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KID syndrome -  Keratitis Ichthyosis Deafness (Pathology Acronym)

Dr Sampurna Roy MD

 

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KID syndrome ( 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 KID syndrome.

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 - 

Neurosensory deafness.

 

Treatments with antibiotics, antifungals, and systemic retinoids have been reported with variable response.

 

Further reading:

KID syndrome (keratitis-ichthyosis-deafness).

Keratitis, ichthyosis, and deafness (KID) syndrome in half sibs.

Keratitis, ichthyosis, and deafness syndrome: a review of infectious and neoplastic complications.

Diagnostics of keratitis-ichthyosis-deafness syndrome (KID- syndrome).

KID syndrome (keratitis, ichthyosis and deafness).

Keratitis, ichthyosis, and deafness (KID syndrome): review of the literature and proposal of a new terminology.

Squamous cell carcinoma in congenital ichthyosis with deafness and keratitis. A case report and review of the literature.

Ocular manifestations of keratitis-ichthyosis-deafness (KID) syndrome.

KID syndrome (keratitis, ichthyosis, and deafness) and chronic mucocutaneous candidiasis: case report and review of the literature.

 

              Do your kids a favor - don't have any. - Robert Orben

 

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Dr  Sampurna Roy  MD

Consultant Histopathologist (Kolkata - India)

 

 

 


 

 

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