Squamous cell carcinomas in a girl with ichthyosis and severe malnutrition.

Abstract

Background. Ichthyosis vulgaris (IV) is the most common inherited keratinization disorder, characterized by fine scaling and xerosis with sparing of flexural areas. Although generally benign, chronic barrier dysfunction and inflammation in IV can increase susceptibility to infection and, in rare cases, malignancy. The association between IV and squamous cell carcinoma (SCC) has not been previously reported, particularly in the pediatric population.
Case report. A 9 year old girl with a six year history of generalized dry, scaly, and pruritic skin presented with a foul odor and nonhealing ulcerated lesions on the back and nose. Examination revealed diffuse scaling, fissures, and erythematous plaques with verrucous, ulcerated masses. Dermoscopy showed a criss-cross pattern of fine white scales. Cytologic examination of fine-needle aspiration cytology revealed malignant epithelial cells with spindle morphology, nuclear pleomorphism, coarse chromatin, and eosinophilic cytoplasm, consistent with squamous cell carcinoma. The patient also exhibited severe malnutrition, anemia, and hypoalbuminemia. Management included topical mometasone furoate–fusidic acid combination cream, white petrolatum, 4% salicylic acid, systemic antibiotics, nutritional supplementation, and symptomatic therapy. On follow-up, skin scaling and odor improved, but the tumor remained extensive. Due to the patient’s poor clinical status, palliative management was advised by pediatric oncology, as chemoradiotherapy was not feasible.
Discussion. Malignant transformation in ichthyosis is exceedingly rare and more commonly described in severe congenital subtypes such as lamellar ichthyosis or congenital ichthyosiform erythroderma. The present case suggests that chronic inflammation, recurrent trauma from fissuring, and malnutrition may create a procarcinogenic environment even in milder ichthyotic forms. This case underscores the importance of vigilance for nonhealing, verrucous, or ulcerated lesions in chronic ichthyosis.
Conclusion. This is the first documented case of squamous cell carcinoma arising in a child with ichthyosis vulgaris. Chronic epidermal barrier dysfunction, persistent inflammation, and systemic malnutrition likely contributed to carcinogenesis. Early recognition, histopathologic confirmation, and multidisciplinary management are essential to improve outcomes in this rare but serious complication.