Severe ichthyosis vulgaris with vitamin D insufficiency and multiple flexion contractures

Abstract

Ichthyosis vulgaris (IV) is a non-syndromic form of congenital ichthyoses and mainly affects the skin, moreover in severe case followed by joints involvement and vitamin D imbalance as chronic complication due to skin barrier impairment. A 16-years-old female presented with scaly skin all over her body. Changes on her hands appeared when she was 8-years-old and several years later gradually she could not use her hands. Dermatological examination obtained thick scales, xerotic skin, and palmo-plantar hyperkeratosis. The dermoscopic finding was a fine white scale with a criss-cross pattern. Her skin biopsy showed hyperkeratosis and thinning of granular layer, meanwhile laboratory results indicated vitamin D insufficiency. Hand x-ray revealed flexion contractures. She received oral vitamin D3 400 IU once daily, calcitriol 0.5 mcg twice daily, and topical emolients. Surgical contracture release was conducted to repair the joints deformity. Manifestation of IV is limited to the skin and rarely accompanied by other systemic disorders. Joint anomaly might be seen in some syndromes accompined with ichthyoses, however IV does not lead to joint contractures. Skin thickness and impaired barrier could lead to reduced ultraviolet B penetration, so that it disturbed and diminished vitamin D synthesis. Those conditions are more likely to cause muscle weakness and atrophy which finally lead to joints contractures.