Immune-Mediated Cutaneous Paraneoplastic Syndromes Associated With Hematologic Malignancies: Skin as a Mirror of Hematologic Neoplasms

Abstract

Malignant neoplasms may present as paraneoplastic syndromes with mucocutaneous manifestations, which may or may not be chronologically associated. The pathophysiological mechanism is complex and not completely understood; therefore, definitive diagnosis may be achieved with a precise differential diagnosis based on the morphology of skin lesions, clinical picture, and histological pattern. The complexities, and low frequency, make the therapeutic approach quite challenging; consequently, the cornerstone of therapy is the eradication of the underlying neoplasms. Corticosteroids are the therapy of choice for most of these immune-mediated manifestations, but for the most part, the successful resolution requires the eradication of the underlying malignancy.

Pearls and updates: cutaneous signs of systemic malignancy

“…Others include juvenile dermatomyositis, Sweet syndrome, and pyoderma gangrenosum [1,45]. In children, PNP is most frequently seen in the setting of Castleman's disease, a rare, benign lymphoproliferative disorder, though it is almost always associated with myeloproliferative and lymphoproliferative disorders in adults [45,46,47 ▪ ,48].…”

“…PNP is diagnosed via a combination of clinical findings, skin biopsy, and blood tests. Histopathology shows suprabasal acantholysis and basal vacuolization with lichenoid lymphocytic infiltrate, direct immunofluorescence of the skin often reveals intercellular deposition of IgG and complement [47 ▪ ], and indirect immunofluorescence detects serum autoantibodies to multiple desmosomal proteins, as well as rat bladder [1,45,49 ▪ ]. Treatment involves high-dose systemic corticosteroids followed by steroid-sparing immunosuppressive drugs along with removal of the underlying tumor, if applicable.…”

“…Prognosis is poor, with 90% mortality with comorbid malignancy or if respiratory epithelium is involved as respiratory damage is permanent. Full recovery is possible in Castleman's disease or benign thymoma patients [45,46,47 ▪ ,48].…”