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.

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.

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.

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.

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.

Patients with idiopathic granulomatous mastitis accompanied by erythema nodosum.

BACKGROUND: Idiopathic granulomatous mastitis (IGM) is a rare, chronic inflammatory disease of the breast. The aetiology is unknown. Erythema nodosum (EN) is a rare skin disease associated with IGM. EN is a disease in which the aetiologic factors include infection, malignancy, autoimmune diseases, and pregnancy. OBJECTIVE: The objective of this study was to evaluate the incidence and coexistence of EN with IGM and the approach to treatment. METHODS: Patients who were admitted to the General Surgery and Dermatology Department of our hospital and diagnosed with EN and IGM were evaluated. Demographic data, symptoms, examination findings, number of birth, smoking, diagnosis and treatment methods, recurrence, and follow-up periods of the patients were evaluated. RESULTS: EN was seen in six of the patients admitted to the clinic for IGM. Four patients with EN had pregnancy and all had arthritis. Two patients were not pregnant. Oral methylprednisolone treatment was started in patients whose IGM diagnosis was verified pathologically. CONCLUSION: IGM may be associated with extramammary symptoms such as EN, arthralgia, and episcleritis. These extramammary findings suggest that IGM may be an autoimmune disease. For this reason, breast examination and history of IGM of the breast should be questioned in female patients admitted to different clinics with EN. This way, delays in the diagnosis and treatment of IGM can be prevented.

Löfgren Syndrome with Hypercalcemia and Neuroendocrinological Involvement: A Case Report.

BACKGROUND: Sarcoidosis is a systemic inflammatory disease of unknown etiology that can affect virtually any organ. Löfgren syndrome, characterized by erythema nodosum, hilar lymphadenopathy, fever and polyarthritis, represents only 20-30% of the cases of sarcoidosis. Only 2- 10% of the cases feature hypercalcemia. CASE: The case of a 42-year-old Hispanic woman with a history of erythema nodosum and three weeks of nausea, emesis, constipation, asthenia, adynamia, polydipsia, and somnolence, concomitant with hypercalcemia, but normal parathyroid hormone (PTH) and 25-hydroxyvitamin D has been presented. The initial diagnostic approach was based upon the suspicion of multiple myeloma or bone metastases; however, further findings of bilateral hilar lymphadenopathy, elevated serum angiotensin-converting enzyme (ACE) and a right inguinal lymphadenomegaly suggested an alternate diagnosis. Biopsy of the latter supported sarcoidosis as the diagnosis. She was successfully treated in the hospital with zoledronic acid and as an outpatient with immunosuppressive therapy. Persistence of a previously undisclosed symptom of oligomenorrhea led to the detection of hyperprolactinemia secondary to hypophyseal infiltration, refractory to immunosuppressive therapy but with an adequate response to cabergoline. CONCLUSION: This case strays from Löfgren Syndrome's expected behavior, presenting a more progressive, multisystemic disease. This case report was written in adherence to the CARE guidelines of 2013 to include information in it.

Generalized chronic erythema nodosum: to consider Weber-Christian disease. / Eritema nudoso crónico de localización generalizada: a considerar enfermedad de Weber-Christian.

BACKGROUND: Weber-Christian disease is a recurrent, non-suppurative, febrile nodular panniculitis, more frequent in young women. It is characterized by recurrent outbreaks of subcutaneous nodules, distributed symmetrically. It has an idiopathic origin, or it is secondary to pancreatic disease, physicochemical agents or alpha-1 antitrypsin deficiency. It can affect any tissue with body fat. Histologically is a lobular panniculitis without vasculitis. CASE REPORT: 23-year-old woman. She was admitted with subcutaneous painful nodules in pelvic, gluteus and forearms, with erythematous and hyperpigmented plaques; ecchymosis in the right upper eyelid and bilateral hyposphagma; fever of 38 °C; diaphoresis and oral ulcers, not painful. The hemogram showed pancytopenia, elevation of acute phase reagents, amylase and normal lipase. Blood culture was negative. Antinuclear antibodies and complement were normal. Computed tomography of the abdomen showed disseminated hyperdensity in subcutaneous tissue. The biopsy showed lobular panniculitis without vasculitis compatible with WeberChristian panniculitis. The patient was treated with steroids and immunosuppressive therapy. CONCLUSIONS: Infectious etiology and other causes of erythematous nodules were ruled out through clinical evolution and complementary studies. The patient presented spontaneous involution in several weeks and sometimes the lesions evolved to sterile abscesses, in addition to systemic clinical manifestations. Corticosteroid therapy was deemed successful.

INTRODUCCIÓN: La enfermedad de Weber-Christian es una paniculitis nodular recidivante, no supurativa, febril, más frecuente en las mujeres jóvenes. Se caracteriza por brotes recurrentes de nódulos subcutáneos, de distribución simétrica. Tiene un origen idiopático o es secundaria a enfermedad pancreática, agentes fisicoquímicos o déficit de alfa-1-antitripsina. Puede afectar cualquier tejido del organismo que tenga grasa. Histológicamente presenta paniculitis lobulillar sin vasculitis. CASO CLÍNICO: Mujer de 23 años que ingresó por nódulos subcutáneos dolorosos con equimosis en miembros pélvicos, glúteos y antebrazos, con placas eritematosas e hiperpigmentadas; equimosis en párpado superior derecho e hiposfagma bilateral; fiebre de 38 °C; diaforesis y úlceras orales no dolorosas. El hemograma mostró pancitopenia, elevación de los reactantes de fase aguda, y amilasa y lipasa normales. Los cultivos fueron negativos. Los anticuerpos antinucleares y el complemento fueron normales. La tomografía del abdomen presentó hiperdensidad en tejido graso subcutáneo diseminada. La biopsia reveló paniculitis lobulillar sin vasculitis, compatible con paniculitis de Weber-Christian. Se trató con esteroide e inmunosupresor. CONCLUSIONES: Se descartaron la etiología infecciosa y otras causas de nódulo eritematoso por la evolución clínica y los estudios complementarios. La paciente presentó en varias semanas una involución espontánea; en ocasiones, las lesiones evolucionaron a abscesos estériles, además de manifestaciones clínicas sistémicas. El tratamiento corticoideo tuvo buen resultado.

[Idiopathic granulomatous mastitis associated with erythema nodosum]. / Mastite granulomateuse idiopathique associée à un érythème noueux.

BACKGROUND: Idiopathic granulomatous mastitis (IGM) is a rare, chronic, granulomatous, inflammatory disorder with potentially misleading clinical and radiological features. IGM is diagnosed after exclusion of infectious or tumoral diseases. Herein we report a case of erythema nodosum associated with IGM, which serves as a reminder that erythema nodosum may constitute an extra-mammary sign of IGM. PATIENTS AND METHODS: A 36-year-old nulliparous woman presented with fever and skin rash at our dermatology clinic at the North Franche-Comté hospital in July 2017. Symptoms had occurred 2 days previously. The patient had a fever of 38.8°C and typical erythema nodosum on the limbs. Physical examination showed an irregular, inflammatory, abscessed mass measuring 20cm with multiple shrinkage zones that had been developing for 4 weeks and for which breast examinations were being performed. Laboratory tests showed an inflammatory state (CRP 155mg/mL, WBC 14.6×109/L), other tests (serum electrolytes and calcium, hepatic and renal tests, Streptotest, T-spot, HIV, HBV, HCV serology, anti-streptolysin, anti-streptodornase, local microbiology samples, antinuclear antibodies, soluble antigens antibodies, hemocultures, angiotensin-converting-enzyme and chest x-rays) were normal. Microbiology investigations were negative. Mammography revealed invasive mastitis. Breast sample biopsies showed giant cell granulomas without caseous necrosis or tumor cells, and histochemical staining (PAS, Ziehl, Grocott, Gram) was negative. The final diagnosis was of IGM associated with erythema nodosum. Symptoms rapidly improved with oral steroids. DISCUSSION: As reported herein, erythema nodosum may be associated with IGM and support the diagnosis thereof. This association is rare, with fewer than 30 case reports described in the literature. IGM is an uncommon benign disorder of the breast that can mimic two frequent breast disorders: breast carcinoma and breast abscess. It usually occurs in young sexually active women. It appears as a tumor with an inflammatory solid painful mass, with nipple and skin retraction, occasionally with abscesses and accompanied by homolateral axillary lymphadenopathies. Radiologic findings are usually not specific for or suggestive of cancer. The histopathological picture of IGM is characterized by the presence of multinucleated giant cells and epithelioid histiocytes forming non-caseating granulomas around lobules. Minor ductal and periductal inflammation is usually present. IGM is rarely associated with autoimmune system manifestations, especially erythema nodosum, arthritis, episcleritis or hidradenitis suppurativa. Although the physiopathology of IGM remains unclear, this case serves as additional evidence that the etiology of IGM is of autoimmune origin. While there is no recommendation for the treatment of IGM, oral steroids remain the cornerstone of therapy. CONCLUSION: We report a case of IGM associated with erythema nodosum. Dermatologists must be aware of this association.

Primary cutaneous anaplastic large cell lymphoma arising from a long-standing erythema nodosum: A case report.

RATIONALE: Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a rare subtype of non-Hodgkin lymphoma, which is rarely associated with erythema nodosum (EN). PATIENT CONCERNS: A 57-year-old woman complained of recurrent rashes involving her abdomen, back, upper and lower limbs for over 20 years, with severity in symptoms for 2 weeks. DIAGNOSES: The first skin biopsy was performed in 2011 in another hospital and she was diagnosed idiopathic EN. The second skin biopsy was performed in 2014 and she was diagnosed as pcALCL with stage IA. INTERVENTIONS: She was treated with oral prednisone, cyclosporine, and thalidomide. OUTCOMES: One month later, the ulcerative lesion was decreased in size and became smooth and the patient achieved partial remission. She is still under treatment and has been monitored closely for 4 years. LESSONS: The case suggested that stimulation of inflammation in the skin lesions for a long period might be related to clonal transformation into pcALCL and hence should be closely monitored. Immunosuppressive treatment may be effective and safe for patients with pcALCL at early stages.