Acute Febrile Neutrophilic Dermatosis

Acute Febrile Neutrophilic Dermatosis

Key Points
Systemic condition known to follow or run concurrently with other diseases
Manifests as tender red lumps on the limbs or neck
Often found in women of middle age, but men and even children may be affected
Also known as Sweet's syndrome

Acute neutrophilic dermatosis is a condition which causes tender red lumps, or plaques, on the neck and limbs, particularly the backs of the hands. It can develop as a reaction to other conditions, such as infections, blood disorders, arthritis, cancer, inflammatory bowel disease, even pregnancy, however, the disorder can develop in persons with no underlying condition. While the majority of affected persons will only have the disorder once, approximately 1/3 will see recurrence of the disorder.

In addition to the development plaques on the limbs and neck which begin as small red bumps, the disorder is usually accompanied by fever, instances of joint pain, conjunctivitis, or ulcers in the mouth. Plaques can persist and even grow for several weeks during the course of the disorder.  Lesions often have a blister-like appearance termed “pseudovesicular”.

Differential Diagnosis (Other conditions with similar appearance)
Pyoderma gangrenosum
Furuncle
Erythema elevatum diutinum
Lupus
Urticaria
Erythema multiforme

Diagnosis
Key Points
Diagnosis based on skin appearance and usually skin Biopsy
Often associated with a corollary illness
Health care professionals may recommend further testing to rule out association with serious illness

Treatment
Symptoms will alleviate in several weeks/months without treatment
*Treatment of symptoms, however, cuts healing time to a few days

While Acute Neutrophilic Dermatosis can and will run its course and disappear on its own in several weeks or longer, treating the plaques and other symptoms will greatly reduce healing time. Generally, systemic steroids such as Prednisone are prescribed, and will clear up most symptoms within a few days. However, health care professionals may recommend a lower dosage of steroids to follow the initial course to prevent a relapse of the disorder.

In cases where steroids are ineffective, medications such as solution of saturated potassium iodide (SSKI), Dapsone, Colchicine, non-steroidal anti-inflammatories, and biologic response modifiers may be used to combat the disorder.