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Background. KID syndrome (keratitis-ichthyosis-deafness) is an orphan genetic multisystem disease with autosomal recessive and dominant types of inheritance, it manifests in the neonatal period. The leading triad of symptoms is: skin lesions, ophthalmological diseases and hearing organ pathology. Clinical Case Description. Girl V., 17 years old, with KID syndrome applied to the hospital complaining on painful infiltrates of the parietal region. Multiple abscesses were lanced. Hyperkeratotic crusts were removed, unviable skin regions were excised, and abscesses' cavities were washed with antiseptic solution during daily dressings. Purulent discharge from wounds has been maintaining for 7 days. Conclusion. Where is no pathogenetic treatment for KID syndrome yet. Prevention of secondary surgical infections remains the crucial aspect in the management of such patients. Local wound treatment, symptomatic and antibacterial therapy are effective in case of skin infection.

The paper presents a rare clinical case of an infant with KID (Keratitis, Ichthyosis, Deafness) syndrome (about 100 patients reported so far) admitted for histological verification of oral mucosa lesions. Disease pathogenesis defines inadequate reparation and skin and mucosa innate immunity defect leading to higher incidence of bacterial and fungal infections, so the 4-years old girl received treatment for vegetating candidiasis of the oral mucosa for several weeks with no clinical improvement. Initial examination showed that the oral lesions resulted from sharp edges of severely affected carious teeth. Histological study of multifocal biopsy revealed pyogenic granulomas and no signs of SCC. Teeth extraction and symptomatic treatment leaded to significant clinical improvement and some remained mucosal changes may be attributed to syndrome manifestations.