Palisaded Neutrophilic Granulomatous Dermatitis Research Articles

Beyond rheumatoid nodules in rheumatoid arthritis.

Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease predominantly affecting synovial joints. Extra-articular manifestations, including skin involvement, can also occur. The most frequent cutaneous manifestation in RA patients is rheumatoid nodules, occurring in 20-30% of seropositive individuals. These nodules are typically firm, painless, and located on pressure points such as the hands and elbows. However, in a minority of cases, other skin conditions can complicate RA, notably palisaded neutrophilic granulomatous dermatitis (PNGD). PNGD presents as erythematous papules or plaques, often pruritic and distributed symmetrically over extensor surfaces, making it challenging to differentiate from rheumatoid nodules. Histopathological examination is crucial to establish the diagnosis. High clinical suspicion and appropriate referral to Dermatology are essential for accurate diagnosis and management. Treatment of PNGD is focused on underlying disease control. Other options include topical, intralesional or systemic corticosteroids, dapsone or hydroxychloroquine. Herein, we present the case of a 71-year-old woman with RA who developed PNGD, highlighting the importance of a multidisciplinary approach for achieving a favorable clinical outcome.

Interstitial Granulomatous Dermatitis and Palisaded Neutrophilic Granulomatous Dermatitis: Retrospective Clinicopathological Analysis of 16 Cases.

Reactive granulomatous dermatitis (RGD) is a new entity, which is highly associated with systemic disorders. There is scarce data regarding interstitial granulomatous dermatitis (IGD) and palisaded neutrophilic granulomatous dermatitis (PNGD). We aimed to evaluate clinical and histopathological characteristics of IGD and PNGD as unified entities under the term of RGD. Observational, retrospective, single-center study of patients diagnosed with IGD and PNGD between 2012 and 2021 were included in the study. Of 16 patients (14 females and 2 males) with RGD, 13 had IGD and 3 had PNGD with a mean age of 62.5 years. The most common clinical presentation was plaques 37.5% (N=6), followed by patches 25% (N=4). The most common localization of involvement was lower extremity 75% (N=12), followed by trunk and upper extremity. Multiple localization of involvement was determined in 75% (N=12) of patients. None of the patients had rope sign. Associated comorbidities such as autoimmune diseases and malignancies were detected in 68.7% (N=11) of patients. In majority of biopsies (87.5%; N=14), there were lymphohistiocytic cell infiltration. Other accompanying cells were scarce neutrophils 31.2% (N=5) and eosinophils 31.2% (N=5). All of the biopsies had interstitially located lymphohistiocytic cell infiltration surrounding with swollen and degenerated collagen. Palisaded pattern was determined in 18.7% (N=3) of patients and floating sign was seen in 18.7% (N=3) of biopsies. RGD is a rare entity and most patients with RGD had associated disorders such as autoimmunity or malignancy. There is overlapping between IGD and PNGD, therefore supporting the usage of umbrella term as reactive granulomatous dermatitis is compatible with the literature.

Underlying Systemic Diseases in Interstitial Granulomatous Dermatitis and Palisaded Neutrophilic Granulomatous Dermatitis: A Systematic Review

Background: Interstitial granulomatous dermatitis (IGD) and palisaded neutrophilic granulomatous dermatitis (PNGD) are uncommon presentations of reactive granulomatous dermatitis. Histologic lesions characterized by IGD/PNGD patterns have been associated with systemic diseases, causing an unmet need for revealing clinical correlates. Objective: The aim of this study was to unravel the systemic diseases beyond dermatitis of IGD/PNGD. Methods: This study analyzed data from case studies, case series, and retrospective cohorts by searching PubMed, Embase, Web of Science, and the Cochrane Library, with no start date or language restrictions on Sep 4, 2021. Results: One hundred ninety-six publications were included (458 cases in total, 216 with details). Systemic diseases associated with IGD/PNGD were classified into 5 groups. Autoimmune disorders (n = 103, 47.6%) including rheumatoid arthritis (n = 51, 23.6%), systemic lupus erythematosus (n = 20, 9.3%), and others were the most common across all underlying diseases, followed by drug eruption (n = 52, 24.1%) such as tumor necrotic factor inhibitor reaction (n = 18, 8.3%) and malignancies (n = 27, 12.5%) such as hematologic malignancy (n = 20, 9.3%). The rest were infectious diseases (n = 12, 5.6%) and accidental conditions (n = 3, 1.4%). Conclusion: IGD/PNGD might be associated with autoimmune disorders, drug eruption, malignancies, infectious diseases, and accidental conditions. Patients with IGD/PNGD need further follow-up.

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

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare histopathological pattern belonging to a group of cutaneous granulomatous eruptions that typically manifests with asymptomatic skin-colored, erythematous, or violaceous papules or nodules. PNGD can be triggered by various systemic conditions, including medications and autoimmune and autoinflammatory disorders, as well as malignancies; for example, lymphoproliferative disorders. Therefore, in patients with PNGD an extended diagnostic workup is mandatory as well as follow-up in the case of idiopathic PNGD. To the best of our knowledge, this is the first reported case in the literature of PNGD causally related to a relapse of HLA-B27-negative axial spondyloarthritis.

Bowel-associated dermatosis arthritis syndrome and palisading neutrophilic granulomatous dermatitis as presentation of ulcerative colitis

article: Bowel-associated dermatosis arthritis syndrome and palisading neutrophilic granulomatous dermatitis as presentation of ulcerative colitis - Italian Journal of Dermatology and Venereology 2021 December;156(Suppl. 1 to No. 6):7-8 - Minerva Medica - Journals

Extensive Palisaded Neutrophilic Granulomatous Dermatitis with Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a multi-system disease with a myriad of mucocutaneous and systemic findings. One of the atypical cutaneous manifestations is palisaded neutrophilic granulomatous dermatitis (PNGD). This uncommon condition presents as tender or asymptomatic, flesh-colored, red to violaceous subcutaneous nodules. The diagnosis may be suspected clinically but is confirmed by biopsy. The impact of the disease may be the direct result of pain, psychosocial, cosmetic concerns, or be the initial presentation of an underlying systemic disease. We present a patient with known SLE who developed PNGD. We also review similar clinical and microscopic disease entities with a summative comparison of neutrophilic dermatoses in patients with autoimmune connective tissue diseases.

Palisaded neutrophilic granulomatous dermatitis: Case report and review of literature

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare inflammatory dermatosis. We report a 45-year-old female with a 2-year history of asymptomatic persistent skin lesions. The patient had rheumatoid arthritis well controlled with abatacept, methotrexate, and hydroxychloroquine. Multiple nonscaly umbilicated papules with crusted center were scattered on her abdomen, chest wall, and back. Biopsy showed pandermal infiltrate of neutrophils and areas of basophilic degenerated collagen surrounded by palisades of histiocytes, neutrophils deep in the dermis, and subcutaneous fat. A diagnosis of PNGD was made. The patient was treated with betamethasone dipropionate (0.05%) ointment and oral colchicine with good improvement.

Bowel-associated dermatosis arthritis syndrome and palisading neutrophilic granulomatous dermatitis as presentation of ulcerative colitis

article: Bowel-associated dermatosis arthritis syndrome and palisading neutrophilic granulomatous dermatitis as presentation of ulcerative colitis - Giornale Italiano di Dermatologia e Venereologia 2019 Mar 29 - Minerva Medica - Journals

Palisaded Neutrophilic Granulomatous Dermatitis Associated with Adult Onset Still's Disease

A 66-year-old male presented with a new onset of rash. The rash was erythematous, macular and localized to the shoulders and neck. Biopsy showed palisading neutrophilic granulomatous dermatitis with features of atypical granuloma annulare tissue. Improvement was noted with steriods. To our knowledge, there has only been one reported case of PNGD in a patient with AOSD. It is diagnosed with skin biopsy and historically glucocorticoids have been the mainstay of treatment. However, clinicians should have a high suspicion of PNGD in patients with AOSD who present with features of atypical rash, as treatment may be required. This association is extremely rare, since this is only the second reported case. We strongly believe that to further delineate different forms of PNGD and rare instances such as in patients with AOSD, further case reports are needed. Additional research into the pathophysiology of PNGD is also warranted, as this would optimize medical management.

Interstitial Granulomatous Dermatitis: A Clinicopathological Study.

Interstitial granulomatous dermatitis (IGD) is an uncommon granulomatous dermatitis occurring in the setting of highly reactive immune states, with a polymorphic clinical presentation. Because there is overlap with other entities [namely palisading neutrophilic granulomatous dermatitis (PNGD)], controversy exists regarding its classification. To understand if there are features allowing clear-cut distinction between IGD and PNGD. Retrospective analysis of 10 cases previously diagnosed as IGD or PNGD, from 2000 to 2013, with review of the histopathologic findings and clinical correlation. Six females and 4 males presented mostly with erythematous papules/nodules (n = 7) but also with erythematous annular plaques (n = 3). In 2 patients, the lesions coexisted. They were mostly distributed symmetrically on the limbs. Associated disease was observed in 6 patients. Regarding histopathology, an inflammatory infiltrate occupying the superficial and mid dermis was present in 40% of cases, with an interstitial component in all biopsies and a palisaded arrangement in 60%. Neutrophils and mononuclear cells were present in all cases in varying proportions. Necrobiosis was found in 70%, and leukocytoclasia was observed in 80% of biopsies. Coexistence of the interstitial and palisaded inflammatory patterns occurred in 90% of cases, with no correlation between tissue neutrophilia and the predominant pattern of the infiltrate. There was also no clear-cut correlation between the infiltrate pattern and semiologic aspect of the lesions. Therefore, the features described in our study suggest that IGD and PNGD belong to the same clinicopathological spectrum.

Palisaded Neutrophilic and Granulomatous Dermatitis Associated With Ankylosing Spondylitis

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare neutrophilic dermatosis that shows a broad clinical and histopathological spectrum. The clinical presentation of PNGD varies from asymptomatic papules, to nodules, to annular plaques. The most common clinical presentation is erythematous papules on the extensor surface of extremities, especially fingers and elbows. Histopathological findings demonstrate a spectrum of changes that reflect the evolution of the lesions, from only sparse mixed perivascular infiltrates to interstitial or palisaded granuloma formation with dermal fibrosis. The cause of PNGD is unknown, but there is a prominent association with systemic conditions, particularly with autoimmune diseases, representing rheumatoid arthritis and systemic lupus erythematosus, the most common associations. It has also been described associated to systemic sclerosis, sarcoidosis, systemic vasculitis, inflammatory bowel disease, and lymphoproliferative disorders, and also drugs have been implicated. Recently, it has been described the potential association with tumor necrosis factor alpha inhibitors. This dermatosis should be considered as a marker of systemic disease and particularly it should be considered in patients with history of autoimmune disorders who present with papular eruptions on the extremities.

Palisaded neutrophilic granulomatous dermatitis without a definable underlying disorder treated with dapsone

To the Editor: Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare neutrophilic dermatosis that characteristically presents in patients with a definable underlying connective tissue disease, vasculitis, or malignancy.1 We present a case of PNGD in a patient with no definable autoimmune disorder or malignancy, whose cutaneous manifestations, fevers, and joint symptoms responded well to dapsone.

Palisaded neutrophilic granulomatous dermatitis with leukocytoclastic vasculitis in a patient without any underlying systemic disease detected to date

Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare dermatologic condition which shows various clinical and histopathological features. Although the PNGD lesions have been suggested to begin as leukocytoclastic vasculitis (LCV), there is still insufficient clinicopathological information in the reported cases of PNGD in acute stage with LCV. The relationship between PNGD and interstitial granumatous dermatis (IGD) also remains unclear. This report presents the case of a 60-year-old female patient with multiple erythematous nodules on the extremities. She had no underlying systemic disease detected to date, although transient, abnormal liver function tests were seen. The histopathological examination of an erythematous nodule revealed the features of PNGD in the acute stage. The patient presented the characteristic features of LCV including palisaded granulomatous pattern, and the interstitial granulomatous pattern was seen together, suggesting that PNGD with LCV can show an interstitial granulomatous pattern. The present case also suggested that PNGD in the acute stage with LCV tends to clinically manifest as erythematous nodules on the extremities and histopathologically shows a remarkable papillary edema and an extensive fibrin deposition in and around the vessel wall. PNGD may be associated with transient liver dysfunction.

Palisaded neutrophilic granulomatous dermatitis associated with systemic lupus erythematosus presenting with the burning rope sign

Palisaded neutrophilic granulomatous dermatitis is a rare but increasingly recognized cutaneous manifestation of connective tissue disorders. It is reported most commonly with rheumatoid arthritis but also occasionally in association with systemic lupus erythematosus, inflammatory bowel disease, lymphoproliferative disorders, and systemic vasculitides. The clinicopathological presentation is highly variable, which has led to suggestions that it encompasses a number of distinct diseases. Most previous cases have reported only a single clinical and histologic manifestation of the condition within an individual. Here, we present a case of systemic lupus erythematosus-associated palisaded neutrophilic granulomatous dermatitis in which a striking evolution of both clinical and histologic features was observed during the course of 7 years, providing compelling evidence for the proposal that palisaded neutrophilic granulomatous dermatitis represents a disease spectrum rather than separate disease entities.

Skin manifestations of rheumatoid arthritis: a study of 215 Turkish patients

To investigate the frequency and clinicopathological features of skin involvement in rheumatoid arthritis (RA), to find out whether early and aggressive disease-modifying treatment is changing the spectrum towards a milder disease pattern. Two hundred and fifteen consecutive RA patients were enrolled. Main outcome measures were the frequency of cutaneous features and their relation to disease severity and treatment modality, ultrasound (USG) examination of nodules, histopathological examination of nodules and papules. Mean age and disease duration were 55.3 years and 138.1 months, respectively. Sixty-six percent of the patients had erosive disease, 70% were rheumatoid factor (RF) positive and 44% had Classes III and IV functional index. Among 43 patients having nodules, 20 were diagnosed as rheumatoid nodules (RNs) and the others as synovitis, bursitis, and so on, on clinical basis and by ultrasound. In 7 of 20 RNs, diagnosis was confirmed by histopathological examination. When clinical, histopathological, and USG data were analyzed collectively, sensitivity, and specificity of USG were found as 100 and 75%, respectively. Sixteen patients had relevant papular lesions. Fourteen of these were diagnosed as palisading neutrophilic granulomatous dermatitis (PNGD) and two as rheumatoid neutrophilic dermatitis (RND) on histopathological examination. Accelerated nodulosis was seen in one, and vasculitis in two of the patients. We observed a milder disease pattern compared to Anglo-Saxon countries with lower RN and vasculitis frequency. This cannot be explained by early and aggressive treatment as disease onset to treatment interval was long in our patient group. PNGD resembles RN in terms of frequency and association with severe disease.

Cutaneous granulomas in rheumatoid arthritis

A 57-year-old man with rheumatoid arthritis and myasthenia gravis developed an asymptomatic erythematous plaque on his nose and skin-colored nodules on the elbows. Histopathological examination was suggestive of palisaded neutrophilic granulomatous dermatitis (PNGD). PNGD is a rather uncommon cutaneous finding in different collagen vascular disorders that can manifest with a broad range of clinical symptoms and histological signs. Based on the case report of the patient described here, we briefly discuss what is currently known about this disease.

Palisading Neutrophilic Granulomatous Dermatitis in a Japanese Patient with Wegener's Granulomatosis

Wegener's granulomatosis (WG) is an etiologically obscure entity with multiple systemic manifestations. Recently, cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) has become recognized as a valuable adjunct in the diagnosis of this disorder. WG typically involves the upper airway, lungs, and kidneys, but any other organ can be involved, including the skin. We encountered a unique case in which a 27-year-old Japanese man with WG presented with various typical cutaneous manifestations. Purpuric skin lesions and erythematous rash on the lower extremities progressively involved and changed into a necrotizing ulceration on his toe. Additionally, several nodules developed on the extensor surfaces of his elbows. His serum C-ANCA level increased remarkably. Leukocytoclastic vasculitis, the most common histopathological finding in WG patients, was detected in a purpuric lesion on his hand. A biopsy of a nodule on his elbow revealed palisading epithelioid histiocyte granulomas with features of leukocytoclastic vasculitis. The distinctive pattern of papules has been referred to as "palisading neutrophilic granulomatous dermatitis". An open lung biopsy confirmed WG with focal necrotizing granuloma. A renal biopsy demonstrated necrotizing vasculitis and crescentic glomerulonephritis. He showed a good response to oral corticosteroids and cyclophospamide with total remission of symptoms. We believe that a careful balance between the clinical manifestations and the histopathological evidence allows for timely treatment of WG, which may prevent serious morbidity or death. Although uncommon, WG can present with various types of accompanying cutaneous lesions. Therefore, clinicians should keep this diagnosis in mind when presented with these manifestations.

Palisaded neutrophilic granulomatous dermatitis in rheumatoid arthritis

Palisaded neutrophilic granulomatous dermatitis (PNGD) is an entity that has not been clearly defined either clinically or histopathologically. It is seen in patients with rheumatoid arthritis and other connective tissue diseases. In the past, many cases of PNGD have been described under several different names including palisaded neutrophilic and granulomatous dermatitis, linear subcutaneous bands, interstitial granulomatous dermatitis with cutaneous cords and arthritis, rheumatoid papules, and Churg-Strauss granuloma. We report 7 additional cases of PNGD. Clinically, 6 patients presented with erythematous to violaceous plaques, papules, and nodules on multiple body sites; one presented with subcutaneous linear bands on the shoulder. Five had rheumatoid arthritis; one had adult-onset Still's disease; and one showed clinical signs of rheumatoid arthritis, although serologically the rheumatoid factor was negative. On histologic examination, a spectrum of changes was observed ranging from urticaria-like infiltrates to leukocytoclastic vasculitis and granuloma annulare with neutrophils. We report these cases to expand the histologic spectrum of this entity and to further delineate the different forms of clinical presentation. (J Am Acad Dermatol 2002;47:251-7).