Cutaneous Vegetating Candidiasis in Keratitis‐Ichthyosis‐Deafness Syndrome

A school-age adolescent with keratitis-ichthyosis-deafness (KID) syndrome, harboring a germline GJB2 variant (c.263C>T), presented with generalized progressive erythrokeratoderma and palmoplantar hyperkeratosis. Examination revealed a foul-smelling, oozing, tumor-like vegetative lesion on the left buttock (Figure 1a), significantly impairing quality of life. Skin biopsy ruled out malignancy and showed verruciform epidermal hyperplasia with pseudohyphae (Figure 1b), highlighted by periodic acid–Schiff staining (Figure 1c). Fungal culture and antifungal susceptibility test identified fluconazole-resistant Candida albicans. Given repeated treatment failures with fluconazole, itraconazole, and oral acitretin, alongside concerns for malignant transformation, the lesion was surgically excised and reconstructed with a split-thickness skin graft. Healing was complete and recurrence-free after 1 year (Figure 1d). KID syndrome is a rare ectodermal dysplasia characterized by vascularizing keratitis, sensorineural hearing loss, and erythrokeratoderma [1]. It is primarily caused by heterozygous pathogenic variants in the GJB2 gene, which encodes connexin 26, leading to impaired gap junction communication and dysregulated epidermal differentiation and keratinization. Heterogeneous cutaneous findings include tumor predisposition, notably for squamous cell carcinoma (SCC), and recurrent infections [2]. Additional features include partial alopecia, abnormal nails, and benign adnexal tumors such as trichilemmal cysts. Superinfections, particularly mucocutaneous candidiasis, are well-recognized complications in KID syndrome [3] and can exacerbate dermatologic symptoms, often leading to therapeutic resistance. The verrucous and fungating morphology warrants a broad differential diagnosis that includes SCC, verrucous carcinoma, keratoacanthoma, deep fungal infections, mycobacterial infections, and syphilis. The precise mechanism of vegetating candidiasis in this context remains unclear but may involve skin barrier dysfunction or immune dysregulation [4]. Management is multidisciplinary and primarily symptomatic, focusing on emollients, keratolytics, and systemic retinoids. Antimicrobial therapy is indicated in suspected or confirmed infections. However, existing treatments exhibit variable efficacy and significant adverse effects and lack disease-modifying properties. Future therapeutic approaches, including hemichannel inhibitors and gene-based therapies [5], show promise in early studies but remain investigational. The authors have nothing to report. The research protocol was approved by an institutional reviewer board. Written informed consent has been obtained from the patient for the use of their image for scientific and educational purposes. The authors declare no conflicts of interest. The data that support the findings of this study are available from the corresponding author upon reasonable request.