Keratitis, Ichthyosis, and Deafness (KID Syndrome): Review of the Literature and Proposal of a New Terminology

Abstract

The so‐called KID (keratitis, ichthyosis, deafness) syndrome is a congenital disorder of ectoderm that affects not only the epidermis, but also other ectodermal tissues such as the corneal epithelium and the inner ear. Sixty‐one patients who fulfill the criteria for this syndrome were identified in a review of the literature through December 1993. All had cutaneous and auditory abnormalities, and 95% also had ophthalmologic defects. The most frequent clinical features were neurosensory deafness 90%, erythrokeratoderma 89%, vascularizing keratitis 79%, alopecia 79%, and reticulated hyperkeratosis of the palms and soles 41%. All of these findings constitute the major criteria for the diagnosis. The KID acronym does not accurately define this entity since the disorder is not an ichthyosis, because scaling is not the main cutaneous feature and not all patients have keratitis early in the course. We suggest that this syndrome should be included under the general heading of congenital ectodermal defects as a keratodermatous ectodermal dysplasia (KED).

Você conhece esta síndrome?

“…The lesions often appear in the form of wart-like or hyperkeratotic plaques of erythrokeratodermia, located symmetrically on the scalp, face, and skin folds. 3 The presence of generalized xerosis, keratosis pilaris and hypohidrosis is common. In 79% of cases there may be diffuse alopecia of the scalp as well as atrichia and partial loss of lateral ends of the eyebrows.…”

Uso do bevacizumab (Avastin®) na síndrome KID: relato de caso

“…As manifestações oculares da síndrome KID ocorrem geralmente na infância, após o surgimento das alterações cutâneas e auditivas. Portanto, o acrônimo KID pode não estar completo nas fases precoces da doença (7) . O mecanismo da doença corneana não parece ser inflamatório, como implica o termo "ceratite", mas resultante de um distúrbio ectodérmico generalizado, com atrofia ou ausência das glândulas de Meibomius, queratinização dos ductos das glândulas lacrimais e deficiência de células basais limbares.…”

Congenital atrichia and hypotrichosis

“…In a classic review, [17] 61 patients who met the criteria for this syndrome were identified. All had cutaneous and auditory abnormalities, and 95% of them also had ophthalmologic defects.…”