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- Title
Neutrophilic Urticaria with Systemic Inflammation Associated with Immunoglobulin A Myeloma.
- Authors
PAUGOY, Héloïse; SAUSSINE, Anne; FRUMHOLTZ, Laure; BATTISTELLA, Maxime; JACHIET, Marie; RIVET, Jacqueline; LEPELLETIER, Clémence; DUVERGER, Lucie; BAGOT, Martine; TALBOT, Alexis; ARNULF, Bertrand; BOUAZIZ, Jean-David
- Abstract
Neutrophilic dermatoses are a heterogeneous group of disorders characterized by neutrophilic infiltration of the skin (1). Major types of neutrophilic dermatoses are pyoderma gangrenosum (PG), Sweet's syndrome, subcorneal pustular dermatosis (SPD), erythema elevatum diutinum (EED), and aseptic abscess. They have been reported in association with various conditions, including autoimmune diseases, inflammatory bowel diseases, myeloid malignancies (acute myeloid leukaemia and myelodysplasia) and monoclonal gammopathies (MG). A previous study (2) revealed an association between IgA isotype MG and neutrophilic dermatoses, mostly EED, SPD and PG. Neutrophilic urticaria with systemic inflammation (NUSI) has been defined as neutrophilic urticarial lesions with fever in which the interleukin-1 cytokine could play a key pathophysiological role (3). Neutrophilic urticaria may be linked with inherited hyperactivation of the inflammasome pathway, such as NLRP3 gain of function (4), or external stimuli that may activate the inflammasome, such as IgM MG in Schnitzler syndrome. We report here a case of a NUSI associated with an IgA myeloma.
- Subjects
SWEET'S syndrome; INFLAMMATORY bowel diseases; URTICARIA; ACUTE myeloid leukemia; PYODERMA gangrenosum; MONOCLONAL gammopathies
- Publication
Acta Dermato-Venereologica, 2021, Vol 101, Issue 4, p1
- ISSN
0001-5555
- Publication type
Article
- DOI
10.2340/00015555-3787