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KID syndrome - Keratitis Ichthyosis Deafness (Pathology Acronym)
( Keratitis-ichthyosis-deafness ) is a rare congenital disorder
characterized by lesions involving cornea, epidermis and internal ear.
In 1981 Skinner
et al introduced the term
It is a rare genodermatosis associated with mutations in the connexin 26 gene.
KID syndrome predisposes to various cutaneous lesions both infectious and neoplastic in nature.
Chronic mucocutaneous candidiasis, benign tumours like trichilemmal tumors and malignant tumour like squamous cell carcinoma of both mucosa and skin, at the acral sites have been reported.
K = Keratitis -
Vascularizing keratitis leading to visual impairment.
[ The ocular signs include loss of eyebrows and lashes, thickened and keratinized lids, recurrent corneal epithelial defects, superficial and deep corneal stromal vascularization with scarring and keratoconjunctivitis sicca.]
I = Ichthyosis -
Affected patients have a distinctive ichthyosis characterized by a fine dry scale.
There may be diffuse hyperkeratosis, keratotic plaques, reticulated pattern of hyperkeratosis on the face, the palms and soles, peribuccal grooves, and heavy-grain leather-like keratoderma.
D = Deafness -
Treatments with antibiotics, antifungals, and systemic retinoids have been reported with variable response.
Do your kids a favor - don't have any. - Robert Orben
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