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Query: UMLS:C0022568 (keratitis)
 5,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a male patient suffering from a congenital ichthyosiform dermatosis, whose family history offered no clue to the diagnosis. The skin lesions were mainly localized on the trunk, hands, elbows, and knees. During the last few years, the cutaneous lesions had slowly changed for the worse and the following additional symptoms had developed: keratitis, deafness, diffuse alopecia, dysarthria, and ataxic abasia. According to the clinical features, we established the diagnosis of KID syndrome (keratitis, ichthyosis, deafness). Although the skin lesions of our patient clinically and histologically belong to the group of atypical erythrokeratodermias, the term "KID syndrome" recently described by Skinner represents an useful, unifying clinical term.
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PMID:[Atypical erythrokeratodermia with deafness, keratitis and double mycotic infection]. 323 40

The KID syndrome is characterized by congenital ichthyosis, vascular keratitis and neurosensorial deafness. We report a 17 year old female patient, the first case of KID syndrome in Spanish literature. Red, dry, scaling skin was present at birth with sparse hair. At the age of six, malar erythema was prominent, with perioral ragades and onset of progressive neurosensory deafness. At the age of ten, vascularizing keratitis developed. At 12, treatment with etretinate failed to improve the ichthyosis. We review the clinical, pathological and analytical features of KID syndrome and discuss its relationship to other ichthyoses.
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PMID:[KID syndrome (keratitis-ichthyosis-deafness)]. 331 67

Neurological studies were performed in a young boy with keratitis, ichthyosis and deafness syndrome. Skin biopsy showed features of ichthyosis. Clinically and electrophysiologically, he had normal motor and sensory systems, but there was an acoustic nerve lesion and absence of tendon reflexes. Audiometry and brainstem auditory evoked potentials showed bilateral neurosensory deafness. Poor vision may not be due to an optic nerve lesion as evidenced on visual evoked potential findings, but is probably due to pronounced vascularizing keratitis of the cornea. Computer tomography scan showed mild hypoplasia of the inferior vermis and left side of the cerebellum.
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PMID:Clinical and electrophysiological studies in a patient with keratitis, ichthyosis and deafness (KID) syndrome. 355 95

A father and daughter had typical clinical features of the keratitis, ichthyosis, and deafness (KID) syndrome, as described by Skinner et al in 1981. To our knowledge, ours is the first observation of a vertical transmission of this syndrome. The mechanism of inheritance is uncertain. These two patients as well as the 26 previously described exhibited a typical hyperkeratotic eruption, which should not be confused with ichthyosis. The characteristic features are diffuse hyperkeratosis, keratotic plaques, reticulated hyperkeratosis on the face, peribuccal grooves, and heavy-grain leatherlike keratoderma. The occurrence of multiple squamous cell carcinomas underlines the seriousness of this congenital ectodermal defect. Etretinate can relieve the lesions without actually improving the chances of survival.
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PMID:Keratitis, ichthyosis, and deafness (KID) syndrome. Vertical transmission and death from multiple squamous cell carcinomas. 357 58

The first case of invasive squamous cell carcinoma (SCC) arising in the skin of a patient afflicted with keratitis, ichthyosis, and deafness (KID) syndrome is reported. A 35-year-old man, diagnosed as having KID syndrome in early childhood, developed bilateral fungating lesions on his feet. The entire left foot became involved with a multinodular fungating mass which proved to harbor a SCC, necessitating a left below-knee amputation. Although rare, KID syndrome can be associated with SCC of the skin.
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PMID:Squamous cell carcinoma in congenital ichthyosis with deafness and keratitis. A case report and review of the literature. 395 9

We treated two unrelated boys with ichthyosis follicularis, a rare skin disorder characterized by extensive noninflammatory spiny follicular hyperkeratoses, severe photophobia, and generalized noncicatricial alopecia. This disorder must be differentiated from keratosis follicularis spinulosa decalvans; ulerythema ophryogenes; keratosis pilaris rubra atrophicans faciei; atrichia with papular lesions; atrophodermia vermiculata; and keratitis, ichthyosis, and deafness syndrome, all of which share some clinical features. Ichthyosis follicularis with alopecia and photophobia appears to be a familial disorder, but too few cases have been reported to establish the exact mode of inheritance.
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PMID:Ichthyosis follicularis with alopecia and photophobia. 403 43

In 1981 Skinner et al (1) proposed the term KID syndrome for a previously disparate group of congenital disorders principally made up of keratitis (K), ichthyosis (I), and deafness (D). The coexistence of congenital deafness associated with ichthyosis had been previously recognized (2,3), yet similarities among affected patients were overlooked because of both the paucity of cases and their seeming diversity. The patient reported by Skinner is similar to one whom we have been following at the Clinique Universitaire de Dermatologie in Geneva since 1978. After having reviewed the literature, we believe that KID syndrome is a useful, unifying clinical term.
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PMID:KID syndrome (keratitis, ichthyosis, and deafness) and chronic mucocutaneous candidiasis: case report and review of the literature. 639 Mar 93

We describe here a patient with ichthyosis, keratitis, deafness, and recalcitrant cutaneous bacterial and fungal infections, who was previously described as having hereditary hypohidrotic ectodermal dysplasia. Similarly affected patients described in the literature have the following features in common: (1) a distinctive ichthyosis characterized by a fine dry scale, follicular hyperkeratotic spines, and a reticulated pattern of hyperkeratosis on the palms and soles; (2) a vascularizing keratitis that results in notable visual impairment; and (3) neurosensory deafness. One half of the affected patients also display frequent, severe cutaneous infections. We suggest that the name "the KID syndrome" to emphasize the characteristic features of the syndrome-keratitis, ichthyosis, and deafness.
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PMID:The keratitis, ichthyosis, and deafness (KID) syndrome. 722 57

A patient with the keratitis, ichthyosis and deafness (KID) syndrome is described. The patient had recurrent skin infections which led to complete scalp hair loss. The nails were dystrophic. Physical development was normal; however, his intelligence was subnormal. The erythrokeratodermatous plaques over the face, trunk and extremities were characteristic and the skin in general was dry and hyperkeratotic. Palms and soles showed marked thickening with a stippled appearance.
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PMID:Keratitis, ichthyosis, deafness (KID) syndrome--the first report from India. 803 1

We report a case of KID (Keratitis, Ichthyosis, and Deafness) syndrome in which the patient died at the age of 2 months. Detailed histological study of the affected organs, particularly the inner ear and external auditory meatus, was performed. This case is the first in which previously described principles relating to the biology of the ear canal epithelium have been applied to a patient with skin disease affecting this area.
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PMID:Fatal keratitis ichthyosis and deafness syndrome (KIDS). Aural, ocular, and cutaneous histopathology. 843 34


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