Palisaded Neutrophilic and Granulomatous Dermatitis in a Patient with Granulomatosis with Polyangiitis

Abstract

KeywordsGranulomatosis with polyangiitis · PR3-ANCA · M2 macrophage · Palisaded neutrophilic and granulomatous dermatitis · CD163 AbstractPalisaded neutrophilic and granulomatous dermatitis (PNGD) shows various clinical features and is histologically characterized by palisaded granulomas surrounding degenerated collagen. PNGD is known to be associated with a variety of systemic conditions such as rheumatoid arthritis and systemic lupus erythematosus. Furthermore, PNGD has been reported to be associated with antineutrophilic cytoplasmic antibody-associated vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis. Here, we report a case of PNGD associated with GPA, which showed the infiltration of CD163-positive M2 macrophages in the skin lesion with elevated serum level of soluble CD163 (sCD163). The serum sCD163 level was reduced to normal range after systemic steroid therapy. Thus, M2 macrophages may play a role in the pathomechanisms of PNGD associated with GPA.

Reactive Granulomatous Dermatitis: A Descriptive Study of 10 Patients

“…2 Besides arthritis and inflammatory bowel disease, systemic lupus erythematosus, systemic sclerosis, ANCA vasculitis, Sjogren's syndrome, and mixed cryoglobulinemia have also been described with RGD. [1][2][3][8][9][10][11][12][13] Analogous to previous publications, TNF inhibitors could be a trigger for RGD. 14 Out of the 10 patients in our study, 2 cases were possibly related to this medication.…”

“… 2 Besides arthritis and inflammatory bowel disease, systemic lupus erythematosus, systemic sclerosis, ANCA vasculitis, Sjogren’s syndrome, and mixed cryoglobulinemia have also been described with RGD. 1 - 3 , 8 - 13 …”

Reply

“…We also note that panniculitis (1), neutrophilic dermatosis (2), granulomatous dermatitis (3), and other nonspecific or atypical cutaneous manifestations have been reported in AAV. These “nonvasculitic” manifestations lack histologic findings of vasculitis but nonetheless are cutaneous complications of AAV that may correlate with disease onset or flare and may aid in diagnosis.…”

Palisaded neutrophilic granulomatous dermatitis in a patient with HLA-B27–negative axial spondyloarthritis: a case report and literature review

“…PNGD is a reaction pattern that can occur a few years prior to diagnosis, concomitantly with or many years after numerous lymphoproliferative, autoimmune, and autoinflammatory diseases, including Hodgkin's lymphoma, non-Hodgkin's lymphoma, chronic myelomonocytic leukemia, ulcerative colitis, systemic lupus erythematosus, rheumatoid arthritis, sarcoidosis, systemic vasculitides (i.e., microscopic polyangiitis and granulomatosis with polyangiitis), and Behçet's disease (4)(5)(6)(7)(9)(10)(11)(12)(13)(14). An association of PNGD with HLA-B27-negative axSpA has not been reported in the literature so far, making our case unique.…”