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1/6. Xanthoma secondary to reticulo-histiocyte infiltration.

    Three cases of xanthomas occurring secondarily are reported: The primary dermatoses were erythroderma, actinic reticuloid and histiocytosis X. Although a slight elevation of pre-beta-lipoprotein was recognized, xanthomas occurring in these particular 3 cases were assumed to be due to the proliferation or hyperplasia of reticulohistiocytes in the skin. The foam cells found in the xanthoma case with erythroderma and actinic reticuloid appeared different from those with histiocytosis X in their fine structures. The lipid constituents of the xanthoma lesion were found to be quite dissimilar from those of serum lipids.
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ranking = 1
keywords = erythroderma
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2/6. methotrexate induced false photosensitivity reaction.

    methotrexate is now being used in the treatment of a variety of intractable rheumatologic conditions. We report a patient with polymyositis who developed mild erythroderma after sun exposure. Forty-eight h later he received his weekly dose of methotrexate. Within 24 h the resolving rash erupted into an intense dusky red dermatitis with eventual formation of confluent bullae. The patient had a methotrexate induced ultraviolet reactivation reaction also known as a "false photosensitivity" reaction. This reaction has been reported in dermatologic publications but not in the rheumatologic literature.
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ranking = 0.5
keywords = erythroderma
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3/6. deafness, ichthyosiform erythroderma, corneal involvement, photophobia and dental dysplasia.

    Early childhood deafness, congenital non-bullous ichthyosiform erythroderma, corneal involvement, photophobia, chronic blepharoconjunctivitis, hypotrichosis, anhidrosis, hyperkeratosis of the nails and dental dysplasia were the symptoms observed in the 19-year-old girl described in this paper. The literature comprises reports on 8 patients with this syndrome, which McKusick (1975) listed as no. 24215.
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ranking = 2.5
keywords = erythroderma
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4/6. Development of photosensitivity and an SLE-like syndrome in a patient with psoriasis.

    An unusual case of photosensitive psoriasis and systemic lupus erythematosus-related syndrome was characterized by erythroderma, chronic urticaria, angioneurotic edema, intermittent low-grade fever, and polyarthralgias. Investigation revealed no measurable total hemolytic complement and markedly diminished levels of C4, C2, and C3. Microscopic examination of three skin biopsy sections of sun-exposed skin showed psoriasis. skin biopsy sections of sun-exposed psoriatic plaques and of non-sun-exposed, uninvolved skin (which were stained with fluorescein-tagged anti-IgG, anti-IgM, anti-IgA, and anti-C3) showed granular deposits of IgM and C3 at the dermal-epidermal junction in the sun-exposed plaques, and IgM alone in a granular pattern at the dermal-epidermal junction in uninvolved skin. antibodies to single-stranded but not double-stranded dna were detected in the patient's serum. In addition, serum immune complex-like material was detected by sucrose density-gradient ultracentrifugation, standard anticomplementary assays, and radioimmunoassays using both C1q and monoclonal rheumatoid factor.
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ranking = 0.5
keywords = erythroderma
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5/6. Persistent light reaction with erythroderma caused by musk ambrette: a case report.

    Persistent light reaction is an uncommon type of photodermatitis caused mainly by musk ambrette, a synthetic fragrance material commonly used in foods and cosmetics. Erythrodermic persistent light reaction is rare. We report a case of erythroderma with underlying persistent light reaction due to musk ambrette. A 71-year-old man showed a photodermatitis that waxed and waned for five years before it became more persistent and finally evolved into erythroderma. Positive results of a photopatch test to musk ambrette and a low minimal erythema dose to ultraviolet B were noted. A biopsy specimen of the erythrodermic lesion revealed spongiotic dermatitis. The erythroderma and photodermatitis responded to systemic steroids and psoralen/ultraviolet A therapy (total dose: 90 J/cm2). We suggest that persistent light reaction be included in the differential diagnosis of erythroderma.
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ranking = 4
keywords = erythroderma
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6/6. Photoaccentuated erythroderma associated with CD4 T lymphocytopenia: successful treatment with 5-methoxypsoralen and UVA, interferon alfa-2b, and extracorporeal photopheresis.

    We describe a 53-year-old hiv-negative white man who had chronic CD4 T lymphocytopenia and photoaccentuated erythroderma with lymphoma-like histologic changes. The erythroderma completely responded to 5-methoxypsoralen and UVA (PUVA), interferon alfa-2b, and extracorporeal photopheresis. During therapy opportunistic skin infections, including tinea corporis, warts, and disseminated molluscum contagiosum, developed. Although the patient met the current definition of idiopathic CD4 T lymphocytopenia (ICTL), we cannot rule out the possibility that this peripheral CD4 T lymphocytopenia resulted from sequestration of CD4 T lymphocytes in erythrodermic skin.
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ranking = 3
keywords = erythroderma
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