Cases reported "Keratosis"

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11/21. Familial dyskeratotic comedones. A report of three cases and review of the literature.

    Three family members, at initial evaluation, had generalized comedonal lesions with histologic changes of acantholysis and dyskeratosis. A total of nine cases of this entity, termed familial dyskeratotic comedones, have been documented in the literature. It appears to have autosomal dominant inheritance and onset in childhood or adolescence. Lesions are asymptomatic except for occasional pruritus or inflammation, and general health is undisturbed. A history of acne vulgaris is seen in four of nine patients and is the only associated skin disease. Treatment with oral isotretinoin produced no improvement in two patients. Electron microscopy revealed changes similar to those seen in Darier's disease.
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12/21. Paraneoplastic acrokeratosis: Bazex syndrome.

    Paraneoplastic acrokeratosis, or Bazex syndrome, is a rare, distinct dermatosis characterized by psoriasiform acral hyperkeratosis. In most cases, it is a specific cutaneous sign of an occult squamous cell carcinoma of the upper aerodigestive tract that has metastasized to cervical lymph nodes. We report the fifth American case of paraneoplastic acrokeratosis. The patient's skin disease was more remarkable for its hyperpigmentation than its hyperkeratosis. Nearly all of the hyperpigmentation resolved, but the nail dystrophy persisted seven months after the tumor had been treated using surgery and radiation.
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13/21. Disseminated epidermolytic acanthoma.

    A 65-year-old Japanese male suffered from numerous brownish papules on his back for 6 months. Clinical appearance resembled seborrheic keratosis, but light microscopic examination revealed epidermolytic hyperkeratosis. Electron findings were the same as those of epidermolytic hyperkeratosis and systemic nevus verrucosus. It may be concluded that this type of skin disease is an acquired epidermolytic hyperkeratosis.
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14/21. Familial elastosis perforans serpiginosa.

    BACKGROUND--Elastosis perforans serpiginosa (EPS) is an uncommon skin disease characterized by transepidermal elimination of abnormal elastic fibers. The disease is frequently associated with congenital connective tissue disorders or Down's syndrome. The pathogenesis of EPS is still unclear. There are a few reports in the literature about a familial occurrence of EPS in which different modes of inheritance are suggested. To support the hypothesis of a congenital origin of the disease, we have studied another family with EPS. OBSERVATIONS--In this study, we describe a family in which two sisters and a brother were affected by EPS. The father and three paternal uncles were most probably affected by the same disease. There were no signs of other congenital connective tissue disease in the family members. CONCLUSION--An autosomal dominant mode of inheritance with variable expression of EPS is suggested.
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15/21. Changes of verruciform xanthoma in an hiv-1 patient with diffuse psoriasiform skin disease.

    Verruciform xanthoma occurs most commonly in the oral mucosa; however, rare cutaneous lesions have been described. Although the pathogenesis of this entity is not known, dysregulation of epithelial proliferation and degenerative changes in the epithelium may explain the occurrence of this lesion in association with inflammatory dermatoses, epithelial hamartomas, and epithelial dysplasia. We report an hiv-1 patient with diffuse psoriasiform skin lesions that showed histologic changes of verruciform xanthoma.
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16/21. Kindler syndrome. Clinical and ultrastructural findings.

    BACKGROUND: Kindler syndrome is a genodermatosis that combines clinical features of hereditary epidermolysis bullosa and poikiloderma congenitale. The ultrastructural level of blister formation has not been well characterized. OBSERVATIONS: Two brothers with Kindler syndrome had a history of primarily acral blistering since infancy as well as photosensitivity. blister formation was found through the basal layer. Marked tonofilament clumping was found in intact keratinocytes adjacent to the blisters. The younger brother (aged 21 years) had actinic keratoses, which have not been previously described in Kindler syndrome. CONCLUSIONS: The findings of basal layer separation in both spontaneous and induced blisters in Kindler syndrome suggest this is the true level of blister formation. The finding of actinic keratoses in a young patient with Kindler syndrome suggests that some patients may be at increased risk for early solar-induced skin disease. The presence of clumped tonofilaments in keratinocytes adjacent to blistered areas suggests an abnormality of keratin 5 or 14 could be present and may play a role in blister formation in patients with Kindler syndrome.
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17/21. keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK-syndrome): a rare, autosomal recessive disorder of keratohyaline formation?

    We report a 32-year-old man with an unusual combination of congenital ichthyosis, sclerosing palmoplantar keratoderma with pseudoainhum, and bizarre striate hyperkeratosis in the fixtures, but no systemic involvement. The condition, which improved on oral etretinate therapy, had not appeared previously in the family. On light microscopy the involved epidermis showed marked acanthosis with hypergranulosis and hyperkeratosis. Electron microscopy disclosed numerous large keratohyaline granules in superficial keratinocytes. The clinical picture and histology are virtually identical to those of a Spanish family suffering from an autosomally recessive skin disease of unknown etiology. We hypothesize that the condition is due to a genetic defect in the formation of keratohyaline granules.
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18/21. Clear cell papulosis: case report and literature review.

    Clear cell papulosis is a newly described skin disease characterized by multiple white papules. Histopathologically, diagnostic clear cells were seen among the basal cells of the epidermis. We report clear cell papulosis on the lumbar area and buttocks of a 1-year-old girl.
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19/21. Hyde's prurigo nodularis and chronic HCV hepatitis.

    The authors describe a woman with chronic active hepatitis, Hyde's prurigo nodularis and hepatitis c virus infection. The association of these three pathologies and their parallel evolution during treatment suggest a possible pathogenic link between the chronic hepatitis c virus infection and the skin disease.
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20/21. Detection of new human papillomavirus sequences in skin lesions of a renal transplant recipient and characterization of one complete genome related to epidermodysplasia verruciformis-associated types.

    Human papillomavirus (HPV) dna, originally isolated from patients suffering from the skin disease epidermodysplasia verruciformis (EV), and a growing number of related sequences have recently been detected in a high percentage of benign and malignant skin lesions of both immunosuppressed and immunocompetent people. HPV L1 dna fragments (374-389 bp long) from a solar keratosis and a squamous cell carcinoma (SCC) of a renal transplant recipient were amplified, cloned and sequenced. In 54 clones, six different HPV sequences were identified. One of these six corresponded to the known type HPV-8 and two (RTRX3 and RTRX7) have been described previously in cutaneous lesions of immunosuppressed patients. The remaining three sequences were different from all known HPV types: an HPV-9-related sequence (77.4% identity), an RTRX2-related sequence (82.6% identity), and an HPV-22-related sequence (83.7% identity). These three sequences, representing putatively new HPV types, were named RTRX8, RTRX9 and RTRX10, respectively. RTRX7 was found in the majority of clones from both lesions. The complete genome of RTRX7 (7731 bp) was cloned as six overlapping subgenomic fragments, generated by nested PCR with dna extracts from the SCC. RTRX7 showed a genome organization typical of HPVs associated with EV. The L1 dna sequence differed by 15% from the corresponding region of its closest known relative, HPV-12; thus, RTRX7 can be regarded as a new HPV type. RTRX7 dna could not be detected by Southern blot hybridization with the homologous probe, indicating that the dna concentration was below one copy per 10 cells in the investigated SCC.
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