Neutrophilic disease: a review of extracutaneous neutrophilic manifestations

Sweet's syndrome, pyoderma gangrenosum, subcorneal pustular dermatosis, erythema elevatum diutinum form part of the clinical spectrum of neutrophilic dermatoses. ese conditions share common features, such as the association with systemic diseases, i.e. myeloproliferative disorders, monoclonal gammopathies, rheumatoid arthritis, inflammatory bowel diseases. In addition, extra-cutaneous neutrophilic infiltrates may occur in patients with a neutrophilic dermatosis, thus defining neutrophilic disease. We review here the literature on cases of patients with neutrophilic dermatoses and well-documented, culture-negative, extra-cutaneous, neutrophilic lesions. Sites of involvement include the lungs, bones, joints, liver, central nervous system, lymph nodes, spleen, digestive tract, blood vessels and eyes. The awareness of these systemic manifestations allows the dermatologist to accurately evaluate these patients and to avoid unnecessary, aggresive investigations.