Case Of Interstitial Granulomatous Dermatitis Research Articles

Reactive granulomatous dermatitis as a histological pattern including manifestations of interstitial granulomatous dermatitis and palisaded neutrophilic and granulomatous dermatitis: a study of 52 patients

Confusion exists regarding interstitial granulomatous dermatitis (IGD) and palisaded neutrophilic and granulomatous dermatitis (PNGD). To determine whether IGD and PNGD are two different entities, or whether they must be considered as two subtypes of the same reactive pattern, and thus whether the unification of the nomenclature is necessary. Observational retrospective multicentre study of patients with IGD and PNGD evaluated between 1999 and 2019 and review of their clinical and histological features. We identified 52 patients (38 women and 14 men). Clinical and histological findings of IGD were observed in 88.4% of cases. The most common cutaneous lesions were plaques/macules (IGD) or annular plaques and papules/nodules (PNGD), located mostly on the limbs and trunk. The rope sign was developed in two patients with IGD that associated autoimmune disorders. Similar associated comorbidities (75%) were found in both entities, mainly autoimmune diseases (53.8%). In IGD, the infiltrate was predominantly lympho-histiocytic. Neutrophilic infiltrates, karyorrhexis and skin lesions with limited clinical course were mainly associated with PNGD biopsies. In biopsies with a limited recurrent course, a predominant lymphocytic inflammatory infiltrate was found. Collagen degeneration was present in 75.9% of cases. The floating sign was observed only in IGD type patients (63%). Overlapping histological findings were found in one fourth of cases, especially between IGD and interstitial granuloma annulare. Interface dermatitis, apparently unrelated to drug intake, was observed in 4 cases of IGD. We support the term reactive granulomatous dermatitis to unify both the clinical and histological findings of IGD and PNGD, and the overlapping between IGD and interstitial granuloma annulare. According to this, a spectrum of histological changes will be found depending on the clinical course of the skin lesions.

Interstitial granulomatous dermatitis occurring in the patient with breast cancer: report of one case and a review on interstitial granulomatous dermatitis

Interstitial granulomatous dermatitis is a rare skin disorder in which there is a particular pattern of granulomatous inflammationassociated with rheumatologic diseases, hematologic disorders or underlying malignancies. The most common clinical findings ofinterstitial granulomatous dermatitis are multiple erythematous papules or plaques occurring on the varied sites.Histopathologically, it is characterized by dense perivascular and interstitial histiocytic infiltration around focally degeneratedcollagen fibers in the reticular dermis. We present a case of interstitial granulomatous dermatitis occurring in the patient withbreast cancer.

Interstitial Granulomatous Dermatitis in a Patient with Prostate Cancer

Interstitial granulomatous dermatitis (IGD) is a rare dermatosis, histologically characterized by an interstitial granulomatous infiltrate. It is associated with inflammatory arthritis, various medications, and autoimmune conditions. It is also associated with malignancies such as breast, endometrial, lung, and esophageal cancers as well as hematologic malignancies such as lymphoma and myelodysplastic syndrome. Herein we describe a case of IGD associated with prostate cancer, which has not been reported in the literature.

Interstitial Granulomatous Dermatitis Associated with Rheumatoid Arthritis.

Dear Editor: Interstitial granulomatous dermatitis (IGD) is a rare and peculiar disorder with cutaneous and joint manifestations1. IGD may be associated with rheumatologic and hematologic disorders of underlying malignancies2,3. Herein, we report a case of IGD associated with rheumatoid arthritis (RA). A 59-year-old Korean woman presented with multiple erythematous plaques on both extremities for 10 days (Fig. 1A). Upon physical examination, the plaques were linear shaped, non-tender, and palpable. She had been diagnosed with RA 17 years earlier. She had taken prednisolone, nonsteroidal anti-inflammatory drugs, and methotrexate to treat RA for 7 years. One month earlier, her arthritis symptoms had stabilized and the rheumatologist withdrew methotrexate. Laboratory test findings were non-specific except for an elevated C-reactive protein (CRP) level (7.12 mg/dl; normal range, 0.01~0.47 mg/dl) and erythrocyte sedimentation rate (ESR) (81 mm/h; normal range, 0~15 mm/h). Histopathologic findings indicated perivascular and interstitial lymphohistiocytic infiltration through the dermis and chronic granulomatous inflammation with collagen degeneration in the upper dermis. Immunohistochemically, most of the infiltrating inflammatory cells were CD 68-positive. Alcian blue staining revealed no mucin deposition in the areas of granulomatous inflammation (Fig. 2). Based on clinical and histological findings, she was diagnosed with IGD. A 7.5-mg methotrexate dose was given weekly and the daily prednisolone dose was increased from 5 mg to 15 mg. A topical 0.1% tacrolimus ointment and methylprednisolone cream were also applied daily for 2 months. The lesions were nearly cleared and ESR and CRP were found to be within normal limits after 2-month follow-up (Fig. 1B). Fig. 1 (A) Multiple, erythematous, palpable, and linear shaped plaques on both extremities. (B) Nearly clearing of lesions after a 2-month follow-up. Fig. 2 (A) A perivascular and interstitial lymphohistiocyte infiltration in the entire dermis (HE B: ×100, C: ×400). ... The most common clinical findings of IGD are asymptomatic multiple papules and plaques (70%~90% of cases)2. The rope sign was named for the linear prominent cutaneous cord-like lesions, considered pathognomic for IGD1. However, these lesions are not an essential feature, and are reported in only 9% of cases3. The histopathologic findings show interstitial CD 68-positive histiocyte infiltration around focally degenerated collagen2,4. Epidermal changes, mucin deposition, neutrophil and/or eosinophil infiltration, and vasculitis are usually absent 2. Differential diagnoses include interstitial granuloma annulare (IGA), interstitial granulomatous drug reaction (IGDR), and palisaded neutrophilic and granulomatous dermatitis (PNGD)1,2,4. In IGA, prominent mucin deposition and only focal interstitial infiltration could be found. Recent change of medication is present in IGDR, and IGDR presents with basal cell vacuolization and lichenoid changes with eosinophils2. Leukocytoclastic vasculitis and pandermal infiltration of neutrophil frequently present in early lesion of PNGD, and palisading granulomatous inflammation is the typical feature of fully developed one1. The pathogenesis of IGD may be associated with immune complex deposition caused by immune reactants of systemic inflammatory diseases5. Interestingly, IGD lesions in our case began to occur in association with the flare-up of RA after methotrexate withdrawal, although the causal relationship remains unknown. From our case we suggest that IGD should be considered among the differential diagnoses for patients with rheumatologic disease and cord-like skin lesions.

Interstitial granulomatous dermatitis: a characteristic histological pattern with variable clinical manifestations.

Interstitial granulomatous dermatitis (IGD) is a distinct histopathological pattern with a diverse clinical appearance which can be associated with autoimmune systemic diseases or, rarely, with drug-related adverse reactions. The IGD is a rare entity, first described in 1993 by Ackerman, which belongs to a group of non-infectious cutaneous granulomas [1]. The disease affects mostly women, at the mean age of onset at 58 years [2]. The precise etiopathogenesis remains unknown, but the underlying inflammatory process of the dermis and granulomatous infiltrate can be involved in the development of the disease. One hypothesis suggests that the depositions of immune complexes in the dermal vessels may be the trigger, which is then followed by complement and neutrophil activation and damage of dermal collagen, which in response gives rise to a granulomatous infiltrate [3]. We present a case of IGD that seems to be a distinct entity with variable clinical manifestations, which can be easily differentiated from other granulomatous skin diseases on the basis of histopathological examination. Although the disease occurs more frequently in adults, we present a case of IGD in a child.

Interstitial Granulomatous Dermatitis Associated with Myelodysplastic Syndrome – Complete Clearance Under Therapy with 5-azacytidine

Granulomatous skin diseases comprise a heterogenous spectrum of dermatoses that are characterised by a dermal infiltrate of predominantly histiocytes (1). Among this group, interstitial granulomatous dermatitis (IGD) is a term introduced by Ackerman in 1993 (2). It describes a rare form of granulomatous, non-infectious, reactive dermatosis that is frequently associated with rheumatoid arthritis (3, 4). However, several reports of drug-induced IGD and IGD associated with haematological malignan-cies exist as well (5–8). We report a case of IGD with un-derlying myelodysplastic syndrome (MDS). Successful treatment of MDS with 5-azacytidine led to a complete clearance of IGD, which might indicate a pathogenetic relationship of both diseases in this patient. To our know-ledge, only one previous report exists on the coincidence of IGD and MDS (9). Our report further suggests to screen for MDS or other haematologic malignancies in patients with IGD.CASE REPORT

A Child with Interstitial Granulomatous Dermatitis and Juvenile Idiopathic Arthritis

We describe a case of interstitial granulomatous dermatitis (IGD) with juvenile idiopathic arthritis (JIA) in an 11-year-old girl. She complained of erythematous plaques on her thighs and polyarthritis for 1year. Histopathologic examination revealed the features of IGD. This case indicates that IGD with arthritis can occur in children and should be considered in the differential diagnosis of cutaneous lesions associated with arthritis in children.

Interstitial granulomatous dermatitis: a distinct entity with characteristic histological and clinical pattern

Interstitial granulomatous dermatitis (IGD) is a rare disease for which a clinical-pathological correlation is essential to establish diagnosis. To describe the histological and clinical features of patients with IGD seen in our department from 2004 to 2010, and to undertake a literature review and critical analysis of additional cases. Twelve adult patients (nine women and three men; mean age 58·5 years; range 32-73 years) with IGD were enrolled. Lesions consisted of asymptomatic erythematous papules and plaques, symmetrically distributed on the trunk and the proximal limbs. Two patients had skin-coloured papules. Six patients had articular involvement (arthralgias, spondyloarthritis, rheumatoid arthritis) and three patients had cancer. All cases showed a predominant CD68-positive macrophage infiltrate distributed between collagen bundles of the mid- and deep dermis. Macrophages were also surrounding degenerated collagen fibres. A few neutrophils and/or eosinophils were also present. No vasculitis or significant mucin deposition was observed. Of the 62 cases of IGD reported since 1993, 53 fulfilled stringent diagnostic criteria. Erythematous papules and plaques on the trunk and proximal limbs were the dominant manifestation. Approximately 10% of patients had cord-like lesions. More than 50% of patients with IGD had arthralgia or arthritis, and less commonly other rheumatic disorders. Disease duration is months to years, but long-term prognosis seems favourable. IGD is a distinct entity with a typical histological and clinical pattern. The importance and the nature of the association with extracutaneous diseases remains to be clarified. Patients should be screened for rheumatic and autoimmune diseases.

Lupus attributable to anti-TNF therapy and revealed by interstitial granulomatous dermatitis

Interstitial granulomatous dermatitis belongs to the group of aseptic cutaneous granulomas. It is a histopathological entity encountered in various pathological situations, such as polyarthritis including rheumatoid arthritis, but also systemic lupus erythematosus. It may also occur after systemic administration of medication, thus representing a drug-induced, interstitial granulomatous outbreak. This has recently been described after anti-TNF therapy was taken. We are reporting the case of a patient treated using adalimumab for rheumatoid arthritis and having developed interstitial granulomatous dermatitis during treatment, which revealed lupus erythematosus attributable to the biotherapy. The clinical appearance of interstitial granulomatous dermatitis can vary, and the diagnosis is confirmed by anatomo-pathological examination. Drug-induced interstitial granulomatous outbreaks have specific histological criteria, and secondary cases involving anti-TNF medication have been described. Cases of lupus attributable to anti-TNF therapy have also been described, and they have specific biological characteristics. Like idiopathic lupus, they may be associated with interstitial granulomatous dermatitis, but the association of an anti-TNF-induced lupus and this type of granulomatous has not, to our knowledge, been described before. We are reporting one case, which emphasises the importance of carrying out a complete and systematic aetiological assessment for all cases of interstitial granulomatous dermatitis, including where there is systemic disease or following medical treatment, either of which may provide an evident cause for the granulomatosis. In particular, the outbreak of interstitial granulomatous dermatitis during anti-TNF treatment should lead to screening for a drug-induced lupus, which would require the patient to stop such treatment.

Interstitial granulomatous dermatitis in rheumatoid arthritis responsive to etanercept

Interstitial granulomatous dermatitis (IGD) is a rare dermatological condition presenting as erythematous plaques. It may be associated with drug-related adverse reactions and autoimmune diseases. Recent cases of IGD have been reported in rheumatoid arthritis (RA) patients treated with biologic agents. We report a case of RA patient with persistent erythematous plaques who did not respond to traditional disease-modifying anti-rheumatic drugs with a persistent skin condition of erythematous plaque eruptions. A biopsy showed a homogeneous inflammatory infiltrate in the deep dermis composed of large epithelioid histiocytes with occasional granulocytes, leading us to consider a diagnosis of IGD. The cutaneous lesions disappeared after a 3-month treatment with the tumour necrosis factor-alpha (TNF-alpha) inhibitor etanercept. Anti-TNF-alpha agents can antagonise the multiple effects of TNF-alpha on the immune system, effects that are required for the continued maintenance of granulomatous structure, and offer a therapeutic strategy in the treatment of IGD associated with arthritis.

Interstitial granulomatous dermatitis: a misdiagnosed cutaneous form of systemic lupus erythematosus?

Interstitial granulomatous dermatitis (IGD) is a recently described, rare dermatological entity. The clinical features are diverse and the precise aetiology is unknown. We present a rare and atypical case of IGD in a patient with systemic lupus erythematosus (SLE). A 26-year-old woman had been diagnosed with SLE when she was 15 years old. The diagnosis was based on cutaneous, articular, pulmonary, haematological and immunological features. The patient presented with a cutaneous diffuse macular eruption on the limbs, appearing in a cockade (rosette) pattern with a violaceous centre and erythematous surround. The face and trunk were spared. The cutaneous histological features led us to consider a diagnosis of IGD. The lesions disappeared after 15 days of systemic steroid therapy. This case is a new clinical form of IGD with an atypical location and clinical presentation. IGD has usually been associated with drug-related adverse reactions and autoimmune diseases. Reports in the literature of IGD in patients with SLE are rare.

Direct Immunofluorescence Results of a Case of Railway Track Dermatitis

A 40 year‐old female with sero‐negative arthritis presented with annular and cord‐like configurations (rope sign) of asymptomatic papules and plaques along her flanks, suspected to be granuloma annulare. Medications at the time of presentation included methotrexate, prednisone, Vioxx (rofecoxib), Arava (leflunomide) and Remicade (infliximab). High‐potency topical corticosteroids did not alleviate the eruption. Biopsy was performed demonstrating a deep dermal, interstitial and focally palisading granulomatous infiltrate with eosinophils. Dermal lymphangiectases were present, and the epidermis was unaffected. Direct immunofluorescence (DIF) examination showed deep dermal, interstitial IgG, C3, and IgM deposits coating collagen bundles centered in the granulomatous infliltrate and IgG antinuclear keratinocyte reactivity. Serologic tested also revealed anti‐nuclear antibodies. No other auto‐antibodies were detected. Based on these findings, this patient was diagnosed with “interstitial granulomatous dermatitis (IGD) with cords and arthritis”. Thirteen cases of IGD with cords were identified in a literature review of which 7/12 (58%) were sero‐(rheumatoid factor)‐negative, 3/5 (60%) were anti‐nuclear antibody positive, and 12/13 (92%) had arthritis. One theory of pathogenesis is that immune‐complex deposition in the dermis initiates a granulomatous reaction; DIF findings in this case support this proposed mechanism. IGD with cords is a singular clinicopathologic entity associated with sero‐negative, non‐erosive arthritis and antinuclear antibodies.

Interstitial granulomatous dermatitis with arthritis

Interstitial granulomatous dermatitis with arthritis is an uncommon disorder. In its original description, the presence of linear inflammatory indurations on the lateral aspects of the trunk (the rope sign) in association with arthritis were considered the pathognomonic clinical features. Later cases presenting with plaques and papules have been reported. Herein we describe a case of interstitial granulomatous dermatitis with arthritis without the rope sign. The present case supports the idea that interstitial granulomatous dermatitis with arthritis may have variable clinical appearances.

Arthritis and interstitial granulomatous dermatitis (Ackerman syndrome) with pulmonary silicosis

Objective: To describe the case of a patient suffering from pulmonary silicosis associated with a rheumatoid factor negative, antinuclear antibody positive, symmetrical, nonerosive synovitis, and interstitial granulomatous dermatitis (IGD) and compare it with similar cases reported in the literature. Methods: Literature search to identify published cases of IGD with arthritis and cases associated with silicosis. Results: Thiry-eight cases of IGD with arthritis were found. These cases were associated with various conditions such as drug reactions, autoimmune diseases, chronic infections, or paraneoplastic syndromes for which no specific underlying cause was identified. The patient had skin lesions corresponding to the rope sign, as noted in other reports. Histology showed a histiocytic, granulomatous dermatitis, which, in association with arthritis, was first described by Ackerman et al. Circulating immune complexes or altered apoptosis have been discussed as mechanisms, although there is no experimental evidence to support either hypothesis. As in other cases, treatment had limited success. Most relief was achieved with hydroxychloroquine, dapsone, and corticosteroids. Conclusions: Arthritis and IGD associated with silicosis is a rare clinical entity that can be differentiated from other conditions. This condition should be considered when patients present with typical dermatologic features, such as the rope sign, and arthritis. Semin Arthritis Rheum 32:334-340. Copyright 2003, Elsevier Inc. All rights reserved.