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At least 19 recordsLinked to original sources
PubMed1976

[Infantile papular acrodermatitis. Acrodermatitis papulosa and the infantile papulovesicular acrolocalized syndrome].

Papular acrodermatitis of childhood is an infectious disease characterized by a non-relapsing, non-itching, monomorphic erythemato-papular dermatitis limited to the face and limbs. It is always associated with anacute hepatitis, with hepatitis B antigen in the serum and with a reactive reticulohistiocytic lymphadenitis. In childhood other types of papular or papulovesicular acro-located eruptions, itching or non-itching, associated with reactive lymphadenitis, are observed, in the course of known diseases and with unknown cause. These acro-located cutaneous eruptions of unknown origin, which show varying features, should be classified as "papulovesicular acrolocated syndrome" until their ethiopathogenesis is known.

Acrodermatitis↗
PubMed1989

Acrodermatitis enteropathica with normal serum zinc levels: diagnostic value of small bowel biopsy and essential fatty acid determination.

We report a patient with acrodermatitis enteropathica and a normal serum zinc level in whom the diagnosis was confirmed by plasma phospholipid fatty acid and a small bowel biopsy response to oral zinc therapy. Acrodermatitis enteropathica is a rare autosomal recessive condition of zinc deficiency characterised by chronic diarrhoea associated with failure to thrive, periorificial dermatitis and alopecia, susceptibility to infections and behavioural changes. Diagnosis is usually established by reduced serum zinc levels (classical acrodermatitis enteropathica). Paneth cell abnormalities on electron microscope of a small bowel biopsy can be supportive. A few cases with the typical picture of acrodermatitis enteropathica without hypozincaemia (variant acrodermatitis enteropathica) have been described. The diagnosis of variant acrodermatitis enteropathica to date has been based on an entirely empiric, but nonetheless convincing clinical response to oral zinc therapy. Laboratory aids to diagnosis have been lacking.

Acrodermatitis↗
PubMed2003

Novel SLC39A4 mutations in acrodermatitis enteropathica.

Acrodermatitis enteropathica is an autosomal recessive disease characterized by skin involvement due to defective intestinal zinc absorption. Usually, the skin lesions include erythema, erosions, and small blisters in perioral, perianal regions, and hands and feet, which develop soon after weaning from the breast. The acrodermatitis enteropathica gene has been localized to chromosomal region 8q24.3 and subsequently the SLC39A4 gene has been disclosed as the acrodermatitis enteropathica gene. SLC39A4 mutations have been demonstrated in several acrodermatitis enteropathica families, and in this study we have examined two Japanese acrodermatitis enteropathica families for SLC39A4 mutations. The mutation detection strategy consisted of polymerase chain reaction amplification of all 12 exons and flanking intronic sequences, followed by direct nucleotide sequencing. It revealed three novel mutations, 1017ins53, which creates a premature termination codon, and two mis-sense mutations, R95C and Q303H.

Absorption↗
PubMed2000

Acrodermatitis enteropathica-like eruption in an infant with nonketotic hyperglycinemia.

Acrodermatitis enteropathica is a rare inherited disorder characterized by zinc deficiency and a triad of dermatitis, diarrhea, and alopecia. It is an autosomal recessive condition thought to be due to the inability to absorb zinc from the gastrointestinal tract. Acquired zinc deficiency due to a variety of etiologies may produce a similar clinical picture. These causes include inadequate supply, malabsorption, and low zinc stores. In addition to zinc, deficiencies of other nutrients such as branched chain amino acids have induced an acrodermatitis enteropathica-like eruption. We describe a case of a 26-month-old boy with a rare inborn error of metabolism known as nonketotic hyperglycinemia who developed an acrodermatitis enteropathica-like eruption. In addition to zinc deficiency, the patient was deficient in branched chain amino acids due to a low protein diet instituted to reduce his elevated glycine levels. The rash did not respond to zinc replacement alone, and therefore is most likely a combination of amino acid and zinc deficiency. Acrodermatitis enteropathica-like eruptions have been described in other conditions that cause decreased serum amino acids, such as maple syrup urine disease and organic acidurias. This is the first case describing an association between acrodermatitis enteropathica and nonketotic hyperglycinemia.

Acrodermatitis↗
PubMed1994

Acrodermatitis enteropathica, zinc metabolism, copper status, and immune function.

OBJECTIVE: To study zinc metabolism, copper status, and immune function in a patient with acrodermatitis enteropathica. RESEARCH DESIGN: Case report. PATIENT: A 16-year-old boy with acrodermatitis enteropathica. INTERVENTION: Change of zinc supplementation dosage from 1000 to 525 mumol/d. MEASUREMENTS AND RESULTS: Zinc metabolism was studied with an oral dose of zinc chloride Zn 65 and whole-body counting at both zinc dosages. Zinc, copper status, and immune indexes were also measured at both dosages. The higher dosage of zinc supplementation was found to induce a state of low copper status and immune dysfunction. Lowering the dosage normalized these indexes. Zinc absorption in this patient was found to be within the reference range for healthy subjects. At the lower dosage, zinc retention and the rate of whole-body turnover also normalized. These results suggest that the primary lesion in acrodermatitis enteropathica is a cellular defect in zinc metabolism rather than an impairment of zinc absorption. CONCLUSION: Zinc and copper status and immune function should be monitored regularly in patients with acrodermatitis enteropathica to provide a proper dosage of zinc during different physiologic stages.

Acrodermatitis↗
PubMedDate not supplied

Success and failure in the treatment of acrodermatitis chronica atrophicans.

To determine the most effective treatment for acrodermatitis chronica atrophicans, several clinical trials were undertaken in recent years to evaluate whether a 2-week course of ceftriaxone would be superior to oral antibiotics. Of the 46 patients suffering from acrodermatitis chronica atrophicans, 14 were treated with ceftriaxone 2g for 15 days. The remaining patients received either oral penicillin V 1.5 million IU t.i.d. or doxycycline 100 mg b.i.d. for 20 to 30 days. Patients were followed up for at least 1 year. Of the 14 ceftriaxone-treated patients four showed incomplete regression of the inflammatory skin changes after 6 to 12 months. Two out of five patients who were monitored for Borrelia burgdorferi DNA excretion were still positive after 12 months as compared to none of six patients who were treated orally for 20-30 days. Six out of 11 patients treated orally for only 20 days needed retreatment after 6 months because of continuing skin manifestations, neuropathy or arthralgia. A 30-day duration of treatment with oral antibiotics and not the chosen antibiotic is crucial for curing acrodermatitis chronica atrophicans. The duration of treatment with ceftriaxone needed for eradication of Borrelia in acrodermatitis chronica atrophicans has yet to be determined in future studies.

Acrodermatitis↗
PubMed2001

Genomic localization, organization and amplification of the human zinc transporter protein gene, ZNT4, and exclusion as a candidate gene in different clinical variants of acrodermatitis enteropathica.

Acrodermatitis enteropathica is an inherited disorder of zinc metabolism, the molecular basis of which is currently unknown. Recent transgenic mouse studies have highlighted the potential significance of certain zinc transport proteins, for example ZnT4, in providing clues to the pathogenesis of zinc-related disorders such as acrodermatitis enteropathica. Specifically, mice of any genotype suckled on ZnT4-deficient mice fail to absorb intestinal zinc and ZnT4-deficient mice also develop dermatitis, alopecia and stunted growth. Therefore, to assess human ZnT4 as a candidate gene/protein in acrodermatitis enteropathica or related disorders, we characterized the intron-exon organization of the human ZNT4 gene, which comprises seven distinct exons spanning approximately 38.7 kb. High-resolution radiation hybrid mapping placed ZNT4 on 15q21.1. We also developed a PCR-based mutation detection strategy using primers placed on flanking introns followed by direct sequencing of the PCR products. Using this approach, we sequenced DNA from five individuals with acrodermatitis enteropathica; no mutations were identified. Thus, ZNT4 is unlikely to be the correct candidate gene for this disorder. We also identified and characterized two common single nucleotide polymorphisms in exon 5 and in the 3' UTR of ZNT4, which will be useful for future genetic linkage studies in assessing ZNT4 as a candidate gene for other inherited disorders of zinc metabolism.

Acrodermatitis↗
PubMed1982

Malignant melanoma and dermatofibrosarcoma in a 60-year-old patient with lifelong acrodermatitis enteropathica.

The life course of acrodermatitis enteropathica is recorded in a 62-year-old white man. Initially saved in infancy by breast-feeding and good medical care, later in his twenties he responded well to diiodohydroxyquinoline (Diodoquin) therapy, his only residua being dermatitis, hoarseness, and short stature. Subsequently untreated, this patient years later developed not only a dermatofibrosarcoma but also a large amelanotic malignant melanoma. Both were successfully excised. Subsequently, oral zinc therapy initiated for the first time cleared his acrodermatitis, which had been present for 60 years. It is suggested that this patient's malignancies developed as a result of an immune deficiency state typically found in acrodermatitis enteropathica. On this basis, acrodermatitis enteropathica may be viewed as having a malignant potential over the long term. The zinc-dependent nature of the immune deficit, however, suggests that lifelong daily zinc supplementation is an appropriate prophylactic measure.

Acrodermatitis↗
PubMed1998

Identification of three species of Borrelia burgdorferi sensu lato (B. burgdorferi sensu stricto, B. garinii, and B. afzelii) among isolates from acrodermatitis chronica atrophicans lesions.

In Europe, at least three species of Borrelia are known to be causative agents of Lyme borreliosis: B. burgdorferi sensu stricto, B. garinii, and B. afzelii. Observable differences in the molecular characteristics of the three species have led to speculation that they may also differ in their pathogenic potential and/or tissue tropisms. Several studies have found an association between the chronic skin manifestation of Lyme borreliosis, acrodermatitis chronica atrophicans, and infection by B. afzelii. We sought to find further evidence for such a correlation by studying the genetic profiles of 22 strains of B. burgdorferi sensu lato derived from 21 patients who presented to the University Medical Center, Ljubljana, Slovenia between 1992 and 1995. Strains were isolated in culture from skin biopsies of acrodermatitis chronica atrophicans lesions; in the case of one patient two separate acrodermatitis chronica atrophicans lesions were cultured. All 21 patients had clinically typical lesions with "classic" histopathology and high IgG antibody titers to B. burgdorferi sensu lato. Strains were characterized and typed by 16S ribosomal RNA-specific polymerase chain reaction and determination of their large restriction fragment patterns using pulsed-field gel electrophoresis of MluI-digested genomic DNA. Of the 22 isolates studied, 17 possessed the highly conserved MLa1 pattern characteristic of B. afzelii. The remaining five isolates possessed large restriction fragment patterns that were typical of B. garinii (MLg2, four isolates from three patients) and B. burgdorferi sensu stricto (MLb2, one isolate). The results of 16S ribosomal RNA-specific polymerase chain reaction were concordant with these species designations. These data show that B. afzelii is the predominant, but not the exclusive, etiologic agent of acrodermatitis chronica atrophicans.

Acrodermatitis↗
PubMed2000

Differential expression of cytokine mRNA in skin specimens from patients with erythema migrans or acrodermatitis chronica atrophicans.

Erythema migrans, the characteristic skin manifestation of acute Lyme borreliosis, is a self-limited lesion. In contrast, acrodermatitis chronica atrophicans, the typical cutaneous manifestation of late Lyme borreliosis, is a chronic skin condition. In an effort to understand pathogenic factors that lead to different outcomes in dermatoborrelioses, skin biopsy samples from 42 patients with erythema migrans and 27 patients with acrodermatitis chronica atrophicans were analyzed for mRNA expression of five pro-inflammatory cytokines (tumor necrosis factor alpha, interleukin-1 beta, interleukin-6, interferon-gamma, and interleukin-2) and two anti-inflammatory cytokines (interleukin-4 and interleukin-10) by in situ hybridization with cytokine-specific riboprobes. Among the 27 patients who had erythema migrans alone with no associated signs or symptoms, the major cytokines expressed in perivascular infiltrates of T cells and macrophages were the pro-inflammatory cytokine interferon-gamma and the anti-inflammatory cytokine interleukin-10. In the 15 erythema migrans patients who had associated signs and symptoms, including headache, elevated temperature, arthralgias, myalgias, or fatigue, a larger number of macrophages and greater expression of macrophage-derived pro-inflammatory cytokines, tumor necrosis factor alpha, interleukin-1 beta, and interleukin-6, were also found. In comparison, infiltrates of T cells and macrophages in the skin lesions of acrodermatitis chronica atrophicans patients had very little or no interferon-gamma expression. Instead, they usually expressed only the pro-inflammatory cytokine tumor necrosis factor alpha and the anti-inflammatory cytokine interleukin-4. Thus, the activation of pro-inflammatory cytokines in erythema migrans lesions, particularly interferon-gamma, seems to be important in the control of the spirochetal infection. In contrast, the restricted pattern of cytokine expression in acrodermatitis chronica atrophicans, including the lack of interferon-gamma, may be less effective in spirochetal killing, resulting in the chronicity of this skin lesion. J Invest Dermatol 115:1115-1123 2000

Acrodermatitis↗
PubMed1994

Molecular subtyping of Borrelia burgdorferi in erythema migrans and acrodermatitis chronica atrophicans.

Recently, three subtypes of Borrelia burgdorferi have been identified: Borrelia burgdorferi sensu stricto, Borrelia garinii, and the VS 461 group of Borrelia burgdorferi. These subtypes differ by nucleotide sequence variations within several Borrelia burgdorferi specific genes and most likely by their pathogenetic potential. To assess whether different subtypes of Borrelia burgdorferi might be associated with different cutaneous manifestations and clinical courses of Lyme disease, lesional skin biopsies from 35 patients with erythema migrans and 18 patients with acrodermatitis chronica atrophicans were analyzed. A Borrelia burgdorferi specific gene segment encoding a 26-kD protein with subtype specific nucleotide sequence variations was amplified by a nested polymerase chain reaction technique. For molecular subtyping, the products were transcribed into complementary RNA. Upon polyacrylamide gel electrophoresis, complementary RNA molecules separate into several metastable conformational forms resulting in patterns of bands highly specific for the nucleotide sequence of the transcribed molecules. In biopsy specimens of erythema migrans, the VS 461 subtype was detected in 28 of 35 and the Borrelia garinii subtype in six of 35 cases. In one of 35 cases of erythema migrans Borrelia burgdorferi sensu stricto as well as Borrelia garinii was detected. In contrast, in all 18 biopsies of acrodermatitis chronica atrophicans, only the VS 461 subtype was identified. This subtype is rarely found in the USA, where acrodermatitis chronica atrophicans is almost unknown. These data indicate that acrodermatitis chronica atrophicans might be closely associated with the VS 461 group of Borrelia burgdorferi.

Acrodermatitis↗
PubMed1986

Abnormal immune responses during hypozincaemia in acrodermatitis enteropathica.

Immune responses were characterized in six patients with acrodermatitis enteropathica during a break in zinc supplementation and during resupplementation. During hypozincaemia the number of T-cells increased but the amount of B-cells and the responses of T- and B-lymphocytes to phytohaemagglutinin, concanavalin A-, pokeweed mitogen- and Staphylococcus aureus stimulations in vitro were subnormal. Cell counts and stimulation results both normalized when serum zinc values improved. One patient was anergic to tuberculin while showing signs of acrodermatitis enteropathica; she converted during supplementation whereas the others were continuously positive. Three females had antibodies against nuclear antigens and slightly elevated serum IgE concentrations; these values were not affected by the break in supplementation. Four females were continuously rheumatoid factor positive. Our findings suggest that zinc deficiency is closely associated with impaired immune responses in patients with acrodermatitis enteropathica, and laboratory markers of autoimmunity occur in a considerable number of the acrodermatitis enteropathica patients, irrespective of their zinc status.

Acrodermatitis↗
PubMed1976

[Acrodermatitis enteropathica--zinc as a life-saving drug].

Formerly acrodermatitis enteropathica was treated with oxiquinoline without full control of the disease and with the danger of blindness. Moynahan has introduced zinc into the treatment with excellent results, based on the finding that acrodermatitis enteropathica is a zinc-deficiency disorder. Other authors and own observations have confirmed the findings of Moynahan. There are clinical, genetical, immunological and therapeutical parallels between the acrodermatitis enteropathica of man and the so-called hereditary parakeratosis of calves which also is a hereditary disturbance of zinc metabolism. The treatment of acrodermatitis enteropathica with zinc is furthermore a great success in the prevention of blindness, as zinc is not dangerous to the retina and the optic nerve.

Acrodermatitis↗
PubMed1998

Rheumatic manifestations related to acrodermatitis chronica atrophicans. A review of four cases.

BACKGROUND: Acrodermatitis chronica atrophicans is a delayed manifestation of Lyme disease caused by a Borrelia burgdorferi subspecies, B. afzelii. Although rheumatic manifestations are rare, they can result in deformities of the fingers and toes if they are not treated promptly. METHODS: We report four cases of acrodermatitis chronica atrophicans seen over a 15-year period. RESULTS: Two patients had a noninflammatory unilateral knee effusion and one had swelling of the dorsum of one hand. Antimicrobial therapy was followed by a full recovery in the three patients who received an early diagnosis. The remaining patient, a 63-year-old woman, had swelling and dysesthesia in the fingers of both hands. She developed finger deformities over a period of two years. Although the swelling resolved under antimicrobial therapy, she had persistent reducible deformities of the fingers consistent with Jaccoud's arthropathy. CONCLUSION: The diagnosis of acrodermatitis chronica atrophicans rests on a history of a tick bite, a suggestive skin biopsy histology and a positive Western blot for B. afzelii. A positive response to antimicrobial therapy is also required. Acrodermatitis chronica atrophicans, a common condition in central and northern Europe, can cause joint manifestations and persistent finger deformities in the absence of early treatment.

Acrodermatitis↗
PubMed1997

[PUVA-bath photochemotherapy in Hallopeau's acrodermatitis continua suppurativa].

A 73-year-old man presented with severe, relapsing acrodermatitis continua of Hallopeau, which had been resistant to prior local and systemic therapy for eight years. The patient was treated with selective hand PUVA-bath photochemotherapy. The cumulative dose of UVA used over 10 weeks of treatment was 54.6 J/cm2 on the palms and 26.8 J/cm2 on the dorsum of the hands. The single UVA doses ranged from 0.5 to 2.5 J/cm2 on the palms and 0.2 to 1.4 J/cm2 on the dorsum of the hands. After 16 treatment sessions, the acrodermatitis continua started to improve, and after 24 treatments, had cleared completely. In the four months following the PUVA therapy, there was no relapse. PUVA-bath photochemotherapy is an efficient therapeutic alternative in the treatment of acrodermatitis continua due to its clinical effectiveness and lack of any systemic side effects. It also possesses the advantage of allowing selective photosensitization of certain areas of the skin such as the hands.

Acrodermatitis↗