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1.
Front Immunol ; 15: 1295759, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38529282

RESUMO

Idiopathic granulomatous mastitis (IGM) is a noncancerous, chronic inflammatory disorder of breast with unknown causes, posing significant challenges to the quality of life due to its high refractoriness and local aggressiveness. The typical symptoms of this disease involve skin redness, a firm and tender breast mass and mastalgia; others may include swelling, fistula, abscess (often without fever), nipple retraction, and peau d'orange appearance. IGM often mimics breast abscesses or malignancies, particularly inflammatory breast cancer, and is characterized by absent standardized treatment options, inconsistent patient response and unknown mechanism. Definite diagnosis of this disease relies on core needle biopsy and histopathological examination. The prevailing etiological theory suggests that IGM is an autoimmune disease, as some patients respond well to steroid treatment. Additionally, the presence of concurrent erythema nodosum or other autoimmune conditions supports the autoimmune nature of the disease. Based on current knowledge, this review aims to elucidate the autoimmune-favored features of IGM and explore its potential etiologies. Furthermore, we discuss the immune-mediated pathogenesis of IGM using existing research and propose immunotherapeutic strategies for managing this condition.


Assuntos
Eritema Nodoso , Mastite Granulomatosa , Feminino , Humanos , Mastite Granulomatosa/diagnóstico , Mastite Granulomatosa/etiologia , Mastite Granulomatosa/terapia , Qualidade de Vida , Febre , Imunoglobulina M/uso terapêutico
2.
Skin Res Technol ; 30(2): e13600, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38297958

RESUMO

BACKGROUND: Previous studies have demonstrated the coexistence of erythema nodosum (EN) and inflammatory bowel disease (IBD), while the exact etiology of the co-occurrence of the two disorders remains uncertain. METHODS: A bidirectional two-sample Mendelian randomization (MR) design was employed to determine the causal link between EN and IBD. Genetic variations associated with Crohn's disease (CD) and ulcerative colitis (UC) were derived from accessible genome-wide association studies pertaining to European ancestry. The FinnGen database was used to find the genetic variations containing EN. In the forward model, IBD was identified as the exposure, whereas in the reverse model, EN was identified as the exposure. The causal link between IBD and EN was examined using a range of different analysis techniques, the primary one being the inverse variance weighted (IVW) method, including inverse variance weighted-fixed effects (IVW-FE) and inverse-variance weighted-multiplicative random effects (IVW-MRE). To strengthen the results, assessments of sensitivity, heterogeneity, and pleiotropy were also conducted. RESULTS: MR results showed that IBD increased the risk of EN (IVW-MRE: OR = 1.242, 95% CI = 1.068-1.443, p = 0.005). Furthermore, there was a strong correlation found between CD and a higher risk of EN (IVW-FE: OR = 1.250, 95% CI = 1.119-1.396, p = 8.036 × 10-5 ). However, UC did not appear to be linked to EN (IVW-FE: OR = 1.104, 95% CI = 0.868-1.405, p = 0.421). The reverse MR analysis findings did not imply that EN was linked to IBD. Horizontal pleiotropy did not appear to exist, and the robustness of these findings was confirmed. CONCLUSION: The current investigation found that in European populations, IBD and its subtype CD could raise the incidence of EN.


Assuntos
Eritema Nodoso , Doenças Inflamatórias Intestinais , Humanos , Eritema Nodoso/epidemiologia , Eritema Nodoso/genética , Estudo de Associação Genômica Ampla , Análise da Randomização Mendeliana , Doenças Inflamatórias Intestinais/epidemiologia , Doenças Inflamatórias Intestinais/genética , Bases de Dados Factuais
3.
JAMA Dermatol ; 160(2): 229-232, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38170490

RESUMO

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


Assuntos
Eritema Nodoso , Humanos , Eritema Nodoso/diagnóstico , Eritema Nodoso/complicações , Recidiva
4.
Dermatology ; 240(2): 226-232, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38185114

RESUMO

INTRODUCTION: Erythema nodosum (EN) is the most common form of panniculitis that predominantly affects the shins. While EN in atypical sites has been described by many authors, there are currently only case studies published on this topic. This study aimed to evaluate clinical differences between patients suffering from EN on the shins, compared to patients with EN in atypical locations. METHODS: We analyzed 105 patients in a retrospective, single-center study at a university hospital in Switzerland. Typical EN was defined as lesions, found only on the lower legs, while atypical EN as lesions on the upper legs, trunk, arms, or face, only or in addition to lesions on the lower legs. The patients were assessed for age, gender, dermatologic history, time until first medical consultation, time to diagnosis, and time until remission. Further, etiology, symptoms, and applied therapies were investigated. Findings were then compared between the typical and atypical EN cohorts. RESULTS: Overall, we included 70 patients (37.99 ± 15.67 [3-81] years) with EN solely on the shins and 35 patients (41.27 ± 16.85 [9-76] years) with EN on other locations. Interestingly, time until diagnosis was significantly shorter in atypical EN (p = 0.034, 1.14 ± 4.68 vs. 0.46 ± 1.14 months). Time to remission was similar in both groups (3.61 ± 2.73 vs. 3.05 ± 2.86 months, respectively). Sarcoidosis was the only etiologic factor significantly more frequent in atypical EN compared to typical EN (23% vs. 9%, p = 0.042). Besides that, solely subtle differences were seen regarding etiology, gender, age at onset, course of the disease, and symptoms. CONCLUSIONS: Our study suggests that only minor alterations between both study populations exist. Significant differences were found in time to diagnosis (shorter for atypical EN), as well as in sarcoidosis as an etiologic factor (more frequent in atypical EN). While adalimumab was only prescribed in atypical EN cases, prognosis seems to be similar for typical and atypical EN (similar time to remission, similar amount of reoccurring cases). Due to the limited sample size, however, our study population may have been too small to detect the relevant differences, and bigger studies may be needed.


Assuntos
Eritema Nodoso , Paniculite , Sarcoidose , Humanos , Eritema Nodoso/diagnóstico , Estudos Retrospectivos , Paniculite/complicações , Paniculite/diagnóstico , Paniculite/patologia , Resultado do Tratamento
5.
Acta Med Port ; 37(3): 215-219, 2024 Mar 01.
Artigo em Português | MEDLINE | ID: mdl-38271690

RESUMO

Hansen's disease, commonly known as leprosy, is an infectious disease caused by Mycobacterium leprae. Being rare in developed countries, it is an increasingly common imported disease due to the migratory flow from countries where it is endemic. We present the case of a 21-year-old man who went to the emergency department with complaints of additive polyarthralgia involving large joints, papules, and erythematous plaques on the limbs with bullae and central necrosis and fever with chills for one week. Skin biopsy was performed revealing neutrophilic infiltrate with perineural granulomas. The bacilloscopy detected acid-alcohol resistant bacilli. The diagnosis of multibacillary HD with type 2 lepromatous reaction (erythema nodosum leprosum - ENL) was established, showing clinical improvement under corticosteroid therapy. ENL usually presents with painful lesions, being an atypical presentation of leprosy, especially in the presence of bullae and necrosis, making diagnosis difficult and challenging. Social stigma is often present making it difficult to accept the disease as well as adherence to treatment.


A doença de Hansen, vulgarmente conhecida como lepra, é uma doença infecciosa causada por Mycobacterium leprae. Sendo rara nos países desenvolvidos, configura uma doença de importação cada vez mais frequente considerando o fluxo migratório de países onde é endémica. Apresentamos o caso de um homem de 21 anos que recorreu ao serviço de urgência por poliartralgias de caráter aditivo envolvendo grandes articulações, pápulas e placas eritematosas nos membros com bolhas e necrose central e febre com calafrio com uma semana de evolução. Foi realizada biópsia cutânea que revelou infiltrado neutrofílico com granulomas de distribuição perineural e baciloscopia com deteção de bacilos ácido-álcool resistentes. Foi estabelecido o diagnóstico de DH multibacilar com reação lepromatosa tipo 2 (eritema nodoso leproso), apresentando melhoria clínica sob corticoterapia. O eritema nodoso leproso cursa habitualmente com lesões dolorosas, configurando uma apresentação atípica de lepra, sobretudo na presença de bolhas e necrose, tornando este diagnóstico altamente desafiante. O estigma social é frequentemente limitativo na aceitação da doença e adesão ao tratamento.


Assuntos
Eritema Nodoso , Hanseníase , Masculino , Humanos , Adulto Jovem , Adulto , Vesícula , Hanseníase/tratamento farmacológico , Hanseníase/epidemiologia , Hanseníase/patologia , Pele/patologia , Eritema Nodoso/diagnóstico , Eritema Nodoso/tratamento farmacológico , Eritema Nodoso/patologia , Necrose/patologia
7.
Trans R Soc Trop Med Hyg ; 118(1): 77-79, 2024 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-37632458

RESUMO

BACKGROUND: Erythema nodosum leprosum (ENL) is an immunologically mediated phenomenon complicating the course of leprosy. Reverse Koebner phenomenon is the term used to describe the sparing of previously injured or diseased skin by new skin lesions of the disease. METHODS: A middle-aged woman with a known case of lepromatous leprosy for the past year presented with an eruption of reddish painful nodules over her body. The lesions were found to characteristically spare the sites of previous scars. RESULTS: This sparing phenomenon of previous scar sites has been termed reverse Koebner phenomenon, a site of the body that offers greater resistance than the rest of the body to the onset of the disease, seen in various diseases, but it has never been described in ENL. CONCLUSION: This sparing of scar sites in ENL can be attributed to reverse Koebner phenomenon.


Assuntos
Eritema Nodoso , Hipersensibilidade , Hanseníase Virchowiana , Hanseníase , Humanos , Pessoa de Meia-Idade , Feminino , Cicatriz/complicações , Cicatriz/patologia , Hanseníase Virchowiana/complicações , Hanseníase Virchowiana/tratamento farmacológico , Hanseníase Virchowiana/patologia , Pele/patologia , Hanseníase/complicações , Hipersensibilidade/complicações , Hipersensibilidade/patologia
8.
Int J Rheum Dis ; 27(1): e14817, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37401805

RESUMO

INTRODUCTION: Azathioprine hypersensitivity can occasionally present as Sweet-like syndrome, a dose-independent side effect characterized by the unanticipated onset of macules, papules, and pustules. CASE PRESENTATION: A 35-year-old woman with systemic lupus erythematosus presented with complaints of generalized maculopapular rash, facial swelling, and bilateral lower extremity edema with a duration of 4 days and a 2-day history of constitutional symptoms within 2 weeks of the beginning of azathioprine therapy to treat existing lupus nephritis (class 2/3). DISCUSSION: Patients who experience azathioprine hypersensitivity syndrome can present with erythema nodosum, small-vessel vasculitis, acute generalized exanthematous pustulosis, Sweet syndrome, and nonspecific dermatosis. The following signs and symptoms are used as criteria to diagnose drug-induced Sweet syndrome: (a) abrupt onset of painful erythematous plaques, (b) histopathological evidence of dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis, (c) temperature higher than 39.7°C, (d) temporal relationship between drug ingestion and clinical presentation, and (e) temporal resolution of lesions after drug withdrawal. Our patient met three out of five criteria and was diagnosed with Sweet-like syndrome. CONCLUSION: Our case highlights the uncommonly presented azathioprine-induced Sweet-like syndrome that occurs abruptly after the commencement of the offending drug. This diagnosis can be established through basic laboratory workup and skin biopsy findings.


Assuntos
Eritema Nodoso , Lúpus Eritematoso Sistêmico , Síndrome de Sweet , Feminino , Humanos , Adulto , Azatioprina/efeitos adversos , Síndrome de Sweet/induzido quimicamente , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/tratamento farmacológico , Pele/patologia , Lúpus Eritematoso Sistêmico/diagnóstico
14.
Am J Trop Med Hyg ; 110(1): 133-135, 2024 Jan 03.
Artigo em Inglês | MEDLINE | ID: mdl-38081053

RESUMO

Erythema nodosum (EN) is characterized by rapidly developing, painful, erythematous subcutaneous nodules, most of which are located in the pretibial areas. This cutaneous finding can be caused by a variety of conditions, however Burkholderia pseudomallei is rarely the cause. This particular patient presented with a high-grade fever with characteristic EN on both pretibial areas. All of the typical EN causes were investigated, but the findings were all negative. The lesions progressed to severe hemorrhagic bleb features, and because the patient resided in Northeast Thailand, a melioidosis-endemic region, testing for B. pseudomallei was performed. Because a high level of melioidosis serology of more than 1:10,240 was detected, melioidosis therapy was started. At the 12-week follow-up after melioidosis therapy, the titer had declined to 1:1,280, indicating that melioidosis-related severe, cutaneous EN symptoms were the most likely diagnosis in this patient. We discovered a case of EN with severe hemorrhagic bleb features as a unique clinical manifestation of melioidosis. When a patient resides in an endemic area, B. pseudomallei should always be considered as a possible causative organism.


Assuntos
Burkholderia pseudomallei , Eritema Nodoso , Melioidose , Criança , Humanos , Melioidose/complicações , Melioidose/diagnóstico , Melioidose/tratamento farmacológico , Tailândia/epidemiologia , Eritema Nodoso/diagnóstico , Dor
16.
Skinmed ; 21(6): 448-450, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38051249

RESUMO

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).


Assuntos
Eritema Nodoso , Mastite Granulomatosa , Sarcoidose , Feminino , Gravidez , Humanos , Adulto , Gestantes , Mastite Granulomatosa/complicações , Mastite Granulomatosa/diagnóstico , Eritema Nodoso/complicações , Eritema Nodoso/diagnóstico , Eritema Nodoso/patologia , Abscesso
17.
Front Immunol ; 14: 1234535, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37954590

RESUMO

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.


Assuntos
Colite Ulcerativa , Doença de Crohn , Eritema Nodoso , Doenças Inflamatórias Intestinais , Pioderma Gangrenoso , Humanos , Doenças Inflamatórias Intestinais/tratamento farmacológico , Doença de Crohn/tratamento farmacológico , Colite Ulcerativa/tratamento farmacológico , Pioderma Gangrenoso/terapia , Pioderma Gangrenoso/complicações , Eritema Nodoso/terapia , Eritema Nodoso/complicações
18.
J Invest Surg ; 36(1): 2257770, 2023 Sep 08.
Artigo em Inglês | MEDLINE | ID: mdl-37875261

RESUMO

PURPOSE: Granulomatous lobular mastitis (GLM) is a benign inflammatory disease of the mammary gland with unknown etiology. Erythema nodosum (EN) is a rare, extramammary symptom of GLM. The purpose of this article was to investigate the clinical features of EN associated with GLM. METHODS: We recruited 102 GLM patients diagnosed between December 2018 and December 2021 at Renmin Hospital of Wuhan University. The clinical characteristics and laboratory indices of the EN group (n = 12) and the non-EN group (n = 90) were compared. RESULTS: The proportion of GLM patients with lesions involving ≥2 quadrants and high peripheral white blood cell count, absolute neutrophil count and neutrophil-to-lymphocyte ratio (NLR) was larger in the EN group than in the non-EN group (p = 0.002, 0.025, 0.014, 0.002, respectively). The duration of EN was longer in GLM patients with EN than in GLM patients without EN (p = 0.005). GLM patients with EN had more abscesses and sinus tracts than those without EN (p = 0.003, 0.038). Lesions involving ≥2 quadrants and the NLR were positively associated with the occurrence of EN (R = 0.304, 0.0302, p = 0.002, 0.002). Receiver operating characteristic curve analysis revealed that the area under the curve of the NLR was 0.770. When NLR > 5.73, the sensitivity and specificity of predicting EN were 66.67% and 87.78%, respectively. CONCLUSION: Our findings suggest that GLM concomitant with EN suggests the presence of a more severe condition and extensive lesions.


Assuntos
Eritema Nodoso , Mastite Granulomatosa , Feminino , Humanos , Eritema Nodoso/diagnóstico , Eritema Nodoso/epidemiologia , Eritema Nodoso/etiologia , Mastite Granulomatosa/complicações , Mastite Granulomatosa/diagnóstico , Linfócitos , Inflamação , Neutrófilos
20.
JBJS Case Connect ; 13(3)2023 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-37683077

RESUMO

CASE: A 22-year-old female patient was referred to the orthopaedic department for further examination after a radiopaque area was observed in the T6 vertebra in her chest radiograph. Computed Tomography (CT) showed a sclerotic mass with smooth borders, involving the entire body of the T6 vertebra, left posterior elements, posterior of the rib past the left zygapophyseal joint, and a "flowing candle wax" image toward the T7 vertebra. Spinal melorheostosis was considered radiologically in the patient, but malignancy could not be completely excluded. Thereupon, open biopsy was performed under general anesthesia. CONCLUSION: Spinal melorheostosis is a rare condition. Histological examination should be considered in cases where the diagnosis remains suspicious after clinical and radiological evaluations.


Assuntos
Eritema Nodoso , Melorreostose , Feminino , Humanos , Adulto Jovem , Adulto , Melorreostose/diagnóstico por imagem , Vértebras Torácicas , Tomografia Computadorizada por Raios X
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