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Acrodermatitis chronica atrophicans--a spirochetosis. Clinical and histopathological picture based on 32 patients; course and relationship to erythema chronicum migrans Afzelius.

The recent discovery that spirochetes transmitted by the tick Ixodes ricinus are involved in the etiology of erythema chronicum migrans Afzelius (ECMA), Bannwarth's syndrome, and acrodermatitis chronica atrophicans (ACA) has thrown new light upon these disorders. Thirty-two patients showing clinical and serological evidence of ACA were investigated. Histologically, constant findings in active ACA lesions were telangiectases and a lymphocytic infiltrate with a moderate to rich admixture of plasma cells. Clinically, besides ACA lesions, lichen sclerosus et atrophicus (LSA)-like lesions were found in five patients. Four of these patients displayed a histopathological picture compatible with LSA. These findings suggest a relationship between ACA and LSA. In six patients spontaneous healing of ECMA was followed by ACA lesions after a latency period of 1-8 years. Six patients reported histories of cranial nerve involvement. Radiography revealed subluxation of joints in hands or feet in six patients, and periosteal thickening in another three patients. The results indicate that ACA may be a late manifestation of infection with the same spirochete that causes ECMA and Bannwarth's syndrome. If untreated, the infection may continue for many years and result in irreversible degenerative lesions.

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A clinical, histological, and immunohistochemical comparison of acrodermatitis chronica atrophicans and morphea.

We compared 19 patients with acrodermatitis chronica atrophicans (ACA), a dermatosis caused by Borrelia burgdorferi infection, and 40 patients with morphea, a disease of heterogeneous origin where a borrelia etiology has been suggested in some cases, both clinically and histologically to define the differences between these two dermatoses. Clinically, ACA involves acral body sites with lower temperatures, is seen mostly in elderly persons, and presents as a livid discoloration that is not sharply demarcated. Morphea can be localized in embryonal structures, affects any age and body site, and exhibits extension at the periphery of the lesions. Histologically, ACA shows atrophy of collagen and elastic tissue as well as hypertrophic basophilic elastic tissue; whereas in morphea, sclerosis and polarizing elastic tissue are prominent. Graft-versus-host-like reactions may be present in both dermatoses. Immunohistochemical testing with different lymphocyte markers showed differences only in the expression of HLA-DR antigens. These conditions can be distinguished from each other on a clinical and histological basis in most cases. In 17% of morphea biopsy specimens, however, histological differentiation from ACA was not possible. Moreover, the histological pattern of morphea was not associated with a positive borrelia serology.

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Bullous lesions in acrodermatitis enteropathica. Histopathologic findings regarding two patients.

Acrodermatitis enteropathica (AE) is an autosomic recessive disorder affecting early infancy. Two cases of infantile AE with low plasma zinc levels are reported in which unusually prominent bullous and vesicobullous lesions were seen on the hands and feet, in addition to the more typical erythematous and scaly patches. Both psoriasiform and bullous lesions responded dramatically to oral zinc-sulfate supplementation. The histopathologic features of the bullous lesions of AE have not previously been fully examined. Histologically, the bullous lesions were characterized by intraepidermal vacuolar changes with massive ballooning, leading to intraepidermal vesiculation and blistering, with prominent epidermal necrosis and with no acantholysis. The bullous lesions did not arise on erythematous patchy lesions, but developed ex novo on unaffected skin. The histopathologic differential diagnosis with other bullous conditions is discussed.

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Picolinic acid in acrodermatitis enteropathica: evidence for a disorder of tryptophan metabolism.

Three children with acrodermatitis enteropathica (AE) were treated with oral zinc dipicolinate (zinc-PA). The daily dose of zinc required to prevent exacerbations, when administered as the dipicolinate complex, was one-third the minimum amount of zinc required as the sulfate salt. The concentration of picolinic acid in the plasma of asymptomatic children with AE was significantly less than that of normal children. However, oral treatment with PA alone was ineffective. The plasma of the three AE children contained a measurable quantity of kynurenine which was undetectable in plasma from normal children. Absorption of an oral zinc load was normal. The results support the hypothesis that the genetic defect in AE is in the tryptophan pathway, although the role of PA in zinc metabolism remains to be defined.

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Acrodermatitis chronica atrophicans Herxheimer can often mimic a peripheral vascular disorder.

During the past few years five patients have been referred to the angiology section at Danderyd Hospital under the diagnosis of chronic venous insufficiency but who were instead suffering from acrodermatitis chronica atrophicans (ACA). The typical case of ACA starts with a limited inflammatory lesion, which is gradually replaced by atrophy and the skin shows a bluish, red discoloration. Late changes may be subluxation of joints in hands or feet and periosteal thickening. Capillary microscopy often reveals a clear picture of atrophy and a prominent, dilated subpapillary venular plexus. If localized to the leg the blood flow of the foot and lower leg may be increased, skin temperature elevated and venous capacity and return augmented. All these variables can consequently mimic venous insufficiency of the leg. ACA is caused by a Borrelia infection and serological testing will always show a significantly elevated titer to Borrelia. The disease is most often easily cured by 2-3 g of penicillin daily for two to three weeks.

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Case of acrodermatitis continua accompanied by psoriatic arthritis.

Acrodermatitis continua of Hallopeau (ACH) is a rare chronic pustular eruption that predominantly involves the fingertips. The characterization of this disease has been confused. Some have considered it as a separate entity while others as a variant of pustular psoriasis. The presented patient simultaneously had ACH and joint lesions which were diagnosed as psoriatic arthritis. We believe that because ACH may be accompanied by psoriatic arthritis, as in this case, it could be evidence that it is a variant of psoriasis.

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Acrodermatitis enteropathica.

The first publication identifying acrodermatitis enteropathica as a definite disease (Danbolt & Closs, 1942) is reviewed. Later studies are briefly surveyed, resulting in the recognition of the disease as a zinc deficiency which can be effectively corrected by administration of small oral doses of zinc.

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Papular acrodermatitis of childhood and other papulo-vesicular acro-located syndromes.

Papular acrodermatitis of childhood (PAC), first recognized in Milan and described by Gianotti in 1955, is an infectious disease of childhood, of low infectivity, fairly widespread, and characterized by: (1) Non-relapsing erythemato-papular dermatitis localized to the face and limbs, lasting about 3 weeks. (2) Paracortical hyperplasia of lymph-nodes. (3) Acute hepatitis, usually anicteric, which lasts at least 2 months and may progress to chronic liver disease.

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65Zinc absorption in patients suffering from acrodermatitis enteropathica and in normal adults assessed by whole-body counting technique.

65Zinc absorption was studied in five acrodermatitis enteropathica (AE) patients and in eight normal adults by means of a whole-body counting assay. The absorption was calculated from retention values recorded in the time interval 8-30 days after oral administration of the isotope. Two AE patients (7 and 13 years old) had a low absorption, 3.3 and 1.8% respectively, corroborating their high need for additional elemental zinc (about 2 mg/kg/day). Three adult AE patients, all in their twenties, had a considerably lower need for extra zinc (about 0.2 mg/kg/day). Their zinc absorption ranged from 28 to 36% (mean 34%). In the controls the range was 27 - 65% (mean 43%). Turnover of retained 65Zn from day 8 - 30 was about 0.7% in the patient as well as in the control groups. Oral zinc therapy was withdrawn prior to the study. During the zinc-free period (3-7) a marked decrease in serum zinc and serum alkaline phosphatase values was noted in the two children with AE and they showed clinical evidence of zinc deficiency (angular stomatitis, scaling around finger nails, and irritability). None of the adult patients showed such evidence of impending zinc deficiency. One complained of exacerbation of facial acne, and another of pain in her feet. All symptoms disappeared promptly when oral zinc therapy was resumed.

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Zinc deficiency with transitory acrodermatitis enteropathica in a boy of low birth weight.

A premature male baby fed on his mother's milk developed zinc deficiency and a skin disorder inseparable from acrodermatitis enteropathica. Following zinc therapy the skin lesions healed. Later the treatment was withheld and no recurrence was seen during 30 months' observation. The boy's zinc deficiency was thought to be due to a high requirement secondary to rapid growth, to poor zinc supply in food and, possibly, to inefficient zinc absorbtion.

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Acrodermatitis chronica atrophicans: a case report and review of the literature.

We report a 55-year-old woman with acrodermatitis chronica atrophicans (ACA) and a peripheral sensory neuropathy. ACA is an uncommon late cutaneous manifestation of Lyme disease, which follows disseminated Borrelia burgdorferi infection. This is the second published case from the U.K. since serological diagnosis has been available. In this patient the diagnosis was confirmed by serology using a sensitive enzyme-linked immunosorbent assay and immunoblotting techniques. B. burgdorferi DNA was demonstrated in the affected skin using the polymerase chain reaction, although staining and cultures for the organism were negative. Recommended treatment of ACA is with oral doxycycline 100 mg twice daily for 28 days, but our patient did not respond well to this regimen. She was therefore treated with ceftriaxone intravenously for 21 days, which resulted in a rapid symptomatic and clinical response.

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Successful treatment of acrodermatitis continua suppurativa with topical tacrolimus 0.1% ointment.

Acrodermatitis continua suppurativa of Hallopeau (ACS) is a rare pustular variant of psoriasis in which numerous treatment modalities have been used without any consistent long-term effect. We report for the first time two patients with ACS which was successfully treated with topical tacrolimus 0.1% ointment. Our observations raise hopes that this new treatment strategy for ACS may constitute a novel effective therapeutic option for this recalcitrant condition.

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Acrodermatitis continua responding to cyclosporin therapy.

A 69-year-old man with painful, recalcitrant, localized acrodermatitis continua developed widespread pustulation which was resistant to high-dose methotrexate therapy. Low-dose cyclosporin induced a rapid and persistent remission.

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Acrodermatitis enteropathica in Saudi Arabia.

Four patients with acrodermatitis enteropathica (AE) are presented from three Saudi families. The clinical picture in these patients is basically similar to that described elsewhere. These patients, however, showed different degrees of involvement of various body systems. In all cases, low serum zinc levels were documented, and they responded well to zinc supplementation. In the follow-up evaluation, angular cheilitis was observed as a feature that appears to herald the relapse of disease. Interestingly, when patients are treated, it was the last sign to disappear. Current information on zinc is summarized.

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Papular acrodermatitis of childhood associated with hepatitis A virus infection.

A 2-year-old girl developed an erythematous papular eruption on her face and extremities a week after an epidemic of hepatitis A had occurred in her school. Clinical and laboratory signs of acute hepatitis, together with serologic verification, confirmed hepatitis A infection. That diagnosis should be considered in the etiology of papular acrodermatitis of childhood.

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Polarization microscopy of hair in acrodermatitis enteropathica.

We studied the hair of a 10-month-old girl who was suffering from acrodermatitis enteropathica, using light and polarizing microscopy before and after institution of zinc therapy. The hair was very thin and brittle. On light microscopy the shafts showed uneven diameter and some displayed atypical trichorrhexis nodosa with stretched fractures. Ten percent of the hair fibers exhibited nodal swellings of the pseudomonilethrix type. Polarization microscopy disclosed in 70% of all hair shafts an irregular pattern of alternating dark and bright bands. This anomaly was still present in 10% of the hair shafts after one and one-half years of zinc therapy, but could no longer be detected after two years of zinc supplementation. Repeated determinations of hair probes before and after treatment gave a low cystine content, however, being still in the normal range. We assume that the observed changes and the low hair cystine content can be attributed to the underlying zinc deficiency.

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