Neutrophilic dermatoses associated with hematologic disorders

Abstract

The neutrophilic dermatoses are a group of disorders characterized histologically by dermal neutrophilic infiltrates, having no infectious or other identifiable etiology. These entities include acute febrile neutrophilic dermatosis, pyoderma gangrenosum, erythema elevatum diutinum, subcorneal pustular dermatosis, and neutrophilic eccrine hidradenitis. The neutrophilic dermatoses are frequently considered to be part of a continuous spectrum because they share many features including cutaneous neutrophilic infiltrates, similar clinical eruptions, systemic involvement, association with systemic diseases, and responsiveness to immunosuppressive agents.1 When evaluating patients with the clinical lesions of a neutrophilic dermatosis, infection, leukemic cell infiltration, and allergic contact dermatitis must be excluded as causes.2 The pathogeneses for these disorders are still unclear, but they are recognized paraneoplastic phenomena.3,4 The neutrophilic dermatoses may be associated with a variety of systemic disorders including myeloproliferative disorders, monoclonal gammopathies (especially IgA), inflammatory bowel disease, and rheumatoid arthritis.4 This report discusses the neutrophilic dermatoses and their associations with hematologic disorders. Acute Febrile Neutrophilic Dermatosis Acute febrile neutrophilic dermatosis (AFND) was described by Dr. Robert Sweet in 1964 and soon came to be known as Sweet’s syndrome.5 It is a clinically and pathologically distinct entity of unknown etiology6 and is characterized by fever, neutrophilia, erythematous plaques on the limbs, face and neck, and dense dermal neutrophilic infiltration.5 Additional criteria include absence of infection and responsiveness to corticosteroids.5 Although most cases are thought to be idiopathic,7 AFND can be associated with a variety of disorders, including malignancies, infections, autoimmune disorders, pregnancy, drugs, trauma, subacute thyroiditis, Behcet’s syndrome, acute renal failure, and human im