Clinical Characteristics of Erythema Nodosum and Associations With Chronicity and Recurrence.

This cohort study characterizes the presentation, causes, treatment, and disease course of erythema nodosum, as well as identifies associations with chronicity and recurrence.

Idiopathic Granulomatous Mastitis Associated with Erythema Nodosum in a Pregnant Woman.

A 32-year-old woman at 17 weeks' gestation presented with fever and a 1-week history of an acute nodular eruption involving her legs, along with bilateral ankle and knee pain. She also had had a recurrent right breast abscess for 2 months for which she had been treated with oral antibiotics and surgical drainage, but with slight improvement. Cultures of the abscess showed no bacteria or fungi. She had no history of tuberculosis, sarcoidosis, trauma to the breast, or a family history of breast pathology. Cutaneous examination revealed multiple, tender, erythematous, subcutaneous nodules on her legs (Figure 1) and an ill-defined tender mass involving the inferior quadrant of the right breast without nipple discharge or retraction. There was a scar with drainage on her right breast (Figure 2). There were no regional lymphadenopathies. Left breast and left axilla examination was unremarkable. Significant laboratory findings included an erythrocyte sedimentation rate of 54 mm/hour (normal levels [NL] < 20 mm/hour), an elevated C reactive protein at 148 mg/L (NL < 5 mg/L), and a high level of white blood cells averaging 15,000 elements/mm3 (NL < 10,000/mm3).

Cutaneous manifestations of inflammatory bowel disease: basic characteristics, therapy, and potential pathophysiological associations.

Inflammatory bowel disease (IBD) is a chronic inflammatory disease typically involving the gastrointestinal tract but not limited to it. IBD can be subdivided into Crohn's disease (CD) and ulcerative colitis (UC). Extraintestinal manifestations (EIMs) are observed in up to 47% of patients with IBD, with the most frequent reports of cutaneous manifestations. Among these, pyoderma gangrenosum (PG) and erythema nodosum (EN) are the two most common skin manifestations in IBD, and both are immune-related inflammatory skin diseases. The presence of cutaneous EIMs may either be concordant with intestinal disease activity or have an independent course. Despite some progress in research on EIMs, for instance, ectopic expression of gut-specific mucosal address cell adhesion molecule-1 (MAdCAM-1) and chemokine CCL25 on the vascular endothelium of the portal tract have been demonstrated in IBD-related primary sclerosing cholangitis (PSC), little is understood about the potential pathophysiological associations between IBD and cutaneous EIMs. Whether cutaneous EIMs are inflammatory events with a commonly shared genetic background or environmental risk factors with IBD but independent of IBD or are the result of an extraintestinal extension of intestinal inflammation, remains unclear. The review aims to provide an overview of the two most representative cutaneous manifestations of IBD, describe IBD's epidemiology, clinical characteristics, and histology, and discuss the immunopathophysiology and existing treatment strategies with biologic agents, with a focus on the potential pathophysiological associations between IBD and cutaneous EIMs.

Methotrexate as a corticosteroid-sparing agent in leprosy reactions: A French multicenter retrospective study.

INTRODUCTION: Leprosy reactions (LRs) are inflammatory responses observed in 30%-50% of people with leprosy. First-line treatment is glucocorticoids (GCs), often administered at high doses with prolonged courses, resulting in high morbi-mortality. Methotrexate (MTX) is an immunomodulating agent used to treat inflammatory diseases and has an excellent safety profile and worldwide availability. In this study, we describe the efficacy, GCs-sparing effect and safety of MTX in LRs. METHODS: We conducted a retrospective multicentric study in France consisting of leprosy patients receiving MTX for a reversal reaction (RR) and/or erythema nodosum leprosum (ENL) since 2016. The primary endpoint was the rate of good response (GR) defined as the complete disappearance of inflammatory cutaneous or neurological symptoms without recurrence during MTX treatment. The secondary endpoint was the GCs-sparing effect, safety and clinical relapse after MTX discontinuation. RESULTS: Our study included 13 patients with LRs (8 men, 5 women): 6 had ENL and 7 had RR. All patients had had at least one previous course of GCs and 2 previous treatment lines before starting MTX. Overall, 8/13 (61.5%) patients had GR, allowing for GCs-sparing and even GCs withdrawal in 6/11 (54.5%). No severe adverse effects were observed. Relapse after MTX discontinuation was substantial (42%): the median relapse time was 5.5 months (range 3-14) after stopping treatment. CONCLUSION: MTX seems to be an effective alternative treatment in LRs, allowing for GCs-sparing with a good safety profile. Furthermore, early introduction during LRs may lead to a better therapeutic response. However, its efficacy seems to suggest prolonged therapy to prevent recurrence.

Yersinia enterocolitica Enteritis Diagnosed with Erythema Nodosum.

We herein report a rare case of Yersinia enterocolitica enteritis with a fever and abdominal pain followed by erythema nodosum (EN) a few days later. The diagnosis was confirmed based on characteristic colonoscopy and computed tomography findings, pathology, and mucosal culture. Yersinia enteritis is a curable disease provided a proper diagnosis and treatment are performed. Although EN is a rare clinical course, it should still be considered as a differential diagnosis.

Superficial (nodular) thrombophlebitis as a heterogeneous entity with distinctive clinico-pathological aspects: Correlation with the underlying conditions.

BACKGROUND: Superficial (nodular) thrombophlebitis, referring to a thrombo-inflammatory disorder of dermal-subcutaneous veins, encompass a spectrum of nodular lesions often associated with inflammatory, infectious, neoplastic or thrombophilic diseases. OBJECTIVE: We postulate that distinct clinico-pathological features may correlate with the underlying conditions. METHODS: We conducted a retrospective monocentric study of all patients seen in our dermatology department for a superficial thrombophlebitis, completed with a literature review. Clinical and pathological data were extracted from the patient files and articles. RESULTS: A total of 108 cases of superficial thrombophlebitis was analysed. Forty-five percent manifested as elongated nodules clearly evoking vascular involvement, while the other 55% were presenting as nodules or plaques. The elongated phenotype was strongly associated with malignancy or thrombophilia if a pure thrombosis was demonstrated histopathologically, while tuberculosis was the main underlying condition if a granulomatous vasculitis was present. Panniculitis-like lesions were mainly corresponding to erythema-nodosum-like lesions of Behçet's disease, characterized by the distinctive feature of thrombotic veins with an associated leukocytoclastic vasculitis. LIMITATIONS: Retrospective design and risk of publication bias. CONCLUSION: Superficial (nodular) thrombophlebitis is a heterogeneous entity with a distinct clinico-pathological presentation that strongly points to the nature of the underlying medical condition, thus guiding the medical workup.

Autoimmune rheumatic diseases associated with granulomatous mastitis.

Granulomatous mastitis (GM) is a benign, inflammatory condition of the breast that mainly affects women of reproductive age. Although its pathogenesis remains unknown, previous studies revealed an association between autoimmune rheumatic diseases (ARDs) and GM in a subset of patients implicating immune-mediated mechanisms. The aim of this narrative review was to identify and describe the ARDs associated with GM to shed further light on disease pathogenesis. We conducted a comprehensive literature search of patients presenting with GM and coexisting ARDs using electronic databases. An association between GM and various ARDs has been reported, including sarcoidosis, systematic lupus erythematosus, granulomatosis with polyangiitis, psoriasis/psoriatic arthritis, familial Mediterranean fever, ankylosing spondylitis, Sjogren's syndrome, rheumatoid arthritis, and erythema nodosum, with the most common being granulomatous mastitis-erythema nodosum-arthritis syndrome (GMENA), granulomatosis with polyangiitis (Wegener's) and sarcoidosis. In addition, clinical characteristics, diagnostic and therapeutic approaches were recorded. Further research is warranted to better understand the association between GM and ARDs and raise awareness amongst rheumatologists.

Erythema Nodosum following Nocardia Infection: A Case Report.

Cutaneous nocardiosis is a rare bacterial infection that can result in various dermatologic manifestations such as actinomycetoma, lymphocutaneous infection, superficial skin infection, and secondary infection due to hematogenous dissemination. We report on a Chinese patient with erythema nodosum-like exanthema, possibly secondary to nocardiosis. Our diagnosis for this patient was based on the clinical presentation, histopathological evidence, and microbiological findings. Given the protean manifestation of Nocardia, persistent reports on new presentations of the disease are important for early identification and treatment.

Erythema nodosum as key manifestation of histoplasmosis case.

Histoplasmosis is a systemic infection caused by the fungus, Histoplasma capsulatum Infection of H. capsulatum frequently occurs by inhaling the spores of the fungus, which is found in bat and bird droppings, or soil enriched with their excrement. If not detected and treated, histoplasmosis can develop late, severe complications such as mediastinal fibrosis, or even develop into a disseminated infection. However, histoplasmosis infections are often asymptomatic, making its detection more difficult. Further, only 6% of histoplasmosis cases will present with erythema nodosum, with most cases associated with female patients. This case presents a woman in her 50s with a pertinent history of fibromyalgia and hypothyroidism secondary to Hashimoto's who initially sought medical care for painful nodules on her legs and was eventually diagnosed with histoplasmosis. This report clearly demonstrates the importance of maintaining a broad differential when working up inflammatory manifestations, such as erythema nodosum.

Cutaneous sarcoidosis: Lupus pernio and more.

Sarcoidosis is a multiorgan disease commonly evident with skin involvement. Cutaneous manifestations occur in about 25% of sarcoid patients and are of two types: histologically specific sarcoidal infiltrations and a cutaneous reaction pattern not containing sarcoidal changes, usually erythema nodosum. Cutaneous plaques, nodules, and tumors, sometimes with disfiguring facial features are associated with pain and paresthesia. The disease itself may produce substantial morbidity due to visceral involvement. Advances in therapeutic options include tocilizumab, an IL-6 inhibitor, and tofacitinib - a Janus kinase inhibitor. This review discusses sarcoidosis etiology and pathogenesis, its clinical features, differential diagnosis, and management.

Sequential occurrence of recurrent Sweet syndrome and erythema nodosum without an underlying secondary cause: a case report.

BACKGROUND: Sweet syndrome is a rare cause of acute fever and painful erythematous skin plaques. Erythema nodosum is acute or chronic tender erythematous skin nodules of bilateral shins. The concurrent presence of both dermatoses is rare but reported in the literature. There are no reported cases of recurrent and sequential Sweet syndrome and erythema nodosum without an underlying secondary cause. CASE PRESENTATION: We report the case of a 64-year-old Asian woman, who had possible Sweet syndrome 12 years ago and biopsy-proven erythema nodosum 5 years ago, presenting with an acute episode of Sweet syndrome. Extensive investigations did not reveal any underlying secondary cause. CONCLUSIONS: Recurrent Sweet syndrome and sequential presence with erythema nodosum raises suspicion if Sweet syndrome and erythema nodosum are different presentations of one disease, which warrants further study. This case proves that recurrent Sweet syndrome and erythema nodosum can occur in healthy individuals without an underlying malignancy or secondary cause.

[Idiopathic granulomatous mastitis associated with erythema nodosum: a case report]. / Mastite granulomateuse idiopathique associant un érythème noueux: à propos d'un cas.

Idiopathic granulomatous mastitis (IMD) is a chronic benign inflammatory disease of the breast which can mimic breast cancer. It is more common in young women of childbearing age and poses a challenge in diagnosis of inflammatory breast tumor. We report the case of a 26-year-old female patient who presented with inflammatory breast. Clinical examination showed apyretic patient with edematized and painless irregular smooth mass in the left breast measuring 4 cm surmounted by crusts and multiples fistulizing scars discharging pus by applying pressure, with mobile ipsilateral axillary adenopathy. The remainder of the physical examination showed inflammatory erythematous patches on both legs. A mammogram plus breast ultrasound was performed followed by biopsy that objectified fibrous mastopathy. Lumpectomy was performed due to the persistence of symptoms. Anatomopathological examination objectified granulomatous mastitis. Etiological research didn't identify a clear cause. Corticotherapy associated with antibiotic therapy was started with complete regression of symptoms after two months.

Vedolizumab for the Management of Refractory Behçet's Disease: From a Case Report to New Pieces of Mosaic in a Complex Disease.

Objectives: When treating Behçet's disease (BD), anti-tumor necrosis factor (TNF)-α agents have become a second-line therapy when conventional immunosuppressive drugs have failed. However, in the case of failure of treatment with anti-TNFα drugs, further options are limited. Based on previous reports of the efficacy of vedolizumab (VDZ) in inflammatory bowel diseases, we decided to administer VDZ to treat a patient with intestinal BD. Methods: We present the case of a 49-year-old female patient with BD. Her clinical manifestations included erythema nodosum, oro-genital ulcers, positive Pathergy test, positive HLA-B51, and biopsy-proven intestinal BD. The patient was unsuccessfully treated with conventional immunosuppressive and several biological agents. Results: Treatment with VDZ was started intravenously at a dose of 300 mg at 0, 2, and 6 weeks and then every 4 weeks. After the second dose of VDZ, the patient reported a marked improvement of intestinal BD and a concomitant amelioration of arthralgia, erythema nodosum lesions and aphthosis. Clinical remission was achieved at 6 months after starting VDZ. Conclusion: VDZ might represent a valid option to treat patients with BD who are non-responsive to standard treatments or anti-TNFα agents, particularly, those cases with intestinal involvement.

Idiopathic granulomatous mastitis associated with erythema nodosum may indicate a worse prognosis.

INTRODUCTION: Idiopathic granulomatous mastitis (IGM) is a chronic inflammatory breast disease of unknown etiology, and erythema nodosum (EN) is a rare extramammary manifestation of this entity characterized by reddish, tender nodules of the lower legs. We aimed to investigate whether the association of IGM with EN has a role as a prognostic indicator. There are few case reports, and only 1 original article including 12 IGM patients with EN has been reported. METHODS: We present 43 women with IGM coexisting with EN and 43 with a diagnosis of IGM only, who were randomly selected from 610 patients for a control group. To the best of our knowledge, this paper comprises the first comparative study of the coexistence of IGM and EN to be reported in the literature. RESULTS: Our findings show that the association of IGM with EN indicates a more aggressive disease course. White blood cells, erythrocyte sedimentation rate, and C-reactive protein were significantly higher in the EN-positive group (P < .05). Arthralgia, breast feeding, fistula distribution and recurrence distributions were significantly higher in the EN-positive group (P < .05). CONCLUSION: Since fistula distribution and recurrence rates were higher in EN-positive group, association of IGM with EN may be an indictor of a worse prognosis. The present study highlights the importance of dermatological care. All physicians should not neglect questioning breast complaints in patients with EN since EN may be caused by IGM.

Granulomatous mastitis, erythema nodosum and arthritis syndrome: case-based review.

Granulomatous mastitis (GM) is a rare form of inflammatory breast condition associated with unilateral or bilateral breast pain, swelling and mass formation. Although the disease pathogenesis remains unknown, several reports have associated GM with manifestations such as erythema nodosum and occasionally with arthritis, suggesting that GM might have an autoimmune disease component. We aim to describe two cases of coexistence of GM, erythema nodosum, and arthritis. We also conducted a literature review to comprehensively assess and describe the characteristics of patients with GM, erythema nodosum, and arthritis, and identify effective treatment options. A literature review was conducted using PubMed and EMBASE, and 14 case reports/series were retrieved, with a total number of 29 patients. All patients are women and unilateral breast involvement was evident in the majority of patients. Nine patients (31%) presented with arthritis, 6 patients (20.7%) had a fever, and 6 patients (20.7%) developed the symptoms during pregnancy. All patients had normal chest radiograph and tissue cultures were negative. In most of the cases (n = 25, 86.2%), symptom improvement was observed with glucocorticoids and four patients (13.8%) underwent surgical treatment for the GM. Given the clinical characteristics of patients with GM, with erythema nodosum, with or without arthritis, and the positive response to glucocorticoids, we propose that the described phenotype represents an underrecognized systemic autoimmune disease that could be designated by the acronym "GMENA" (granulomatous mastitis, erythema nodosum, arthritis) syndrome. Further studies are needed to elucidate the pathogenesis of the syndrome.