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ORPHA:3243Disease
Also called Acute febrile neutrophilic dermatosis
What it is
A rare inflammatory disease characterized by abrupt appearance of painful, edematous and erythematous papules, plaques and nodules on the skin, and frequently accompanied by fever and neutrophilia with a dense infiltration of mature neutrophils that are typically located in the upper dermis. The disease is classically associated with inflammatory disease, pregnancy, infection (mostly of the upper respiratory tract), or vaccination but may be idiopathic, associated with a hematological or visceral malignancy, or drug-induced.
Key facts
- Age of onset
- Adult
- Inheritance
- Multigenic/multifactorial
- Classified as
- Disease
Signs and symptoms
Very common80–99%
6Common30–79%
16- Abnormality of the face
- Abnormality of the hand
- Abnormality of the neck
- Abnormality of tumor necrosis factor secretion
- Acne
- Arthralgia
- Elevated circulating C-reactive protein concentration
- Elevated erythrocyte sedimentation rate
- Increased circulating interleukin 6 concentration
- Increased total leukocyte count
- Myalgia
- Myositis
- Neutrophilia
- Non-periodic recurrent fever
- Small vessel vasculitis
- Sterile abscess
Sometimes5–29%
14- Abnormal blistering of the skin
- Abnormal drug response
- Acne inversa
- Acute myeloid leukemia
- Anemia
- Hematological neoplasm
- Inflammation of the large intestine
- Neoplasm
and 6 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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