Mucocutaneous paraneoplastic manifestations of hematologic malignancies

To review the clinical manifestations, pathophysiology, and oncologic implications of the major mucocutaneous paraneoplastic syndromes that can appear in patients with hematologic malignancies.

A comprehensive search of the medical literature was conducted.

In vesiculobullous conditions, although the primary lesions are blisters, observed abnormalities may include large, flaccid bullae (pemphigus vulgaris), superficial crusted erosions (pemphigus foliaceus), or erythema multiforme-like lesions (paraneoplastic pemphigus). Paraneoplastic neutrophilic dermatoses include Sweet's syndrome and pyoderma gangrenosum. In both of these conditions, the skin lesions are characterized by a dermal infiltrate of mature neutrophils. Vascular dermatoses include vasculitis and erythromelagia. Papulosquamous conditions are characterized by small (papules) or large (plaques) raised skin lesions and are usually associated with solid tumors. Amyloidosis is a malignancy-related condition that probably stems from immune dysregulation.

Continued surveillance of patients with potential cutaneous paraneoplastic syndromes is necessary, since the malignancy may not be immediately detectable. Some of the cutaneous paraneoplastic syndromes will respond to specific measures, such as systemic corticosteroid therapy, but for the most part, successful resolution requires eradication of the underlying malignancy.