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2016 ; 95
(11
): e2957
Nephropedia Template TP
gab.com Text
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English Wikipedia
Neutrophilic Dermatoses in Antineutrophil Cytoplasmic Antibody-Associated
Vasculitis: A French Multicenter Study of 17 Cases and Literature Review
#MMPMID26986103
de Boysson H
; Martin Silva N
; de Moreuil C
; Néel A
; de Menthon M
; Meyer O
; Launay D
; Pagnoux C
; Guillevin L
; Puéchal X
; Bienvenu B
; Aouba A
Medicine (Baltimore)
2016[Mar]; 95
(11
): e2957
PMID26986103
show ga
A few reports suggest combination of ANCA-associated vasculitis (AAV) and
neutrophilic dermatoses (ND). We aimed to describe the main characteristics of
patients presenting with both AAV and ND in a French cohort and through a
systematic literature review, and to discuss the possible common pathogenic
process involved. We conducted a retrospective study of patients with both
conditions. Patients were selected via the French Internal Medicine Society
(SNFMI) and the French Vasculitis Study Group (FVSG). A literature review
focusing on a combination of both conditions, concentrated only on publications
with well-established diagnoses and individual detailed data. Seventeen patients
diagnosed with AAV and ND were identified in this cohort. Twelve patients had
granulomatosis with polyangiitis (GPA), 4 had microscopic polyangiitis (MPA) and
one had eosinophilic GPA (EGPA). Eight patients, all with GPA, displayed pyoderma
gangrenosum (PG). Sweet's syndrome was observed in 6 patients (4 with MPA, one
with GPA and one with EGPA) and erythema elevatum diutinum in the other three (2
with GPA and 1 with MPA). The literature review identified 33 additional patients
with both conditions, including 26 with GPA. Altogether, of the 50 patients (17
from our study and 33 from the literature review), 33 (66%) patients presented
with PG associated with GPA in 29 cases (89%). Corticosteroids were the
first-line treatment in conjunction with an immunosuppressive agent in most
cases. Outcomes were good and a total of 15 patients experienced a relapse.
Patients who relapsed were more likely to have ear, nose and throat manifestation
than patients who did not [12/15 (80%) relapsing patients vs. 15/35 (43%)
non-relapsing patients; p?=?0.03)]. In our stud, the most frequent association
concerned GPA and PG. ND should be considered and specifically researched within
the spectrum of cutaneous manifestations observed in AAV.