Neutrophilic Granulomatous Dermatitis Research Articles

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.

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

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.

26441 Palisaded and neutrophilic granulomatous dermatitis in a child

Palisaded and neutrophilic granulomatous dermatitis (PNGD) is a rare entity that occurs in conjunction with autoimmune diseases as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), juvenile idiopathic arthritis, sarcoidosis, and mixed connective tissue disease. Autoimmune diseases in pediatric patients can be challenging to diagnose. We present the first pediatric case of PNGD associated with SLE. A 10-year-old girl with a history of atopic dermatitis, asthma, and a family history of SLE and RA presented to the ED with palpable purpura of the lower extremities and arthralgias and was diagnosed clinically with Henoch- Schoenlein purpura.

AB009. Successful treatment of severe systemic lupus erythematosus and psoriasis with IL-17A inhibition

The coexistence of psoriasis and systemic lupus erythematosus (SLE) is uncommon, with prevalence estimated at 0.5–5%. In this case, a 20-year-old woman with a history of severe SLE complicated by lupus nephritis and mesenteric vasculitis presented with a new rash on the face, scalp, and forearms. Skin biopsy revealed features of psoriasis within the epidermis, in addition to the palisaded neutrophilic granulomatous dermatitis within the dermis explained by her underlying SLE. Treatment options were considered in view of her possible cytokine profile and predominant inflammatory pathway. The Th17 pathway has been implicated as a common immune pathway in both diseases, leading to cytokine production including the proinflammatory cytokine interleukin-17A (IL-17A). Studies have shown increased level of IL-17A in the serum and involved tissues of patients with SLE as well as the biopsied plaques of psoriasis. Randomized control trials have shown efficacy of IL-17A inhibition in decreasing the severity and extent of psoriasis, and case reports have noted improvement in SLE activity scores in patients who had been refractory to other management strategies. This patient was treated with IL-17A inhibition, which dramatically improved her rash and allowed complete tapering of her baseline immunosuppressive regimen while maintaining SLE quiescence. This case demonstrates that there may be a subset of SLE patients who respond to IL-17A inhibition, particularly in the setting of overlap with diseases in which IL-17A inhibition has previously shown efficacy. Teaching point: IL-17A inhibition may be effective treatment for SLE overlapping with inflammatory diseases in which IL17A is known to play a role including psoriasis, seronegative spondyloarthropathies, and rheumatoid arthritis.

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.

P38 A rare case of rheumatoid arthritis with perforating collagenosis

Abstract Background Rheumatoid arthritis has multiple cutaneous manifestations with rheumatoid nodule being the commonest one. A maculopapular rash in setting of rheumatoid arthritis has a varied list of differentials including rheumatoid vasculitis, pyoderma gangrenosum, rheumatoid neutrophilic dermatosis, interstitial granulomatous dermatitis and palisaded neutrophilic granulomatous dermatitis. We present a rare case of patient with rheumatoid arthritis with perforating collagenosis. Methods A case report is presented in the results section. Results A 64 year old lady known to have seropositive rheumatoid arthritis for the past 6 years with poor compliance to treatment presented with maculopapular lesions on both her legs and abdomen. The lesions were itchy in nature and later became ulcerative. The lesions spread to various parts of the body over the next 4 weeks and patient was seen by a dermatologist who initially prescribed topical therapy and the patient came off her disease modifying anti rheumatic agents. She underwent a skin biopsy as initial investigations were otherwise unremarkable. Skin biopsy revealed a diagnosis of perforating collagenosis. She was given topical steroids and counselled regarding the need to take her disease modifying anti rheumatic drugs regularly. There was 95% resolution of skin lesions within 6 months. Conclusion Perforating collagenosis is a skin disorder characterised by transepithelial expulsion of dermal tissue which presents as a papulonodular rash with central keratin plugs. It is usually associated with diabetes mellitus and chronic kidney disease but rarely been reported in association with rheumatoid arthritis. Literature review revealed that there have been only 2 cases reported previously. The first case has been described in 2010 in a man from Singapore on treatment for RA for 2 years. Second case has been described by Ikeda et al in 2019 in a case of rheumatoid vasculitis. Clinically it presents as umbilicated, hyperkeratotic papules with a central white keratotic crust. Koebnerization may be seen and itching of surrounding regions may lead to papules to coalesce and form plaques. A biopsy will reveal features similar to those seen in the primary disease where perforating collagenosis shows cup shaped invagination of the epidermis filled with a plug consisting of keratin and cellular debris. There have been no trials and treatment is based on case reports. We believe that better compliance to her disease modifying anti rheumatic agent therapy, leading to improved treatment of the systemic cause had a significant role in resolving her skin lesions. Disclosures S. Deb None. S. Srirangan None.

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.

Palisaded neutrophilic granulomatous dermatitis as manifestation of Hodgkin lymphoma: Report of a rare case with literature review of paraneoplastic granulomatous dermatitis

Palisaded neutrophilic granulomatous dermatitis as manifestation of Hodgkin lymphoma: Report of a rare case with literature review of paraneoplastic granulomatous dermatitis

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

Skin manifestations of rheumatoid arthritis.

Rheumatoid arthritis is a chronic systemic disease that, in addition to articular involvement, may exhibit a variety of extraarticular manifestations. The skin is frequently involved mainly in the most severe forms of the disease. Rheumatoid nodules, accelerated rheumatoid nodulosis, rheumatoid nodulosis, Felty syndrome, rheumatoid vasculitis, pyoderma gangrenosum, rheumatoid neutrophilic dermatosis, interstitial granulomatous dermatitis, and palisaded neutrophilic granulomatous dermatitis are reviewed both clinically and histologically. Moreover, cutaneous reactions related to treatments of rheumatoid arthritis, in particular to biologic agents, are described. Early recognition of these entities through a clinical and histological correlation permit to optimize the management of patients.

Interstitial Granulomatous Dermatitis (IGD).

We report the case of a 42 years old male patient suffering from skin changes, which appeared in the last 7-8 years. Two biopsies were performed during the evolution of the lesion. Both showed similar findings that consisted in a busy dermis with interstitial, superficial and deep infiltrates of lymphocytes and histiocytes dispersed among collagen bundles, with variable numbers of neutrophils scattered throughout. Some histiocytes were clustered in poorly formed granuloma that included rare giant cells, with discrete Palisades and piecemeal collagen degeneration, but without mucin deposition or frank necrobiosis of collagen. The clinical and histologic findings were supportive for interstitial granulomatous dermatitis. Interstitial granulomatous dermatitis (IGD) is a poorly understood entity that was regarded by many as belonging to the same spectrum of disease or even synonym with palisaded and neutrophilic granulomatous dermatitis (PNGD). Although IGD and PNGD were usually related to connective tissue disease, mostly rheumatoid arthritis, some patients with typical histologic findings of IGD never develop autoimmune disorders, but they have different underlying conditions, such as metabolic diseases, lymphoproliferative disorders or other malignant tumours. These observations indicate that IGD and PNGD are different disorders with similar manifestations.

Palisaded Neutrophilic Granulomatous Dermatitis in a Patient with Systemic Sclerosis-Rheumatoid Arthritis Overlap Syndrome

Palisaded Neutrophilic Granulomatous Dermatitis in a Patient with Systemic Sclerosis-Rheumatoid Arthritis Overlap Syndrome

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.

Palisaded and Neutrophilic Granulomatous Dermatitis in a Patient with Dermatomyositis and Nasopharyngeal Cancer

Palisaded and Neutrophilic Granulomatous Dermatitis in a Patient with Dermatomyositis and Nasopharyngeal Cancer

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.

Koebner Phenomenon in Rheumatoid Arthritis

Koebner phenomenon indicates the newly appearance of isomorphic lesions at the sites of mechanically stimulated or injured skin. This phenomenon can be seen in various inflammatory, auto-immune, viral, fibrotic, and even tumoral disorders. Also, rheumatic diseases such as lupus erythematosus, dermatomyositis, and rheumatoid arthritis (RA) often present with cutaneous manifestations with isomorphic response of Koebner. RA presents with various skin conditions as extra-articular manifestations. Rheumatoid nodule is the representative specific skin lesion which frequently occurs on the hand, elbow, sole, sacrum, occipital area, and so on. These sites are susceptible to both outer and inner mechanical stress. Rheumatoid nodules involve not only skin but internal tissues such as spine, lung, heart valves, and gastrointestinal tract, which are also susceptible to mechanical stress. This isomorphic response may be induced at deeper levels than skin, and thus considered to be “deep” or “internal” Koebner phenomenon. Other than rheumatoid nodules, several specific skin conditions are associated with RA, such as palisaded neutrophilic granulomatous dermatitis and rheumatoid neutrophilic dermatitis, which can be seen on the fingers, elbows, knees and sole. Also, there are various other skin lesions in association with RA, which show Koebner phenomenon, such as neutrophilic dermatosis and autoimmune bullous diseases. Koebner phenomenon may be closely associated with the induction of various specific or nonspecific conditions, not restricted to the skin, in association with RA.

Palisaded neutrophilic granulomatous dermatitis caused by cellulitis in a patient with systemic lupus erythematosus

Palisaded neutrophilic granulomatous dermatitis caused by cellulitis in a patient with systemic lupus erythematosus