Page 417 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Dermatologic Aspects of Systemic Vasculitis 391

Affected patients had constitutional symptoms, arthralgia, leukopenia, agranulocytosis
and cutaneous vasculitis [66, 67] with purpuric lesions of the ears, nose, cheeks, and
extremities. The lesions have bright-red borders with central necrosis (Figure 20). Despite the
severe and dramatic clinical appearance of these lesions, they usually resolve spontaneously
within a few weeks of drug discontinuation but can recur with subsequent contaminated
cocaine abuse. The degree of skin necrosis has been as severe and large as to require skin
grafting. One-half of affected patients each demonstrated positive anti-MPO or positive anti-
PR3 antibodies [66].

Systemic Malignancy

Lymphoproliferative, myeloproliferative, and carcinomatous tumors comprise <5% of
paraneoplastic cutaneous vasculitis (Figure 21), a diagnosis that may be considered in patients
with recurrent purpura, hematologic abnormalities including cytopenia, monoclonal
gammopathy, immature blood cells; hematuria, abnormal tissue or nodal masses on imaging
studies, and refractory responses to immune therapies. There are three such categories of
patients including, those with true paraneoplastic vasculitic syndromes wherein the vasculitis
improves with extirpation or treatment of the tumor; vasculitis masquerading as malignancy
such as lung masses in GPA; and malignancy masquerading as vasculitides as in emboli from
an atrial myxoma and superficial migratory thrombophlebitis with pancreatic cancer. Most
paraneoplastic cutaneous vasculitic syndromes are the result of a paraproteinemia secondary
to lymphoproliferative disorders, including cryoglobulinemia in association with lymphocytic
lymphoma and Waldenström macroglobulinemia.

Figure 21. Myelodysplastic syndrome presenting as erythema elevatum diuntinum. A large ulcerative
tumor shows localized fibrosing leukocytoclastic vasculitis. Courtesy of Juan Carlos Graces, MD,
Guayaquil, Ecuador).

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