Elsevier

Clinics in Dermatology

Volume 32, Issue 3, May–June 2014, Pages 376-388
Clinics in Dermatology

Neutrophilic dermatoses as systemic diseases

https://doi.org/10.1016/j.clindermatol.2013.11.004Get rights and content

Abstract

Neutrophilic dermatoses (ND) are inflammatory skin conditions characterized by a sterile infiltrate of normal polymorphonuclear leukocytes. The main clinical forms of ND include Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutinum, subcorneal pustular dermatosis, and their atypical or transitional forms. ND are often idiopathic, but they may be associated with myeloid hematologic malignancies (Sweet syndrome), inflammatory bowel disease or rheumatoid arthritis (pyoderma gangrenosum), and monoclonal gammopathies (erythema elevatum diutinum, subcorneal pustular dermatosis). The possible infiltration of internal organs with neutrophils during the setting of ND underlies the concept of a neutrophilic systemic disease. ND may be seen as a polygenic autoinflammatory syndrome due to their frequent association with other autoinflammatory disorders (monogenic or polygenic) and the recent published efficacy of interleukin-1 blocking therapies in their management.

Section snippets

Sweet syndrome

Acute febrile neutrophilic dermatosis was described by Robert Douglas Sweet in 1964 and soon came to be known as SS.6 It is characterized by fever; neutrophilia (with blood polymorphonuclear leukocyte level greater than 10,000/mm3); painful, erythematous 0.5 to 12 cm papules or plaques (Figure 1) on the extremities, face, and neck; and a dense dermal neutrophilic infiltrate.6 Additional criteria for SS include absence of infection and responsiveness to corticosteroid.6 Microscopic examination

Lung

Culture-negative pulmonary lesions in patients with ND have been reported in patients with SS, PG, and SPD. Most patients had fever, and pulmonary symptoms were characterized by cough, dyspnea, or chest pain. One patient had aseptic abscesses in many organs, especially the lung, without skin lesions, which underlines the possibility of a neutrophilic systemic disease without skin ND.63 The most recent review of the literature about SS with lung involvement, in 2006, reported 23 cases.64 In SS,

Acute myelogenous leukemia

AML may be associated with ND (more frequently SS, but sometimes PG), and ND may reveal an AML. Recently, the same cytogenetic anomaly in the neutrophils infiltrating the skin and in the bone marrow in myeloid malignancies has been demonstrated, and hence is indicative of a clonal neutrophilic dermatosis.101 A case of SS with folliculitis and neutrophilic panniculitis after remission of an AML with an increased level of endogenous G-CSF has been reported.102 Neutrophilic dermatosis of the

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