title: Neutrophilic skin lesions in autoimmune connective tissue diseases: nine cases and a literature review.
abstract: The pathophysiology of neutrophilic dermatoses (NDs) and autoimmune connective tissue diseases (AICTDs) is incompletely understood. The association between NDs and AICTDs is rare; recently, however, a distinctive subset of cutaneous lupus erythematosus (LE, the prototypical AICTD) with neutrophilic histological features has been proposed to be included in the spectrum of lupus. The aim of our study was to test the validity of such a classification. We conducted a monocentric retrospective study of 7028 AICTDs patients. Among these 7028 patients, a skin biopsy was performed in 932 cases with mainly neutrophilic infiltrate on histology in 9 cases. Combining our 9 cases and an exhaustive literature review, pyoderma gangrenosum, Sweet syndrome (n = 49), Sweet-like ND (n = 13), neutrophilic urticarial dermatosis (n = 6), palisaded neutrophilic granulomatous dermatitis (n = 12), and histiocytoid neutrophilic dermatitis (n = 2) were likely to occur both in AICTDs and autoinflammatory diseases. Other NDs were specifically encountered in AICTDs: bullous LE (n = 71), amicrobial pustulosis of the folds (n = 28), autoimmunity-related ND (n = 24), ND resembling erythema gyratum repens (n = 1), and neutrophilic annular erythema (n = 1). The improvement of AICTDS neutrophilic lesions under neutrophil targeting therapy suggests possible common physiopathological pathways between NDs and AICTDs.
authors.affiliation: From the Dermatology Department (EH, AS, MJ, FC, MarB, MR, JDB) and Pathology Department (MDVP, MaxB, BCB), Paris Diderot University, Sorbonne Paris Cité, AP-HP, Saint Louis Hospital, Paris, France.
authors.forename: Estelle
authors.initials: E
authors.lastname: Hau
authors.forename: Marie-Dominique
authors.initials: MD
authors.lastname: Vignon Pennamen
authors.forename: Maxime
authors.initials: M
authors.lastname: Battistella
authors.forename: Anne
authors.initials: A
authors.lastname: Saussine
authors.forename: Maud
authors.initials: M
authors.lastname: Bergis
authors.forename: Benedicte
authors.initials: B
authors.lastname: Cavelier-Balloy
authors.forename: Michel
authors.initials: M
authors.lastname: Janier
authors.forename: Florence
authors.initials: F
authors.lastname: Cordoliani
authors.forename: Martine
authors.initials: M
authors.lastname: Bagot
authors.forename: Michel
authors.initials: M
authors.lastname: Rybojad
authors.forename: Jean-David
authors.initials: JD
authors.lastname: Bouaziz
mesh_terms: D000293:Adolescent; D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications*; D003240:Connective Tissue Diseases / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000378:metabolism*; D012189:Retrospective Studies; D012867:Skin / Q000378:metabolism*; D012871:Skin Diseases / Q000150:complications*; D055815:Young Adult