Dactylolysis Spontanea: Possible Immunohistologic Etiology Discovered in a Rare Presentation of Bilateral Fourth Digits

Abstract

Dactylolysis spontanea is a rare, progressive disease characterized by fibrous soft-tissue constriction around the base of an appendage. It most commonly occurs bilaterally in the fifth toes of male patients and often progresses to spontaneous autoamputation. The broad spectrum of clinical severity, poorly characterized natural clinical timeline, and previous lack of specific confirmatory tests make this condition difficult to diagnose and treat in the early stages where it may be amendable to conservative treatment. We present a case report of a 29-year-old woman with dactylolysis spontanea involving bilateral fourth digits. The diagnosis of dactylolysis spontanea was made based on clinical and radiographic correlations and by excluding other similar conditions. Following surgical removal of the digits, exploratory histopathologic analysis demonstrated the novel presence of positive glucose transporter 1 immunohistochemical staining. Although the diagnosis of dactylolysis spontanea has historically been considered a diagnosis of exclusion and is often complicated by the heterogeneous clinical presentations, glucose transporter 1 staining offers clinicians a valuable tool in assisting with the diagnosis of this condition. This may finally elucidate the etiology of this rare condition.