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PubMed2000

Polyneuropathy in late Lyme borreliosis - a clinical, neurophysiological and morphological description.

In a prospective study, detailed clinical and neurophysiological examinations were performed in 17 patients with polyneuropathy associated with the late borrelial manifestation acrodermatitis chronica atrophicans (ACA). Similar clinical and neurophysiological signs were found in most of the patients. The findings were those of a sensory polyneuropathy, mainly affecting large nerve fibres. Marked abnormality of vibration threshold was a common finding and in 4 patients this raised a suspicion of spinal cord engagement, in addition to a polyneuropathy. Sural nerve biopsy, performed in 3 of the patients, showed a mainly axonal neuropathy. Biopsy findings did not confirm earlier reports of vasculitis of epineural vessels in ACA-associated polyneuropathy.

Acrodermatitis↗
PubMed2000

Downregulation of class II molecules on epidermal Langerhans cells in Lyme borreliosis.

BACKGROUND: Borrelia burgdorferi can be isolated from the skin of patients with acrodermatitis chronica atrophicans (ACA), a late-stage manifestation of Lyme borreliosis; despite a marked T-cell infiltrate in lesional skin and high antibody titres in patients' sera. OBJECTIVES: To determine whether antigen-presenting Langerhans cells (LCs), which reportedly show signs of injury in erythema chronicum migrans (ECM), the early stage of disease, are altered in ACA. PATIENTS/METHODS: We studied the immunophenotype of cutaneous leucocytes on cryostat sections of lesional skin from both ECM and ACA patients. RESULTS: The total number of CD1a+ cells evaluated by semiautomatic image analysis was lower in ECM (594 +/- 263 cells mm(-2) epidermis) than in ACA (835 +/- 317 cells mm(-2) epidermis). HLA-DR expression was remarkably downregulated on CD1a+ LCs to 29% in ECM and 18% in ACA, whereas in normal skin, most of the epidermal CD1a+ dendritic cells were HLA-DR+. The inflammatory infiltrate was mainly composed of CD68+ macrophages and CD45RO+ memory T cells, with a predominance of CD4+ helper T cells. CONCLUSIONS: It is conceivable that the downregulation of major histocompatibility complex class II molecules on LC in both the early and late skin manifestations of Lyme borreliosis is indicative of a poorly effective anti-B. burgdorferi immune response and thus at least partly responsible for the insufficient elimination of this micro-organism from ACA skin.

Acrodermatitis↗
PubMed2001

Searching for candidate genes in the new millennium.

Completion of the entire sequence of the human genome is having a profound effect on the strategies biological scientists use to identify disease-associated genes. Laborious positional cloning approaches and traditional functional studies are gradually being transformed by emerging genomic and proteomic databases. Some of the exciting challenges investigators now face are the identification of new genes, determining the function of these genes, defining disease associations, and elucidating correlation between genotype and phenotype. To demonstrate how investigative methods for single-gene disorders are changing, we illustrate one possible approach in the search for the gene underlying the autosomal recessive genodermatosis, acrodermatitis enteropathica.

Acrodermatitis↗
PubMed1998

Symptomatic zinc deficiency in breast-fed term and premature infants.

Two 3-month-old exclusively breast-fed infants, one born at full-term and the other born prematurely, developed symptomatic zinc deficiency manifested by an acrodermatitis enteropathica-like eruption. Inadequate breast milk zinc was demonstrated in both cases. A rapid clinical response followed oral zinc supplementation after which their serum zinc levels returned to normal. The infants remained asymptomatic following cessation of zinc therapy. Reports of similar cases suggest that in a group of infants breast milk does not meet their nutritional zinc requirements. Inadequate breast milk zinc is thought to result from a defect in transfer of zinc from maternal serum to breast milk.

Acrodermatitis↗
PubMedDate not supplied

Gianotti-Crosti syndrome preceded by oral polio vaccine and followed by varicella infection.

Gianotti-Crosti syndrome (papular acrodermatitis of childhood) is considered a nonspecific cutaneous host response to a variety of infectious agents, particularly viruses. Herein we report a case of Gianotti- Crosti syndrome preceded by oral polio vaccine and followed by varicella infection. The present case may support the hypothesis of initial immunization and following viral infection as a possible mechanism for Gianotti-Crosti syndrome.

Acrodermatitis↗
PubMed1978

[Serum IgE in skin diseases of children (author's transl)].

Using the paper-radioimmunosorbens test (PRIST) normal values were obtained from 200 non-allergic children. The IgE level was aged-dependent, in part with significant differences between various age groups. These normal values were compared with those in children with seborrhoeic dermitis, constitutional neurodermatitis, parasitoses, urticaria, Quincke oedema, Schönlein-Henoch purpura, pityriasis rosea, multiform exudative erythema, erythema nodosum and infantile papular acrodermatitis. Half-life of IgE in neonates was 16.2 hours.

Acrodermatitis↗
PubMed1985

[Zinc deficiency syndrome as a side effect of chelating agents].

Zinc deficiency syndrome with acrodermatitis enteropathica-like skin changes developed in a 66-year-old patient during treatment of manganese poisoning with the chelating agents CaNa2-EDTA and Ca-trisodium-pentetate, which had reduced the serum-zinc level. The skin changes cleared up after oral administration of zinc aspartate.

Acrodermatitis↗
PubMed2000

Histopathologic features seen in Gianotti-Crosti syndrome secondary to Epstein-Barr virus.

BACKGROUND: Gianotti-Crosti syndrome (GCS) or infantile papular acrodermatitis presents as a symmetric erythematous lichenoid papular and papulovesicular eruption of the face, extremities, and buttocks, usually occurring in young children. GCS has been associated with hepatitis B and enteroviruses, as well as Epstein-Barr virus (EBV) and, rarely, cytomegalovirus. OBJECTIVE: The purpose of this study was to use immunohistochemical studies to determine the pattern of the lymphoid infiltrate and evidence for viral antigens in cases of EBV-associated GCS. METHODS: Routine histologic and immunohistochemical stains were evaluated in 3 patients with typical GCS. All 3 patients showed serologic evidence of an acute EBV infection. The immunohistochemical studies included monoclonal antibodies for CD3, CD4, CD8, CD20, TIA, S-100 protein, KP-1, EBV latent membrane antigen-1, and EBV-encoded nuclear antigen-2. RESULTS: All biopsy specimens showed minimal epidermal spongiosis with marked papillary dermal edema. The associated inflammatory infiltrate showed a mixed mononuclear cell infiltrate with rare eosinophils. Immunohistochemical stains for latent membrane antigen-1 and EBV-encoded nuclear antigen-2 were negative for EBV. The majority of mononuclear cells showed membrane staining for CD3, 30% to 40% of the CD3 mononuclear cells showed positive staining for CD4, and 50% to 60% showed positive staining with CD8. TIA(+) cells appeared to correspond to the CD8(+) cells. CONCLUSION: Although papillary dermal edema has been reported within the spectrum of histologic findings in GCS, it was marked and a consistent finding in the 3 cases in which EBV was the most likely etiologic agent. The presence of large numbers of cytotoxic T cells in the inflammatory infiltrate may have accentuated this histologic finding and may be a relatively distinctive histologic finding with GCS associated with EBV.

Acrodermatitis↗
PubMed2004

Borrelia-specific interferon-gamma and interleukin-4 secretion in cerebrospinal fluid and blood during Lyme borreliosis in humans: association with clinical outcome.

The Borrelia-specific interferon (IFN)- gamma and interleukin (IL)-4 responses of 113 patients and control subjects were analyzed using the sensitive enzyme-linked immunospot method. Cerebrospinal fluid (CSF) and blood samples were obtained, during the course of disease, from patients with chronic or nonchronic neuroborreliosis (NB) and from control subjects without NB. Blood samples were obtained from patients with Lyme skin manifestations and from healthy blood donors. Early increased secretion of Borrelia-specific IFN- gamma (P<.05) and subsequent up-regulation of IL-4 (P<.05) were detected in the CSF cells of patients with nonchronic NB. In contrast, persistent Borrelia-specific IFN- gamma responses were observed in the CSF cells of patients with chronic NB (P<.05). In patients with erythema migrans, increased IFN- gamma (P<.001) was observed in blood samples obtained early during the course of disease, whereas increased IL-4 (P<.05) was observed after clearance. On the contrary, patients with acrodermatitis chronica atrophicans had Borrelia-specific IFN- gamma (P<.001), but not IL-4, detected in blood samples. The present data suggest that an initial IFN- gamma response, followed by up-regulation of IL-4, is associated with nonchronic manifestations, whereas a persistent IFN- gamma response may lead to chronic Lyme borreliosis.

Acrodermatitis↗
PubMed1997

Why is chronic Lyme borreliosis chronic?

Chronic Lyme borreliosis (CLB) can present not only in different organs but also in different patterns. Although many theories exist about the mechanisms leading to CLB, it is known that viable Borrelia burgdorferi can persist for decades and cause late skin manifestations of acrodermatitis chronica atrophicans (ACA). Thus, the immunopathogenetic findings in ACA can serve as a model for studying the chronic course of Lyme borreliosis. Recent findings indicate that the most important cell for antigen presentation, the epidermal Langerhans cell (LC), is invaded by B. burgdorferi in early Lyme borreliosis. Therefore, LCs were stained immunohistochemically with different markers to investigate their functional activity. Numbers of CD1a+ LCs were reduced in erythema migrans but normal or slightly elevated in ACA. In both diseases there was also a marked downregulation of major histocompatibility complex class II molecules on LCs, as measured by staining of human leukocyte antigen DR. This phenomenon might be a mechanism that protects against the presentation of autoantigens and may be the cause of the impaired capacity of LCs to eliminate B. burgdorferi antigens, thus explaining why CLB is chronic.

Acrodermatitis↗
PubMed1976

Zinc deficiency in two infants during total parenteral alimentation for diarrhea.

Zinc deficiency was observed in two infants receiving total parenteral alimentation for chronic diarrhea. An acrodermatitis enteropathica-like rash occurred in both of the infants. Staphylococcus aureus was cultured from the rash. Treatment with zinc resulted in rapid cure of the rash and a subsidence of other signs consistent with zinc deficiency.

Acrodermatitis↗
PubMed1982

Serum alkaline phosphatase and zinc undernutrition in infants with chronic diarrhea.

III infants and children need zinc replacement in total parenteral nutrition solutions, but assessment of these needs and total body zinc status is difficult. Seven infants with severe diarrhea initially given 80 to 100 micrograms/kg/day of elemental zinc developed systemic zinc deficiency as indicated by an acrodermatitis-like skin rash and low serum alkaline phosphatase. Serum zinc levels were borderline low only in conjunction with hypoalbuminemia. Daily urinary zinc excretion was normal. With increased zinc supplementation of 200 to 300 micrograms/kg/day, the rash healed and serum alkaline phosphatase rose to normal levels for age. The activity of the metalloenzyme alkaline phosphatase accurately reflects total body zinc status in infants. With diarrheal illness, infants, need high doses zinc supplementation to replace considerable stool losses.

Acrodermatitis↗
PubMed1993

Different genospecies of Borrelia burgdorferi are associated with distinct clinical manifestations of Lyme borreliosis.

Borrelia burgdorferi sensu lato has been subdivided into three genospecies: B. burgdorferi sensu stricto, B. garinii, and B. burgdorferi group VS461. Sixty-eight isolates cultured from patients and 26 strains from ticks were characterized with use of SDS-PAGE, western blotting, and rRNA gene restriction analysis. Fifty-seven of 58 strains obtained from the skin of 70 patients who had erythema migrams or acrodermatitis chronica atrophicans were of group VS461, whereas the genotype of the remaining strain was unidentifiable. Of 10 strains cultured from CSF (n = 3) and skin (n = 7) of 20 patients with extracutaneous symptoms of Lyme borreliosis, nine were B. garinii and one was B. burgdorferi sensu stricto. Of these 20 patients, 17 had neuroborreliosis, one had arthritis and carditis, one had myalgia, and one had erythema and arthralgia. All 26 isolates from ticks were of group VS461. In conclusion, infections due to group VS461 and B. garinii are associated with cutaneous and extracutaneous symptoms, respectively. Our findings suggest that B. burgdorferi genotypes have different pathogenic potentials.

Acrodermatitis↗
PubMed1981

Zinc absorption in cattle with a dietary picolinic acid supplement.

The effect of picolinic acid and hydroxyquinolines in intestinal zinc absorption of cattle was studied. In contrast to reports of the absorption in rat and in man, picolinic acid did not enhance zinc absorption in cattle. In two calves suffering from Adema disease, the bovine parallel to Acrodermatitis enteropathica, hydroxyquinoline supplementation resulted in an increase in intestinal 65Zn absorption, while picolinic acid had no such effect. The results may indicate a difference in the zinc absorption mechanism between cattle and other species.

Acrodermatitis↗
PubMed1994

Nail matrix hypergranulosis.

Nail pathology shares some common features with skin pathology, but it also has its own peculiar aspects. The anatomical and physiological characteristics of the nail unit probably play a major role in determining these pathological differences. Although the presence of keratohyaline granules is a normal feature of the skin, there is no granular layer in the normal nail matrix. As a consequence, nail matrix hypergranulosis should be considered a separate entity from skin hypergranulosis. In our review of 150 longitudinal nail biopsy specimens, keratohyaline granules were seen in the nail matrix of 24 cases of lichen planus, 29 cases of spongiotic trachyonychia, 10 cases of psoriasis, and three cases of Hallopeau acrodermatitis. In all cases, the presence of keratohyaline granules was associated with the absence of the normal keratogenous zone. Similar nail matrix features were detectable in three cases of malignant melanoma, two cases of primary systemic amyloidosis, and one case of histiocytoid hemangioma compressing the nail matrix. Our data suggest that inflammatory and compressive insults to the nail matrix cause both disappearance of the keratogenous zone and matrix keratinization with the formation of keratohyaline granules. Skin hypergranulosis reflects a hyperplasia of a normal skin component. In the nail matrix, however, hypergranulosis represents the appearance of structures not normally present. Nail matrix hypergranulosis should be considered a pattern of nail matrix reaction to different inflammatory insults. It is therefore more analogous to epidermal parakeratosis than to epidermal hypergranulosis.

Acrodermatitis↗
PubMed1996

Heterogeneity of Borrelia burgdorferi in the skin.

The reliability of various in vitro techniques to identify Borrelia burgdorferi infection is still unsatisfactory. Using a high-power resolution videomicroscope and staining with the borrelia genus-specific monoclonal flagellar antibody H9724, we identified borrelial structures in skin biopsies of erythema chronicum migrans (from which borrelia later was cultured), of acrodermatitis chronica atrophicans, and of morphea. In addition to typical borreliae, we noted stained structures of varying shapes identical to borreliae found in a "borrelia-injected skin" model; identical to agar-embedded borreliae; and identical to cultured borreliae following exposure to hyperimmune sera and/or antibiotics. We conclude that the H9724-reactive structures represent various forms of B. burgdorferi rather than staining artifacts. These "atypical" forms of B. burgdorferi may represent in vivo morphologic variants of this bacterium.

Acrodermatitis↗
PubMed2000

Gianotti-Crosti syndrome presenting as lichenoid dermatitis.

Papular acrodermatitis of childhood (Gianotti-Crosti syndrome) is an uncommon, self-limited disease characterized by an erythematous papular eruption symmetrically distributed on the face and limbs and mild lymphadenopathy, thought to be of viral origin. The histopathologic findings are nonspecific and include focal parakeratosis, mild spongiosis, superficial perivascular infiltrate, papillary dermal edema, and extravasated red blood cells. Interface changes with some basal vacuolization may be present, but are not a conspicuous feature. We present a 2 1/2-year-old boy with multiple papules and plaques on the face and extremities and cervical lymphadenopathy. Histopathologic analysis showed compact orthokeratosis, focal parakeratosis, hypergranulosis, psoriasiform epidermal hyperplasia, and a dense lichenoid lymphohistiocytic infiltrate with extensive exocytosis of mononuclear cells. Immunoperoxidase staining with CD 1 a revealed clusters of Langerhans cells in the epidermis and in the papillary dermis. In view of the clinical findings, a diagnosis of Gianotti-Crosti syndrome was made. Although there are a few reports describing a lichenoid pattern of infiltration in Gianotti-Crosti syndrome, this histologic pattern is not widely known. This case is presented to illustrate the fact that Gianotti-Crosti syndrome can present as lichenoid dermatitis, and, especially in children, should be added to the differential diagnoses of lichenoid infiltrates.

Acrodermatitis↗
PubMed1982

Morphometric study of the jejunal mucosa in various childhood enteropathies with special reference to intraepithelial lymphocytes.

A morphometric study of intraepithelial (IE) lymphocytes per 100 epithelial cells, villous heights (VH), crypt depths (CrD), and epithelial cell heights (ECH) was made on jejunal specimens of 17 patients with cow's-milk allergy (CMA), 52 with celiac disease (CD), seven with congenital lactase deficiency (CLD), four with acrodermatitis enteropathica (AE), four with giardiasis, and four with dermatitis herpetiformis (DH). The aim of this study was to investigate how the morphometric parameters correlate with each other. All cases with CMA, CD, and DH had villous atrophy with hyperplasia of the crypts, both signs being more severe in cases with CD and DH than with CMA. IE lymphocyte infiltration was more intense in specimens of patients with CD and DH (mean 76.0), than those with CMA (mean 62.5). The ECH were equally reduced in patients with CD and CMA. In a follow-up specimen at 1 year and 10 months for CD patients and 11 months for CMA patients the inflammation was reduced, and the VH were increased but still differed from the controls. In CLD cases the morphology of the villi and crypts of the jejunum was quite normal, with no IE lymphocyte infiltration; ECH were reduced. Minor morphological changes were seen in the specimens of patients with AE and giardiasis. In the whole study group there was a significant linear correlation, either positive or negative, between all variables measured (IE lymphocytes, VH, CrD, and ECH).

Acrodermatitis↗