Cases reported "Ichthyosis Vulgaris"

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1/3. Keratotsis punctata of the palmar creases: report of two cases associated with ichthyosis vulgaris.

    Two patients with keratosis punctata of the palmar creases are described. The association with ichthyosis vulgaris and other disorders of keratinization is discussed. In both cases, histopathology revealed a close relation between the keratotic plug and the sweat glands. The role of genetic factors and manual activity in the pathogenesis is discussed. Treatment with oral etretinate resulted in a good improvement in the first patient, but prolonged low-dose maintenance therapy was required to prevent recurrence.
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ranking = 1
keywords = keratosis
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2/3. KID syndrome associated with features of ichthyosis hystrix.

    keratitis-ichthyosis-deafness (KID) syndrome is a congenital ectodermal disorder causing erythrokeratoderma, vascularizing keratitis, and neurosensory deafness. Ichthyosis hystrix is a rare cutaneous disease characterized by well-demarcated, spiky, verrucous, linear plaques that is believed to be a clinical and pathologic chimera of two autosomal dominant diseases: epidermal nevus and epidermolytic hyperkeratosis. We present a patient with the classic triad of KID syndrome with clinical and histologic features of ichthyosis hystrix. This case demonstrates that KID syndrome comprises a spectrum of ectodermal disorders which may include diseases such as hystrix ichthyosis and deafness (HID) syndrome.
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ranking = 1
keywords = keratosis
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3/3. New-onset ichthyosis and diabetes in a 14-year-old.

    Diabetes has not been linked to acquired ichthyosis or ichthyosis vulgaris. We report a newly diagnosed diabetic 14-year-old girl with bilateral tibial and sacral ichthyosiform plaques and a hemoglobin A1c of 20.1%. The patient had no personal or family history of atopy or ichthyosis and lacked keratosis pilaris or hyperlinear palms. A biopsy specimen of an ichthyosiform plaque showed compact lamellar orthohyperkeratosis and hypogranulosis, histopathology consistent with either ichthyosis vulgaris or acquired ichthyosis. We speculate that our patient's new-onset diabetes induced acquired ichthyosis.
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ranking = 2
keywords = keratosis
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