Surgical management of digital ischemia caused by constriction band formation in a patient with ichthyosis vulgaris

Abstract

Ichthyosis is a broad and loosely defined group of hereditary and acquired disorders characterized by filaggrin dysfunction and impaired epidermal homeostasis that results in dry, scaly and thickened skin. Individuals with truncation mutations in the profilaggrin gene coding for filaggrin are strongly predisposed to severe forms of ichthyosis. The phenotypical expression of ichthyosis caused by the same genotypical mutation can vary considerably in severity and in regards to how much constricting scar tissue develops. Here, we report a case of digital ichthyosis threatening the neurovascular integrity to the small finger to discuss the importance of early diagnosis and role of Z-plasty flaps in the surgical treatment of constriction band formation.

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