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

2020 ◽  
Vol 12 (1) ◽  
pp. 52-56
Author(s):  
Mai Akagawa ◽  
Yuki Hattori ◽  
Yoko Mizutani ◽  
En Shu ◽  
Tatsuhiko Miyazaki ◽  
...  

Palisaded 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.

2020 ◽  
Vol 23 (5) ◽  
pp. 334-340
Author(s):  
N. P. Teplyuk ◽  
O. V. Grabovskaya ◽  
Polina A. Razhev

BACKGROUND: This clinical case is interesting because of extreme rarity of lupus panniculitis in the population, as well as the possible risk of developing systemic lupus erythematosus. CASE REPORT: The article presents a clinical observation of a rare skin disorder lupus erythematosus panniculitis and the results of a successful combined treatment of the disease with systemic glucocorticoids (prednisolone), and an antimalarial agent (hydroxychloroquine). A review of the literature on the etiology, pathogenesis, and diagnosis of lupus panniculitis has been conducted. CONCLUSION. Early detection of lupus erythematosus panniculitis (LEP) makes systemic steroid therapy and quinoline drugs more effective in achieving remission. That is why dynamic monitoring of patients with LEP is important to identify new or progressive symptoms.


2021 ◽  
Vol 41 (05) ◽  
pp. 541-553
Author(s):  
Kristin Galetta ◽  
Shamik Bhattacharyya

AbstractSystemic autoimmune diseases can affect the peripheral and central nervous system. In this review, we outline the common inpatient consultations for patients with neurological symptoms from rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, sarcoidosis, immunoglobulin G4–related disease, Behçet's disease, giant cell arteritis, granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis, polyarteritis nodosa, and ankylosing spondylitis. We discuss the symptoms, diagnostic strategies, and treatment options.


Lupus ◽  
2018 ◽  
Vol 27 (14) ◽  
pp. 2220-2227 ◽  
Author(s):  
S Terai ◽  
I Ueda-Hayakawa ◽  
C T H Nguyen ◽  
N T M Ly ◽  
F Yamazaki ◽  
...  

Background Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a commonly occurring condition related to systemic autoimmune disease. It is characterized histopathologically by a distinct pattern of granulomatous inflammation in the presence or absence of leukocytoclastic vasculitis. The properties of granulomatous cells in PNGD are still uncertain. Objective We sought further investigation on the phenotype of the infiltrated cells in PNGD from two patients with systemic lupus erythematosus (SLE) and reviewed the previous published reports in order to provide a comprehensive summary on the clinical features of PNGD in SLE. Methods The immunohistochemical features of granulomatous cells in PNGD associated with SLE were analyzed. Immunohistochemical studies were performed on sections from our two cases using antibodies against CD68, CD163, CD15, Factor XIIIa, myeloperoxidase and neutrophil elastase. The clinical characteristics of the SLE patients who developed PNGD were also evaluated. We included all cases retrieved through a PubMed search with the key words PNGD and SLE. Results Cutaneous lesions consisted of erythematous plaques distributed on the face and upper limbs in both cases. The infiltrated cells were mainly positive for CD68 and CD163, a phenotype suggestive of M2 macrophages. Some mature neutrophils and lymphocytes were also present. A review of the literature of PNGD associated with SLE revealed a predominance in females, high prevalence of lupus nephritis and a good response to systemic steroids, with frequent skin lesions relapses during tapering of the treatment. Limitations This study examined only two cases; the pathogenesis of the disease remains to be clarified. Conclusion PNGD lesions were abundantly infiltrated by M2 macrophages, suggesting that they may have a role in this condition. SLE accompanied by PNGD might be associated with lupus nephritis and frequent relapses of skin lesions.


Lupus ◽  
2019 ◽  
Vol 28 (12) ◽  
pp. 1473-1476 ◽  
Author(s):  
E Takamasu ◽  
N Yokogawa ◽  
K Shimada ◽  
S Sugii

Objective This study aimed to investigate the risk factors of hydroxychloroquine (HCQ)-induced hypersensitivity in patients with systemic lupus erythematosus (SLE) and to propose a simple dose-escalation regimen in cases of mild HCQ-induced hypersensitivity. Methods We identified patients with SLE who started HCQ between 2009 and 2018 and cases of HCQ-induced hypersensitivity by reviewing the electronic medical charts. A simple dose-escalation regimen, starting at 40 mg/day with weekly increments of 40 mg/day to 200 mg/day, was used in patients with HCQ-induced hypersensitivity who did not require hospitalization or systemic steroid therapy. We then compared the clinical parameters of patients with and without HCQ-induced hypersensitivity and evaluated the success of our dose-escalation regimen. Results We enrolled 302 patients with SLE and identified 25 cases of HCQ-induced eruption (8.3%). The mean Naranjo score of these patients was 5.1 ± 1.4 (min 3, max 8), and all 25 patients received a ‘possible’ (9) or ‘probable’ (16) score. A mild, generalized, maculopapular rash occurred in 24 patients, and urticaria occurred in one patient at 24 days (interquartile range 15–40 days) after the start of treatment. The proportion of cyclophosphamide use, glucocorticoid consisting of prednisolone 20 mg/day or more, and initiation of SMX-TMP within 28 days were higher in patients with skin eruptions. On multivariate analysis, only cyclophosphamide use was identified as a risk factor of HCQ-induced hypersensitivity (odds ratio = 12.3 (95% confidential interval 1.4–14.3)). Thirteen of the 14 patients on the dose-escalation regimen (92.9%) tolerated continued HCQ treatment. One patient re-experienced eruptions on day 10 day after starting HCQ. Conclusions Mild late reactions are common in HCQ-induced hypersensitivity. A simpler dose-escalation regimen enables safe and easier reintroduction of HCQ but should not be applied to patients with immediate reactions or moderate late reactions.


2007 ◽  
Vol 131 (4) ◽  
pp. 650-651
Author(s):  
Mohammad Abuel-Haija ◽  
Matthew T. Hurford

Abstract Kimura disease is a benign rare chronic inflammatory disorder of unknown etiology that involves the lymph nodes and subcutaneous tissue of the head and neck regions. Elevated serum immunoglobulin E levels and peripheral blood eosinophilia are also common. This disease is most common in middle-aged Asian men. Although the etiology is unknown, it most probably represents an aberrant chronic immune response. Treatment for Kimura disease includes surgical resection and regional or systemic steroid therapy. Cytotoxic therapy and radiation have also been utilized. The disease has an excellent prognosis, although it may recur locally.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Toshinobu Kubota ◽  
Suzuko Moritani

Orbital IgG4-related disease, which can occur in adults of any age, is characterized by IgG4-positive lymphoplasmacytic infiltrations in ocular adnexal tissues. The signs and symptoms include chronic noninflammatory lid swelling and proptosis. Patients often have a history of allergic disease and elevated serum levels of IgG4 and IgE as well as hypergammaglobulinemia. Orbital IgG4-related disease must be differentiated from idiopathic orbital inflammation and ocular adnexal marginal zone B-cell lymphoma to ensure appropriate and effective treatment. Systemic steroid therapy decreases the size of the lesions, but relapse often occurs when systemic steroid therapy is discontinued.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1551.1-1552
Author(s):  
V. Mazurov ◽  
O. Shadrivova ◽  
M. Shostak ◽  
L. Martynova ◽  
M. Tonkoshkur ◽  
...  

Background:Invasive aspergillosis (IA) is a severe opportunistic infection that is not well understood in rheumatological patients.Objectives:To study risk factors, etiology, clinical manifestations and results of treatment of IA in adult rheumatological patients.Methods:Retrospective analysis of 830 patients (1998-2019) with “proven” and “probable” IA (EORTC / MSG, 2019), adults - 699 (84%). The main group included 18 (3%) adult rheumatological patients with IA, a control group included 610 (87%) adult hematological patients. Rheumatological patients were older, the average age was 59 years (21–75) vs 45 years (18–79), p = 0.005, and among them there were more women – 56% vs 42%, p = 0.01.Results:In rheumatological patients with IA, underlying diseases were ANCA-associated vasculitis (28%), granulomatosis with polyangiitis (22%), periarteritis (11%), systemic lupus erythematosus (22%), rheumatic heart disease (11%) and ankylosing spondylitis (6%). In the control group, underlying diseases were acute leukemia (45%), lymphomas (34%), chronic leukemia (9%), multiple myeloma (7%), myelodysplastic syndrome (3%), and other hematological diseases (2%).The main risk factors for IA development in rheumatological patients were: systemic steroids use (89% vs 69%), prolonged lymphocytopenia (76% vs 65%, median - 14 vs 12 days), treatment in ICU (44% vs 18%, p = 0.01), acute or chronic renal failure (39% vs 1%, p = 0.0008) and immunosuppressive therapy (28% vs 25%). Severe neutropenia was noted significantly less frequently (18% vs 83%, p = 0.0001). Additional risk factors were decompensated diabetes mellitus (17% vs 2%, p = 0.004), previous surgery (17% vs 1%, p = 0.001) and organ transplantation (6% vs 0%). In rheumatological patients, lung (83% vs 98%, p = 0.0001) and ≥2 organs (6% vs 8%) involvement were less common. Heart (11% vs 0%), sinuses (6% vs 5%) and central nervous system (6% vs 4%) involvement more often developed. In rheumatological patients, respiratory failure (61 vs 37%, p = 0.03), hemoptysis (28% vs 7%, p = 0.0001) and chest pain (17% vs 7%, p = 0, 04) were noted more often, less often - fever ≥380С (67% vs 85%, p = 0.01) and cough (61% vs 70%). CT signs of lung damage were similar in both groups, but rheumatologic patients were more likely to show an «air crescent» sign and / or destruction cavity (44% vs 10%, p = 0.0001). In rheumatologic patients, IA was more often confirmed by isolation ofAspergillusspp. from BAL (80% vs 45%, p = 0.005) and by histological examination (22% vs 7%, p = 0.01). The main pathogens wereA. fumigatus(50% vs 43%),A. niger(29% vs 32%), andA. flavus(14% vs 17%).Rheumatological patients were less likely to receive antifungal therapy 89% vs 99%, p = 0,0003. The main drug in both groups was voriconazole. The overall 12-week survival did not significantly differ between groups, but was lower in rheumatological patients with IA (69% vs 81%).Conclusion:In rheumatological patients, invasive aspergillosis more often developed at an older age, mainly in women. The main background diseases were ANCA-associated vasculitis, granulomatosis with polyangiitis, and systemic lupus erythematosus. Typical risk factors were steroids and immunosuppressants use, prolonged lymphocytopenia, ICU stay, and renal failure. The main causative agents wereA. fumigatus,A. niger, andA. flavus. The main localization of infection were lungs. Respiratory failure, hemoptysis and heart involvement were typical. The overall 12-week survival of rheumatological patients with invasive aspergillosis was 69%.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1813.1-1813
Author(s):  
B. Lamoreaux ◽  
M. Francis-Sedlak ◽  
R. Holt ◽  
J. Rosenbaum

Background:Autoimmune inflammatory conditions of the eye may be associated with rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, and granulomatosis with polyangiitis. This is also observed with thyroid eye disease (TED). Loss of immune tolerance to the thyroid stimulating hormone receptor has thyroidal consequences and nearly 40% of patients with Graves’ disease also have clinically evident Graves’ orbitopathy or TED.1TED results from tissue inflammation that causes retro orbital fat expansion2and extraocular muscle enlargement2and stiffening.3Because the orbital cavity is bony and of limited volume, proptosis and, in severe cases, optic nerve compression, can result. In many patients, muscle changes also cause ocular motility issues and double-vision. Because TED can have a similar presentation to other inflammatory orbital diseases (e.g., granulomatosis with polyangiitis) and Graves’ disease patients frequently have other autoimmune conditions (10% of Graves’s patients also have rheumatoid arthritis),4rheumatologists are likely to care for, or even diagnose, patients with TED.Objectives:This analysis sought to understand rheumatologists’ knowledge, and degree of participation in the treatment, of TED including referral patterns from ophthalmologists and endocrinologists for infusion therapies.Methods:Rheumatologists practicing in the United States attended an educational session and agreed to complete a 12-item survey regarding TED awareness, referral patterns, and management.Results:Of the 47 rheumatologists surveyed, 45 (96%) were familiar with TED. Ten (21%) physicians reported managing patients with TED, but the majority of physicians (62%) reported that they co-managed other autoimmune diseases in patients who also had TED. Additionally, 98% and 64% of polled rheumatologists had received referrals from ophthalmologists and endocrinologists, respectively, for autoimmune disease management or infusion therapy. Ophthalmology referrals for intravenous (IV) medication administration were most frequently for biologics (82%), but some referrals were also made for corticosteroids (2%) or other medication (13%) infusions. Only 23% of rheumatologists had administered a biologic specifically for TED (rituximab: 17%, tocilizumab: 2%, other: 4%), but 89% expressed an interest in administering a TED-specific monoclonal antibody therapy, awaiting FDA approval.Conclusion:Nearly all surveyed rheumatologists were aware of the signs and symptoms of TED, although most did not actively manage or administer medication for TED. Given the high level of interest in infusing novel, TED-specific biologics, rheumatologists may become an integral part of TED patient management with the approval of a new biologic, teprotumumab, for thyroid eye disease.References:[1]Bartley GB, et al.Am J Ophthalmol1996;121:284-90.[2]Forbes G, et al.AJNR Am J Neuroradiol1986;7:651-656.[3]Simonsz HJ, et al.Strabismus1994;2:197-218.[4]Cardenas Roldan J, et al.Arthritis2012 2012;864907.Disclosure of Interests:Brian LaMoreaux Shareholder of: Horizon Therapeutics, Employee of: Horizon Therapeutics, Megan Francis-Sedlak Shareholder of: Horizon Therapeutics, Employee of: Horizon Therapeutics, Robert Holt Shareholder of: Horizon Therapeutics, Employee of: Horizon Therapeutics, James Rosenbaum Consultant of: AbbVie, Corvus, Eyevensys, Gilead, Novartis, Janssen, Roche, UCB Pharma; royalties from UpToDate


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