Management of neutrophilic dermatoses

Abstract

Neutrophilic dermatoses, including Sweet's syndrome, pyoderma gangrenosum, and rheumatoid neutrophilic dermatitis, are inflammatory conditions of the skin often associated with underlying systemic disease. These are characterized by the accumulation of neutrophils in the skin. The associated conditions, potential for systemic neutrophilic infiltration, and therapeutic management of these disorders can be similar. Sweet's syndrome can often be effectively treated with a brief course of systemic corticosteroids. Pyoderma gangrenosum, however, can be recurrent, and early initiation of a steroid-sparing agent is prudent. Second-line treatment for both of these conditions includes medications affecting neutrophil function, in addition to immunosuppressant medications.

Sweet’s syndrome revealed by oral pustulosis

“…In addition to the two major criteria (abrupt onset of tender or painful erythematous or violaceous plaques or nodules and neutrophilic infiltration of the dermis without leukocytoclastic vasculitis), he had three minor criteria (elevation of the CRP level, leukocytosis and preceding slight fever) [2]. Like our case report, cases of SS with recurrent oral ulcerations were reported [1,[4][5][6][7]. They had tender erythematous nodular or vesicle-like papular skin eruptions on both forearms [5], over the face, neck, palms and legs [4], multicentric erythema and pustules progressing to ulcers on the legs, face, trunk and extremities [7], and erythematous plaques on the legs [6].…”

“…Neutrophilic dermatoses (ND) are a collection of diseases characterized by the accumulation of neutrophils in the skin without an identifiable infectious agent [1]. Neutrophilic dermatoses, including Sweet's syndrome (SS), pyoderma gangrenosum (PG), subcorneal pustular dermatosis (Sneddon-Wilkinson disease (SW)) and rheumatoid neutrophilic dermatitis, are inflammatory conditions of the skin often associated with underlying systemic disease [2].…”

Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with biliary sepsis

“…SS recurs in approximately one-third of patients. Of the cases that recur, the cause is often malignancy or drug induced 3 4. Although there were four cardinal features of SS when first described, further reports reveal that fever and neutrophilia were not consistent features.…”

Sweet Syndrome in a Patient With Systemic Lupus Erythematosus Flare.

“…Second-line therapies include nonsteroidal anti-inflammatory drugs (e.g., indomethacin, naproxen), dapsone, clofazimine, cyclosporine, and thalidomide. There are reports of efficacy with methotrexate, danazol, interferon-alpha, intravenous immunoglobulins, and etetrinate [17]. Lucia et al reported successfully using rituximab for recalcitrant SS [19], but further studies are required to definitely suggest it as a treatment.…”

“…Corticosteroid therapy is considered first-line treatment for SS [16]. In addition, there are some reports suggesting the use of colchicine and potassium Iodide as first-line treatment options [17]. Symptoms often begin to improve within 48 hours and skin lesions usually resolve within one to two weeks after use of steroids [18].…”