretory pathway. Two other alleles (P200L and G539R) accumulated to high levels in the plasma membrane and had wild-type apparent Km values for 65Zn uptake. However, these mutations decreased the Vmax of uptake to approximately 30% of wild-type. We showed previously that wild-type mZip4 is regulated post-translationally in response to zinc status. In zinc-replete cells, mZip4 is found largely in intracellular compartments. In zinc-limited cells, surface levels increase markedly because the rate of endocytosis decreases. Surprisingly, endocytosis of both P200L and G539R is no longer zinc responsive; these proteins are endocytosed at a slow rate regardless of zinc status. These effects suggest a zinc sensing mechanism for regulating Zip4 trafficking in response to zinc.

Acrodermatitis↗
PubMed1978

Infantile papular acrodermatitis (Gianotti's disease) and intrafamilial occurence of acute hepatitis B with jaundice: age dependency of clinical manifestations of hepatitis B virus infection.

Infantile papular acrodermatitis (IPA, Gianotti's disease) is a clinical manifestation of hepatitis B virus (HBV) infection in childhood. An epidemic of of IPA occurred in Matsuyama, Japan, where 153 patients in a pediatric clinic had IPA between October 1974 and March 1977. In this period 12 mothers and two fathers of patients contracted acute hepatitis B with overt jaundice three to 14 months after their offspring had IPA. Analysis of the subtype of hepatitis B surface antigen (HBsAg) in the infants and their jaundiced mothers and/or fathers disclosed that HBV was transmitted from the infants. All of the index cases were one year old or younger, although the age of patients with IPA ranged from three months to 10 years. In approximately 40% of patients with IPA who were one year old or younger, HBs antigenemia persisted for one year. These facts suggested that the contraction of IPA in children, especially those one year old or younger, was an important route toward establishment of the carrier state of HBV which maintains the reservoir of this virus in the community.

Acrodermatitis↗
PubMed1998

The acrodermatitis enteropathica mutation affects protein expression in human fibroblasts: analysis by two-dimensional gel electrophoresis.

The acrodermatitis enteropathica (AE) mutation affects zinc uptake in human fibroblasts. However, the specific biochemical lesion has not been identified. We have used the technique of two-dimensional gel electrophoresis to identify protein differences in total cell lysate isolated from normal and AE fibroblasts. Two proteins with estimated molecular weights of 49.6 and 49.9 kDa and an isoelectric point of 5.1 were identified in normal fibroblasts but absent from AE fibroblasts. The proteins were purified, subjected to in-gel trypsin digest and the resulting peptides separated by HPLC. Sequences from three peptide fragments (8, 15 and 18 amino acids) were obtained after Edman degradation. None of the fragments exhibited homology to any amino acid sequences in the nonredundant Genbank database. The 15 and 18 amino acid fragments each exhibited 100% homology to a 136 amino acid expressed sequence tag that was homologous (43%) to adipophilin. However, the 15 and 18 amino acid fragments were only 30 and 44% homologous, respectively, to corresponding regions within the expressed sequence tag. Therefore, the 49.6/49.9 kDa protein absent from AE fibroblasts was not related to adipophilin. The 8 amino acid fragment did not exhibit homology to any expressed sequence tag. Therefore, the 49.6/49.9 kDa proteins are novel and may be the cause of the reduced zinc uptake and abnormal zinc metabolism characteristic of fibroblasts carrying the AE mutation.

Acrodermatitis↗