Dupuytren’s contracture… or is it?

Excerpt

A 90-year-old previously healthy man presented with progressive thickening of the skin of his left hand over the prior 4 months. It had become severe enough to limit movements of the fingers of his hand. He had received localized steroid injections for a presumptive diagnosis of ‘Dupuytren’s contracture’ with no symptomatic improvement. He reported no prior or current alcohol use, no cigarette smoking, and did not take any scheduled medications. He reported no trauma to the hands, occupational or accidental. Vital signs were unremarkable but examination of the left hand showed a 6 cm mass involving the thenar eminence partially obliterating the first webspace, with atrophic and shiny overlying skin (Fig. 1a). Basic laboratory studies including blood counts, renal, liver and thyroid functions were within normal limits. The patient was referred to a hand surgeon, and a subtotal resection of the mass was performed. Histopathology demonstrated a nodular proliferation of medium–large-sized, aberrant T-lymphoid cells, concerning for a lymphomatous process. A positron emission tomography (PET) scan showed diffuse uptake in the left hand without systemic uptake (Fig. 1b). Based on the microscopic features and PET findings of isolated hand involvement, a diagnosis of solitary acral CD4 T cell lymphoma (TCL) was made. Despite localized radiation therapy, oral vorinostat (histone deacetylase inhibitor) treatment and ultimately amputation of the hand due to gangrene, he died a few weeks later.

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