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Peripheral neuropathy in acrodermatitis chronica atrophicans - a late Borrelia manifestation.

Clinical and/or neurophysiological signs of peripheral neuropathy were found in 64% of 63 consecutive untreated patients with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA). The neuropathy frequency was significantly higher in the patients than in 30 age- and sex-matched control persons of whom 27% had neuropathy findings. The most common neuropathy in ACA was a symmetric distal sensory polyneuropathy. In a subgroup of patients with localized or asymmetric neuropathy, the changes were found more often in extremities with than without visible ACA lesions. Neuropathy symptoms, most often pain and/or paresthesia, were present in 64% of the patients, compared to in 13% of the control persons. Thus, both symptoms and signs of neuropathy were significantly more frequent in patients with untreated ACA than in control subjects.

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Some ultrastructural aspects of lymph-node cells and hepatocytes in papular acrodermatitis of childhood.

Ultrastructural examination of the inguinal lymph-nodes of six patients with papular acrodermatitis of childhood with hepatitis B antigenemia showed the presence of several Langerhans cells and, in three cases, the presence of some mononucleate cells, the cytoplasm of which contained groups of 20-50 spherical particles, approximately 150 A in diameter and with an electron-dense core. In the hepatocytes of two of these patients we observed spherical particles ranging in size between 150 A and 220 A, having an electron-lucent core and scattered throughout areas limited by a membrane.

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Dermal vessels in acrodermatitis chronica atrophicans.

An ultrastructural investigation of two patients suffering from acrodermatitis chronica atrophicans revealed in the small dermal vessels swelling of the endothelial cells, material of probably plasmatic origin accumulated in the subendothelial area, and sleeves of basement membrane-like material on a concentric perivascular layout.

Acrodermatitis↗

Histopathological study of transient acrodermatitis enteropathica due to decreased zinc in breast milk.

Two children, one born prematurely and the other born at full term developed acrodermatitis enteropathica due to marginal or low levels of zinc in their mothers' breast milk. Skin from both patients was studied with light and electron microscopy. The most characteristic light microscopic features were parakeratosis, absence of the granular layer, and pallor of the upper epidermal cells. Normal flattening of the upper Malphighian cells did not occur. Electron microscopic examination revealed that keratohyalin was decreased to absent, and the upper malpighian cells were edematous with vacuoles and large numbers of ribosomes, but small numbers of tonofilaments. Large amounts of keratinosome-derived-lamellae were found in the intercellular spaces in the keratinization area. The keratinosome-derived-lamellae were focally intermingled with opaque lipid plaques or myelin figures, probably derived from keratinosomes. The electron microscopic findings show abnormal keratinization and suggest that it is related to a disturbance of keratinosome metabolism due to zinc deficiency.

Acrodermatitis↗

Acrodermatitis chronica atrophicans: a light and electron microscopic study.

Degeneration of the elastica and collagen fibres in skin biopsies from patients with acrodermatitis chronica atrophicans was studied with light and electron microscopy. Elastic fibres were involved in the infiltrative stage while the elastin plexus was still present. In the atrophic phase, only fragments of elastic and oxytalan fibres were seen and the elaunin plexus was absent. Some collagen fibres were surrounded by osmiophilic material. In all biopsies, myelin sheaths were collapsed without axon structures. Spirochetes could be demonstrated in 69% of the biopsies and were most numerous in infiltrative and nodular lesions. The loss of elasticity of the skin in the atrophic phase may be caused by the destruction of both elastic and elastin fibres.

Acrodermatitis↗

Successful cultivation of spirochetes from skin lesions of patients with erythema chronicum migrans Afzelius and acrodermatitis chronica atrophicans.

Spirochetes could be cultivated from 9 out of 13 skin biopsies from patients with erythema chronicum migrans Afzelius (ECMA) and from 2 out of 5 biopsies from patients with acrodermatitis chronica atrophicans (ACA) by using a newly modified serumless Kelly's medium. The different spirochete strains grew best at a low oxygen tension. Attempts to grow spirochetes from blood and cerebrospinal fluid failed. The cultivation of spirochetes from secondary ECMA lesions favours the presumption that a spirochetemia may occur in ECMA. The isolation of spirochetes from an ACA patient who had a disease duration of greater than 10 years proves that the spirochetes may survive in the human body for a considerable time.

Acrodermatitis↗

Diagnostic features, confirmation and disease progression in 28 cases of lethal acrodermatitis of bull terriers.

Lethal acrodermatitis (LAD) is a genetically determined metabolic disease of bull terriers first described in the USA in the 1980s. In this study, the largest so far reported, 28 bull terriers born in the UK were diagnosed as suffering from LAD, and the clinical findings and the progression of the disease with time are described. The main characteristics of LAD are stunting, splayed digits, eating difficulties, skin disease of the face and feet, and increased susceptibility to microbial infections. In older dogs, paronychia, nail disease and hyperkeratosis of the footpads develops, becoming severe in dogs over six months of age. A diagnosis of LAD can be strongly suspected in any bull terrier showing a combination of the aforementioned signs from an early age. Dermatohistopathological demonstration of marked parakeratotic hyperkeratosis is strongly supportive of the diagnosis of LAD and, in association with the typical clinical findings, is sufficient to confirm a diagnosis. Although many of the clinical signs and the pathology of this condition suggest zinc deficiency, the measurement of blood zinc levels as a diagnostic aid is of limited value.

Acrodermatitis↗

Malassezia and Candida infections in bull terriers with lethal acrodermatitis.

In 12 cases of lethal acrodermatitis (LAD), four sampling techniques (brush, swab, scrape and adhesive tape strip) were used to study the distribution of yeasts in various body sites and these results were compared with those from five cases of atopic dermatitis and those of 10 normal dogs. Malassezia was frequently isolated from lesional and non-lesional skin and haircoat, footpads, nails and mucous membranes from dogs with either LAD or atopic dermatitis, although, generally, more Malassezia organisms were isolated from LAD cases. In normal dogs, Malassezia was most frequently recovered from the ear canal and the perianal skin. Candida was isolated frequently from dogs with LAD, but only a single isolate of this yeast was found in the other two groups. Fungal hyphae and pseudohyphae, probably Candida albicans, could be detected in samples collected from the nails and footpads of dogs with LAD. Both Malassezia and Candida could be isolated using all four sampling techniques. The MacKenzie (toothbrush) technique and adhesive tape strip cultures proved simple methods for the semiquantitative evaluation of yeasts. The high recovery rate of Malassezia and Candida from dogs with LAD is probably related to immune dysfunction, particularly T-cell dysfunction, known to be present in these dogs. C albicans infection may in part be responsible for the pathogenic changes of the nails and footpads commonly seen in cases of LAD.

Acrodermatitis↗

Antibiotic therapy of early European Lyme borreliosis and acrodermatitis chronica atrophicans.

In a study on 121 consecutive patients with erythema migrans, 65 patients obtained oral penicillin, 36 tetracyclines, and 20 amoxicillin-clavulanic-acid. Follow-up was carried out for a median of 29, 17, and 7 months, respectively. In another limited trial on 29 patients with acrodermatitis chronica atrophicans (ACA), 14 patients received oral penicillin, 9 parenteral penicillin, and 6 tetracyclines. There was no statistically significant difference among treatment groups in both therapeutic trials, with the exception of different follow-ups due to the nonrandomized study design and different occurrence of the Jarisch-Herxheimer reaction in patients with erythema migrans. Later extracutaneous manifestations developed in 27% of the patients with erythema migrans and in 47% of the patients with ACA despite antibiotic therapy. We could not prove the superiority of any antibiotic tested in either early or late European Lyme borreliosis.

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Serodiagnosis of erythema migrans and acrodermatitis chronica atrophicans by the Borrelia burgdorferi flagellum enzyme-linked immunosorbent assay.

The diagnostic performance of an enzyme-linked immunosorbent assay (ELISA) using purified Borrelia burgdorferi flagella as test antigen was compared with that of a B. burgdorferi sonic extract ELISA. We tested sera from 200 healthy controls, 107 patients with erythema migrans (EM), 50 patients with acrodermatitis chronica atrophicans (ACA), and 98 patients with various dermatological disorders without clinical evidence of active Lyme borreliosis. The flagellum ELISA was significantly more sensitive than the sonic extract ELISA. With sera from patients with EM, the diagnostic sensitivity for immunoglobulin G (IgG) antibody detection increased from 11.2 to 35.5% (P less than 0.001) and for IgM antibody detection it increased from 16.6 to 44.8% (P less than 0.001). In the flagellum ELISA, the number of positive tests increased significantly (P less than 0.005) when the duration of EM exceeded 1 month, but still only about 50% of patients with longstanding (1 to 12 months) untreated EM were IgG seropositive. Concomitant general symptoms did not affect the antibody level, whereas patients with multiple erythema were more frequently seropositive. All sera from patients with EM which were positive in the sonic extract ELISA were also positive in the flagellum ELISA. Not only did the overall number of positive tests increase, but the flagellum ELISA yielded a significantly better quantitative discrimination between seropositive patients and controls (P less than 0.002). IgG antibodies to the B. burgdorferi flagellum were found in all sera from patients with ACA, indicating persistence of an antiflagellum immune response in late stages of Lyme borreliosis. IgM reactivity in sera from patients with ACA was shown to be unspecific and the result IgM rheumatoid factor. A rheumatoid factor was detected in sera from 32% of patients with ACA, compared with 7.5% of patients with EM. The improved diagnostic performance, the ease of standardization of the flagellum antigen, and the lack of strain variation make the B. burgdorferi flagellum a needed reference antigen for growing routine serology in Lyme borreliosis.

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Detection of Borrelia burgdorferi sensu lato in lesional skin of patients with erythema migrans and acrodermatitis chronica atrophicans by ospA-specific PCR.

The aim of this study was to develop a sensitive and specific PCR for the detection of Borrelia burgdorferi DNA. The plasmid-located gene coding for the outer surface protein A (OspA [31-kDa protein]) was used as a target. Nucleotide sequence information from different B. burgdorferi ospA genotypes was used to design primers homologous to different genotypes. The sensitivity of the nested PCR differed from 1 fg to 1 pg of borrelial DNA, depending on the strain analyzed. No cross-reactions with DNA from spirochetes other than B. burgdorferi or with human DNA were observed. A total of 22 skin biopsy samples from patients with erythema migrans (EM [n = 10]) or acrodermatitis chronica atrophicans (ACA [n = 12]) were examined for the presence of B. burgdorferi by nested PCR. Of 22 biopsies, 80% from EM patients and 92% from ACA patients were positive by PCR amplification. By comparison, 50% of the EM patients had elevated B. burgdorferi-specific immunoglobulin M (IgM) and/or IgG antibody levels as tested by enzyme-linked immunosorbent assay (ELISA) using purified B. burgdorferi flagella as antigen. A total of 33% of ACA patients had elevated IgM titers, and all had high IgG titers in their sera. Only 30% of specimens from patients with EM and none from patients with ACA were positive by culture. All culture-positive specimens were also positive by PCR. Thus, the sensitivities of the PCR were 80 and 92%, respectively, for patients with EM and ACA on the basis of the clinical and histopathological diagnoses of Lyme disease. From these results, we conclude that PCR is a suitable method to detect B. burdorferi sensu lato DNA in skin biopsy samples and could be applied as an additional diagnostic tool.

Acrodermatitis↗

Reversible intestinal mucosal abnormality in acrodermatitis enteropathica.

In 3 cases of acrodermatitis enteropathica duodenal biopsy performed at the outset of treatment showed a similar abnormality of the intestinal mucosa. Further biopsies taken during treatment showed progressive improvement of the intestinal mucosa with subsequent complete restoration of the normal cellular and villous pattern. The initial treatment was with expressed human breast milk and oral di-iodohydroxyquinoline. The latter was continued alone and later replaced by zinc sulphate. Changes in the intestinal epithelial cells and inflammatory cell infiltration of the lamina propria still detectable on di-iodohydroxyquinoline therapy reverted to normal with oral zinc.

Acrodermatitis↗

Zinc-dependent chemotactic defect in an infant with acrodermatitis.

A 5-month-old infant developed acrodermatitis enteropathica when weaned from breast milk to cows' milk. Plasma zinc concentration was low. T- and B-lymphocyte functions were normal, but there was a severe defect of neutrophil chemotaxis. Oral treatment with zinc sulphate (40 mg daily) induced an immediate clinical improvement, with restoration of normal plasma zinc level and complete correction of neutrophil chemotaxis.

Acrodermatitis↗

Papular acrodermatitis with cytomegalovirus hepatitis.

The syndrome of papular acrodermatitis of childhood with hepatitis (Gianotti-Crosti syndrome) is classically considered to be associated with hepatitis B surface antigen (HBsAg) infection. We report an infant with the syndrome, but with serological evidence of infection by cytomegalovirus.

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Acrodermatitis chronica atrophicans.

Two cases of acrodermatitis chronica atrophicans associated with Borrelia burgdorferi infection are reported; to our knowledge these are the first cases reported in children.

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Ocular histopathology of acrodermatitis enteropathica.

Acrodermatitis enteropathica is the clinical expression of congenital zinc deficiency and is now treated with supplemental zinc. This report details the ocular histopathology of a child who died before efficacious treatment was available. The findings include corneal epithelial thinning and loss of polarity, anterior corneal scarring and loss of Bowman's membrane, cataract formation, ciliary body atrophy, retinal degeneration, RPE depigmentation, and optic atrophy.

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Zinc and di-iodohydroxyquinoline therapy in acrodermatitis enteropathica.

A 25-year-old patient with acrodermatitis enteropathica who had been treated with di-iodohydroxyquinoline for 20 years was changed to zinc sulphate therapy and studied under full metabolic balance control for zinc, calcium, magnesium, and inorganic phosphorus. The results obtained indicate that there is only a small overall deficit of body zinc stores in this disease and that the function of di-iodohydroxyquinoline is to increase the amount of zinc absorbed and retained by the body.

Acrodermatitis↗

Successful treatment of acrodermatitis continua of Hallopeau with sequential combination of calcipotriol and tacrolimus ointments.

Acrodermatitis continua of Hallopeau (ACH) is a rare type of pustular psoriasis affecting the digits. We report on a 43-year-old female patient who had been suffering from ACH for more than 20 years. Despite the fact that the disease was localized on one finger during the whole period, several topical and systemic treatments resulted in only temporary or partial improvement of the lesion. Although the monotherapies with calcipotriol and tacrolimus ointments gave no satisfying results in the long-term management of the disease, the combination of both agents led to a continuous improvement of the patient's skin condition.

Acrodermatitis↗