The keratitis, ichthyosis, and deafness (KID) syndrome

Abstract

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.

Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorèze 2009

“…114,117 Previously, erythrokeratodermia was differentiated from the ichthyosis group as it is not generalized in most cases. However, the majority of the participants thought that the inclusion of EKV into this classification is appropriate and useful and in accordance with the inclusion of KID (keratitiseichthyosisedeafness) syndrome 118,119 (Fig 5, C ), which is identical to ichthyosis hystrix type Rheydt 120 or hystrixlike ichthyosis deafness syndrome. 3 KID syndrome is caused by heterozygous mutations in GJB2 (connexin 26) 121 and patients with congenital presentation in particular have generalized skin involvement.…”

Genodermatosis with Reticulate, Patchy and Mottled Pigmentation of the Neck – A Clue to Rare Dermatologic Disorders

“…In 1981, Skinner et al [11]coined the acronym KID syndrome (MIM 148,210). Traupe [12]suggested redefining the ‘I’ to stand for ichthyosis-like hyperkeratosis.…”

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“…In 1981 Skinner coined the acronym KID, representing the three markers of the disease: keratitis, ichthyosis and deafness. 1 The syndrome has more recently been classified as an autosomal dominant inherited disease affecting the GJB2/GJB6 genes. 2 Skin lesions may be present from birth, with skin looking red, dry and wrinkled.…”