Acute Febrile Neutrophilic Dermatosis

Acute febrile neutrophilic dermatosis may occur with various disorders. It is often classified into 3 categories:

About 25% of patients have an underlying cancer, 75% of which are hematologic cancers, especially myelodysplastic syndromes Myelodysplastic Syndrome (MDS) The myelodysplastic syndrome (MDS) is group of clonal hematopoietic stem cell disorders typified by peripheral cytopenia, dysplastic hematopoietic progenitors, a hypercellular or hypocellular... read more and acute myelogenous leukemia Acute Myeloid Leukemia (AML) In acute myeloid leukemia (AML), malignant transformation and uncontrolled proliferation of an abnormally differentiated, long-lived myeloid progenitor cell results in high circulating numbers... read more . The dermatosis often precedes the cancer diagnosis. Classical acute febrile neutrophilic dermatosis affects mostly women ages 30 to 50, with a female:male ratio of 3:1. In contrast, men who develop the condition tend to be older (60 to 90). In children under age 3, the ratio is male:female 2:1.

The cause of acute febrile neutrophilic dermatosis is unknown; however, type 1 helper T-cell cytokines, including interleukin-2 and interferon-gamma, are predominant and may play a role in lesion formation.

Patients are febrile, with an elevated neutrophil count, and have painful, tender, and edematous red to violet plaques or papules, most often on the face, neck, and upper extremities, especially the dorsum of hands. Oral lesions can also occur. The lesions often develop in crops and may appear annular. Each crop is usually preceded by fever and persists for days to weeks. Rarely, bullous and pustular lesions are present as well.

Less common variants include a bullous form that can ulcerate and resemble pyoderma gangrenosum Pyoderma Gangrenosum Pyoderma gangrenosum is a chronic, neutrophilic, progressive skin necrosis of unknown etiology often associated with systemic illness and sometimes skin injury. Diagnosis is clinical. Treatment... read more and a subcutaneous form involving the subcutaneous fat that typically has 2- to 3-cm erythematous nodules, commonly affecting the extremities. When on the lower extremities, this form can resemble erythema nodosum Erythema Nodosum Erythema nodosum is a specific form of panniculitis characterized by tender, red or violet, palpable, subcutaneous nodules on the shins and occasionally other locations. It often occurs with... read more .

Extracutaneous manifestations are rare and can involve the eyes (eg, conjunctivitis Overview of Conjunctivitis Conjunctival inflammation typically results from infection, allergy, or irritation. Symptoms are conjunctival hyperemia and ocular discharge and, depending on the etiology, discomfort and itching... read more , episcleritis Episcleritis Episcleritis is self-limiting, recurring, usually idiopathic inflammation of the episcleral tissue that does not threaten vision. Symptoms are a localized area of hyperemia of the globe, irritation... read more , iridocyclitis), joints (eg, arthralgia, myalgia, arthritis), and internal organs (eg, neutrophilic alveolitis; sterile osteomyelitis; psychiatric or neurologic changes; transient kidney, liver, and pancreatic insufficiency).

Diagnosis of acute febrile neutrophilic dermatosis is suggested by the appearance of the lesions and is supported by the presence of associated conditions or drugs. Differential diagnosis can include erythema multiforme Erythema Multiforme Erythema multiforme is an inflammatory reaction, characterized by target or iris skin lesions. Oral mucosa may be involved. Diagnosis is clinical. Lesions spontaneously resolve but frequently... read more , erythema elevation diutinum, subacute cutaneous lupus erythematosus Subacute cutaneous lupus erythematosus (SCLE) Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Common manifestations may include arthralgias and... read more , pyoderma gangrenosum Pyoderma Gangrenosum Pyoderma gangrenosum is a chronic, neutrophilic, progressive skin necrosis of unknown etiology often associated with systemic illness and sometimes skin injury. Diagnosis is clinical. Treatment... read more , and erythema nodosum Erythema Nodosum Erythema nodosum is a specific form of panniculitis characterized by tender, red or violet, palpable, subcutaneous nodules on the shins and occasionally other locations. It often occurs with... read more .

If the diagnosis is unclear, skin biopsy Biopsy Diagnostic tests are indicated when the cause of a skin lesion or disease is not obvious from history and physical examination alone. These include Patch testing Biopsy Scrapings Examination... read more should be done. The histopathologic pattern is that of edema in the upper dermis with a dense infiltrate of neutrophils in the dermis. Vasculitis may be present but is secondary.

A complete blood count (CBC) is also done. If the CBC is abnormal, bone marrow biopsy should be considered to diagnose occult cancer.

Treatment of acute febrile neutrophilic dermatosis involves systemic corticosteroids, chiefly prednisone 0.5 to 1.5 mg/kg orally once a day tapered over 3 weeks. Colchicine 0.5 mg orally 3 times a day or potassium iodide 300 mg orally 3 times a day are alternative treatments. Antipyretics are also recommended.

In difficult cases, dapsone 100 to 200 mg orally once a day, indomethacin 150 mg orally once a day for 1 week and 100 mg orally once a day for 2 additional weeks, clofazimine (eg, 200 mg orally once a day for 4 weeks then 100 mg/day for 4 weeks), or cyclosporine (eg, 2 to 4 mg/kg orally 2 times a day) can be given. Other treatments used for refractory disease include infliximab, etanercept, thalidomide, minocycline, and mycophenolate mofetil.

For localized involvement, intralesional corticosteroids (eg, triamcinolone acetonide) may help.