Cases reported "Acrodermatitis"

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1/38. Breaking the relentless course of Hallopeau's acrodermatitis by dapsone.

    We report the case of a 74-year-old man who suffered for four years from recurrent generalized pustular eruptions associated with acrodermatitis continua of Hallopeau (ACH). Initial therapy using topical corticosteroids, tars and PUVA failed to improve the acral lesions. The disease was also unresponsive to systemic therapy using corticosteroids, methotrexate, etretinate, acitretine and cyclosporin A. Serious side-effects were experienced with these drugs. A marked improvement of the generalized pustules was observed under treatment combining etretinate and methotrexate, without having any effect on the acropustulosis. During hospitalization, a topical tar therapy cleared the pustular rash. Again no effect was observed on the acral lesions. Disulone (DDS) therapy, initiated at a dose of 200 mg/day, completely cleared ACH after 4 weeks. The disease relapsed when the dose was reduced to 100 mg/day. Increasing the DDS to 150 mg/day was rapidly followed by a new clearance of the acral lesions. After a 3-month treatment, the patient has remained lesion free and no DDS-related side-effects have occurred. In conclusion, sulfones may be considered as an alternative therapeutic option in drug-resistant ACH.
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2/38. 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.
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3/38. Gianotti-Crosti syndrome in an infant following immunization.

    Gianotti-Crosti syndrome (GCS) is a distinctive, self-limiting acropapular or papulovesicular eruption. Typical clinical presentation is that of a monomorphic, nonpruritic eruption most commonly involving the face, neck, buttocks, and extremities. It tends to occur in childhood, though infrequently it has been reported in adults. We report GCS occurring in an infant following immunization.
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4/38. A case of acrodermatitis enteropathica.

    acrodermatitis enteropathica is a rare hereditary disorder affecting zinc metabolism that is characterized by dermatitis, alopecia, gastrointestinal disturbances, eye infections, and growth failure. We report a 17-month-old girl with acrodermatitis enteropathica. physical examination showed a cutaneous eruption consisting of vesiculobullous and psoriasiform skin lesions symmetrically distributed in the perioral, acral, and perineal areas. Her plasma zinc level was decreased (75 micrograms/dl), but within the normal range (60.00-135.00 micrograms/dl). The patient was given zinc sulfate 50 mg/day. At the end of two months, she had significantly improved.
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5/38. 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.
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6/38. 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.
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7/38. acrodermatitis acidemica secondary to malnutrition in glutaric aciduria type I.

    We encountered a patient with glutaric aciduria type I (GA-I) associated with skin lesions resembling acrodermatitis enteropathica (AE). This child was being fed with a low-protein diet when the skin disorder developed. A deficiency in plasma levels of essential amino acids, particularly isoleucine, and zinc was confirmed. Supplementation of a high-caloric, protein-rich diet together with zinc, selenium and vitamins led to a prompt improvement of the skin lesions. We assume that in our patient the skin lesions were the result of malnutrition, rather than being primarily associated with the underlying metabolic disease. To our knowledge, no other report is so far available concerning GA-I complicated by skin eruptions.
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8/38. Combined vaccination by measles and hepatitis b vaccines: a new cause of Gianotti-Crosti syndrome.

    An 11-month-old boy presented with Gianotti-Crosti syndrome. He had received measles and a third dose of hepatitis b vaccines 2 weeks before the onset of the eruption. There were no clinical symptoms of any viral infection at the presentation. Serological tests for common viral infections were negative. The combination of measles and hepatitis b vaccines was likely the relevant factor in the etiology.
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9/38. acrodermatitis enteropathica-like eruption as the presenting sign of cystic fibrosis--case report and review of the literature.

    In most patients with cystic fibrosis (CF), pulmonary symptoms are the first sign of presentation. Another predominant feature is maldigestion, frequently starting in early childhood. Pancreatic exocrine dysfunction causes maldigestion, characterised by failure to thrive, diarrhoea, hypoproteinaemia, oedema and anaemia. Dermatitis may be the initial manifestation in just a few patients with CF. We report on a patient presenting with refractory dermatitis resembling acrodermatitis enteropathica as the first sign of CF at the age of 4 months, but without pulmonary symptoms. Laboratory findings demonstrated anaemia, hypoproteinaemia and zinc deficiency. Peroral zinc substitution resulted in resolution of the dermatitis within a few days. A further work-up revealed a positive sweat test and homozygosity for the deltaF-580 mutation. CONCLUSION: in rare cases, acrodermatitis enteropathica-like dermatitis is the first symptom of cystic fibrosis.
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10/38. Truncal lesions do not exclude a diagnosis of Gianotti-Crosti syndrome.

    A boy aged 2 years and 10 months had a pruritic eruption and low-grade fever for 1 week. Examination revealed discrete erythematous papules and papulovesicles on his buttocks and extensor surfaces of four limbs. Several papules were also present on his back. No lymphadenopathy and no hepatosplenomegaly were noted. Investigations failed to identify a single viral aetiology. Topical calamine lotion and a sedating oral antihistamine to be taken at night were prescribed. The truncal lesions subsided in 3 weeks. Complete remission of all lesions was seen after 6 weeks. Although most patients with Gianotti-Crosti syndrome only have the typical acrally distributed eruption, additional truncal lesions, if few in number, do not exclude the diagnosis.
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