Subcutaneous Sweet's Syndrome Presenting With a Single Cutaneous Lesion on the Thigh.

Sweet's syndrome (SS), also known as acute febrile neutrophilic dermatosis, manifests as tender, erythematous skin lesions such as papules, nodules, and plaques that may appear vesicular or pustular. The condition is characterized by widespread infiltrates mainly consisting of mature neutrophils, usually in the upper dermis. Erythema nodosum (EN) is a form of septal panniculitis marked by tender, erythematous lesions primarily appearing on the lower legs. Additionally, subcutaneous Sweet's syndrome (SSS) is a rare variant of SS that mainly involves the subcutaneous adipose tissue. Skin lesions in SSS generally present as tender, erythematous subepidermal nodules on the extremities, morphologically resembling EN. Both EN and SS can present with fever, malaise, gastrointestinal disturbances, lymphadenopathy, arthralgia, increased white blood cell (WBC) count with neutrophilia, elevated C-reactive protein (CRP), and elevated erythrocyte sedimentation rate (ESR), making differentiation between them often challenging. Therefore, histopathologic evaluation is necessary for an accurate diagnosis. In our case, the patient exhibited a very painful plaque measuring 20 cm in diameter on the upper thigh without significant neutrophil infiltration in the dermis, but with subcutaneous septal neutrophil infiltration. Generally, SS shows stronger leukocytosis with neutrophilia than EN does. Considering the clinical symptoms, laboratory results, and clinical progression, the clinicopathological findings aligned more closely with SSS than EN. This article describes a rare case of SSS presenting with a single cutaneous lesion on the thigh, which mimicked the histopathological features of EN.

Poncet disease in a patient with smear-negative pulmonary tuberculosis: A case report.

Pulmonary tuberculosis poses a diagnostic dilemma to clinicians especially in the absence of typical presentation. The hypersensitivity to tuberculosis infection in other parts of the body can lead to nondestructive, para-infectious arthritis. This is known as Poncet disease, one of the clinical syndromes of musculoskeletal tuberculosis. Herein, we report a case of smear-negative pulmonary tuberculosis presenting with atypical features. It started with multiple joint pain, followed by the presence of multiple tender nodular skin lesions over the bilateral shins and wrist. Subsequent investigations led to the diagnosis of smear-negative pulmonary tuberculosis. Joint pain and erythema nodosum disappeared soon after antituberculosis therapy, supporting the diagnosis of Poncet disease.

Fatal misdiagnosis of progressive disseminated histoplasmosis.

Disseminated histoplasmosis is the form of a mycosis caused by the fungus Histoplasma capsulatum that mainly occurs in immunosuppressed hosts, usually with non-specific symptoms. In non-endemic areas, where the disease is rarely involved in the differential diagnosis, a delay in treatment may lead to severe medical complications. Due to the rising prevalence of disseminated histoplasmosis in these areas, a thorough medical history is regarded as the decisive factor in prompt diagnosis of the disease. We, herein, report the case of an immunocompetent Greek farmer with disseminated histoplasmosis whose condition was initially misdiagnosed, and the consequential inadequate treatment led to his death.

Comparative host transcriptomics as a tool to identify candidate biomarkers for immune reactions in leprosy using meta-analysis.

Background Leprosy is no longer considered an imprecation, as an effective multidrug therapy regimen is available worldwide for its cure. However, its diverse clinical manifestations sometimes involve acute inflammatory reactions. These complications result in irreversible nerve damage, neuritis and anatomical deformities that emerge before, during the treatment or after the completion of treatment. Reversal reaction (Type-I) and erythema nodosum leprosum (Type-II) are the leprosy reactions generally seen in patients with lepromatous and borderline forms of leprosy. At present, there is no accurate diagnostic test available to detect these leprosy reactions. Objectives To identify potential biomarkers indicative of Type-I and Type-II leprosy reactions that could help in their early diagnosis. Methods and Results Host-transcriptomics investigations have been utilised in this study to decipher a correlation between host-gene expression-based biomarkers and exacerbation of leprosy reactions. We present a comparative analysis of publicly available host transcriptomics datasets (from Gene Expression Omnibus) related to leprosy reactions. Individual datasets were analysed and integration of results was carried out using meta-analysis. Common differentially expressed genes (DEGs) were identified using the frequentist and Bayesian ratio association test methods. We have identified several genes - ADAMTS5, ADAMTS9, IFITM2, IFITM3, KIRREL, ANK3, CD1E, CTSF, DOCK9 and KRT73 to name a few - which can serve as potential biomarkers for Type-II reaction. Similarly, ACP5, APOC1, CCL17, S100B, SLC11A1 among others may likely serve as biomarkers for Type-I reaction. Limitations The number of datasets related to leprosy reactions found after the systematic search is less (n = 4) and may limit the accuracy of identified biomarker genes. This could be resolved by including more studies in the data analysis. Conclusion We provide a comprehensive list of gene candidates which could be prioritised further in research focusing on immune reactions in leprosy, as they are likely important in understanding its complexities and could be useful in its early diagnosis.

Granulomatous mastitis during pregnancy with sudden onset of gait difficulty and erythema nodosum: A case report and review of the literature.

Granulomatous mastitis (GM), a benign inflammatory disease of the breast, often mimics breast cancer on presentation. We present a case of GM during pregnancy manifesting as a breast mass, sudden onset of plantar pain, and erythema nodosum (EN). A 31-year-old pregnant Japanese woman, gravida 2, para 1, was referred to our hospital with severe plantar pain on both soles, causing difficulty walking. This pain worsened and EN appeared on both lower legs, followed by a left breast mass. Ultrasound findings suggested malignancy; however, aspiration biopsy confirmed GM. Her arthritis and EN resolved 2 days after commencing oral prednisolone and her walking improved. EN with/without arthritis is commonly associated with GM, especially during pregnancy. The described manifestations with a breast mass are suggestive of this diagnosis.

Idiopathic Granulomatous Mastitis, Erythema Nodosum, and Polyarthritis.

Idiopathic granulomatous mastitis (IGM) is an inflammatory-mediated rare disease that can be linked to rare manifestations. Erythema nodosum (EN) and polyarthritis, seen in a multitude of autoinflammatory and autoimmune diseases, have been rarely linked to IGM. Despite the cause of IGM being unclear, Corynebacterium infections are thought to play a role in the pathophysiology of IGM. Unusually, IGM has a relapsing and remitting course, which also applies to its systemic manifestations. As such, we present a case of IGM in a middle-aged lady who was initially thought to have Corynebacterium-containing unilateral abscesses for which drainage was performed. However, several abscesses devoid of bacterial growth started recurring, and the disease course was complicated by EN and polyarthritis. IGM, EN, and polyarthritis eventually resolved and were managed with symptomatic treatment.

Neutrophilic leukocytosis and erythema nodosum leprosum in leprosy: insights from a retrospective observational study.

Background: Leprosy reactions represent immunologically mediated episodes of acute inflammation that, if not diagnosed and treated promptly, can cause irreversible impairment of nerve function and permanent disabilities. A frequent type of reaction experienced by patients with lepromatous leprosy (LL) and borderline lepromatous leprosy (BL) is erythema nodosum leprosum (ENL), an inflammatory complication that may become chronic or recur in multiple episodes. Although ENL is commonly described as a neutrophil-mediated immune disease, the role of neutrophils is not fully understood. In this study, we assess neutrophilic leukocytosis in a retrospective cohort of patients affected by BL or LL leprosy. Materials and methods: A retrospective observational study was performed using data from 146 patients with BL and LL leprosy diagnosed and treated at the Souza Araújo Outpatient Clinic, Fiocruz, Rio de Janeiro, Brazil. Clinical, demographic, and hematological data were extracted from medical records. Skin biopsy samples obtained from patients for ENL diagnosis were used for histopathological evaluations. Results: Most patients were male (75%) and had a reactional episode (85%), of which 65% were ENL. Multiple episodes were common, 55% of the 80 patients with ENL presented more than 2 episodes (average of 2.6 episodes). In treatment-naive BL/LL patients, the median blood neutrophil counts of patients who developed ENL at some points of their disease course were higher than those who did not experience any reaction (median= 4,567 cells/mm3 vs 3,731 cells/mm3 respectively, p=0.0286). A correlation between the increase in median neutrophil counts and ENL severity was confirmed (6,066 cells/mm3 for mild ENL vs 10,243 cells/mm3 for moderate/severe ENL, p=0.0009). A longitudinal assessment was also performed in 34 patients, confirming the neutrophilic leukocytosis (BL/LL: 4896 cells/mm3 vs ENL: 8408 cells/mm3, p<0.0001). Moreover, increased NLR was associated with a greater neutrophilic infiltration in ENL lesions. Conclusion: We demonstrate that ENL episodes in patients affected by leprosy are associated with elevated blood leukocyte and neutrophil counts and an increased NLR. These findings highlight the significant involvement of neutrophils in the ENL immunological/inflammatory process.

Lupus Erythematosus Tumidus Misdiagnosed as Erythema Nodosum from Coccidioidomycosis.

Introduction: Lupus erythematosus tumidus (LET) is a rare photosensitive dermatosis that is categorized as intermittent cutaneous lupus erythematosus. It shares clinical similarities and histopathological features with other skin disorders, such as erythema nodosum, lymphocytic infiltrate of Jessner, and reticular erythematous mucinosis, thus making diagnosis quite challenging. We present a patient with LET whose diagnosis was confirmed after seeing several doctors. Case Presentation: A 52-year-old Hispanic female presented with tender erythematous nodules on her thighs for approximately 1 month. She was suspected of having erythema nodosum secondary to coccidioidomycosis and was prescribed fluconazole 200 mg for 30 days but showed no improvement. However, histopathological and direct immunofluorescence tests later confirmed a diagnosis of LET. The patient was treated with hydroxychloroquine, and the lesions improved remarkably after 2 weeks. Conclusion: LET is a rare dermatosis that closely resembles other dermatologic conditions such as erythema nodosum, lymphocytic infiltrate of Jessner, and reticular erythematous mucinosis. Diagnosis based on clinical features alone should be avoided, and ideally, treatment should only be initiated after confirmatory histopathological testing.

The causal relationship between inflammatory bowel diseases and erythema nodosum: a bidirectional two-sample mendelian randomization study.

BACKGROUND: Individuals with inflammatory bowel disease (IBD) exhibit a heightened likelihood of developing erythema nodosum (EN), but the presence of causal link is unknown. The purpose of the present research was to investigate this connection using a bidirectional two-sample Mendelian randomization (MR) analysis. METHODS: Summarized statistics for EN were sourced from the FinnGen consortium of European ancestry. The International Inflammatory Bowel Disease Genetic Consortium (IBDGC) was used to extract summary data for IBD. The inverse variance weighted (IVW) technique was the major method used to determine the causative link between them. RESULTS: The study evaluated the reciprocal causal link between IBD and EN. The IVW technique confirmed a positive causal link between IBD and EN (OR = 1.237, 95% CI: 1.109-1.37, p = 1.43 × 10- 8), as well as a strong causality connection between Crohn's disease (CD) and EN (OR = 1.248, 95% CI: 1.156-1.348, p = 1.00 × 10- 4). Nevertheless, a causal connection between ulcerative colitis (UC) and EN could not be established by the data. The reverse MR research findings indicated that analysis indicated that an increase in EN risks decreased the likelihood of UC (OR = 0.927, 95% CI: 0.861-0.997, p = 0.041), but the causal association of EN to IBD and CD could not be established. CONCLUSION: This investigation confirmed that IBD and CD had a causal connection with EN, whereas UC did not. In addition, EN may decrease the likelihood of UC. Further study must be performed to uncover the underlying pathophysiological mechanisms producing that connection.

When Salmonella Manifests Through the Skin: Erythema Nodosum in a Pediatric Case.

Erythema nodosum (EN) is a non-specific nodular dermo-hypodermic rash characterized by the sudden occurrence of painful lumps located especially in the legs following a non-specific reaction to different internal and external antigens. Clinical and histological manifestations are stereotyped, regardless of the etiology. Erythema nodosum is most frequently associated with infections, particularly bacterial and less commonly viral, fungal, and parasitic. Other conditions can be discussed, including systemic diseases, malignant tumors, medicines, and vaccines. In almost half of cases, erythema nodosum is idiopathic if no cause is found. We report a case of erythema nodosum secondary to a Salmonella infection in a seven-year-old male. The peculiarity of our observation is the initial presentation of systemic signs that preceded the gastrointestinal symptoms.

Löfgren Syndrome: A Case Report and Treatment Challenge.

Sarcoidosis is an inflammatory and immune-mediated multisystemic disorder of unknown etiology, characterized by the presence of non-caseating granulomas, impacting various organs. This indolent condition manifests with numerous nonspecific symptoms and lacks a definitive diagnostic test, typically requiring histopathologic confirmation. However, a distinct and more readily diagnosable form of sarcoidosis does exist. The Löfgren syndrome (LöS) is characterized by the triad of erythema nodosum (EN), bilateral hilar lymphadenopathy, and symmetrical inflammatory arthralgias or arthritis. The simultaneous presence of these elements obviates the necessity for a biopsy. Predominantly affecting women in their second and third decades of life, this syndrome generally carries a favorable prognosis with spontaneous resolution or the requirement for a nonsteroidal anti-inflammatory drug (NSAID) alone. Despite its rarity, in particular cases, the treatment can be more challenging. This article presents a case study of LöS in a young woman, whose more aggressive disease course led to the need for steroidal therapy.

Erythema nodosum leprosum necroticans: a case report of an atypical severe type 2 leprosy reaction and literature review.

Until now, leprosy remains a problem and challenge in the world because it can cause disability and morbidity in affected individuals, including problems due to the emergence of type 2 lepra reaction or erythema nodosum leprosum (ENL). The clinical picture of ENL can appear in an atypical and severe form, called ENL necroticans (ENN), which becomes a problem in diagnosis and therapy. We report a 17-year-old female with lepromatous leprosy and ENN who received therapy in the form of a combination of steroids and methotrexate. Four months after consuming this therapy, the ulcers on the patient's body improved, leaving atrophic and hypertrophic scars. ENN's unusual clinical presentation poses diagnostic difficulties in that its appearance does not follow the typical patterns, making it challenging to identify correctly. Furthermore, managing cases of ENN may necessitate supplementary treatment beyond steroids alone.

Clinico-Laboratory Profile of Tuberculides: An Experience at a Tertiary Care Centre from Chandigarh, India.

Background: Tuberculides are characterised by delayed-type of immunologic reactions to Mycobacterium tuberculosis or its products in immuno-competent individuals. We herein describe clinico-epidemiological features and response to treatment in patients with tuberculides from a tertiary care centre from North India. Methods: This was a retrospective analysis of the clinical records of all the cutaneous tuberculosis (TB) patients (year 2000-2019) enrolled in the TB clinic. The patient records fulfilling the diagnostic criteria of tuberculides were considered for analysis. Results: A total of 225 patients attended the tuberculosis clinic; out of this, 34 were diagnosed as tuberculides. Out of these 34 cases, 21 were identified as LS, 2 erythema induratum of Bazin, 1 papulonecrotic tuberculide, and 10 erythema nodosum. History of contact to open cases of TB was present in 15/34 (44.1%) patients. History of BCG vaccination was found in 15/34 (44.1%) patients. The focus of underlying TB could be identified in 20/34 (58.8%) patients. Skin biopsy was performed in all patients. In all patients, the diagnosis was confirmed by clinico-pathological correlation, positive TST, and the underlying focus of TB. All patients received 6 months regimen of anti-tubercular therapy with first-line drugs. Conclusion: In this study, we demonstrated various forms of tuberculides; lichen scrofulosorum was the most common form. We also included erythema nodosum cases also, which responded well to ATT. Observation from our study showed that tuberculides are important cutaneous markers for underlying tuberculosis infection, which helps in early detection of occult tuberculosis and timely management.

Split Dose of Prednisolone in the Treatment for Erythema Nodosum Leprosum: A Case Series.

Background Erythema nodosum leprosum (ENL) is an immune complex-mediated reaction that clinically presents as tender erythematous evanescent nodules, mostly associated with systemic symptoms. Oral prednisolone is the drug of choice, with doses ranging from 0.5 to 1 mg/kg. Some cases may develop new lesions and systemic symptoms despite 1 mg/kg prednisolone, and in ideal practice, physicians escalate the prednisolone dose for immediate arrest of inflammation to prevent complications. However, a high dose of prednisolone has more side effects in the long term and causes more immunosuppression. Methods In cases of ENL, those not responding to a conventional once-daily regimen were given a split dose of oral prednisolone instead of increasing the dose. They were followed up for response, and serum cortisol was measured to see for hypothalamic-pituitary-adrenal (HPA) axis suppression. Results Eight cases of ENL (three nodular, three necrotic, one pustular, and one nodulcerative) had a dramatic response to split-dose therapy without any relapse and HPA axis suppression. Conclusion A split-dosing regimen can be a good treatment option in ENL with better control, less steroid dependency, and a lower relapse rate.

Erythema nodosum as an unusual skin manifestation of long COVID: A case report.

Erythema nodosum (EN) is a skin manifestation of panniculitis characterized by symmetric, painful, tender nodules, and most cases are self-limiting. Few cases of EN following Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) vaccination have been reported, and they are generally self-limiting. We reported the challenging case of a 63-year-old Asian woman with EN that persisted for more than three months after a coronavirus disease-19 (COVID-19). There was no improvement despite topical steroid and NSAIDs treatment, and the patient was successfully treated with combination of high-dose steroid and NSAIDs. There were long-lasting symptoms involving various organ symptoms persisting over three months after COVID-19, which is known as Long COVID. As part of Long COVID, there are limited cases of skin manifestations. Given that immune dysregulation due to persistent coronaviruses may contribute to refractory EN, Erythema nodosum related to COVID-19 is rare, but can occur; clinicians should be aware of the occurrence of EN following COVID-19 infection.

Access to Urgent Care Practices Improves Understanding and Management of Endemic Coccidioidomycosis: Maricopa County, Arizona, 2018-2023.

BACKGROUND: Coccidioidomycosis within endemic regions is often undiagnosed because appropriate testing is not performed. A dashboard was developed to provide information about the prevalence of coccidioidomycosis throughout the year. METHODS: Banner Urgent Care Service has many clinics within Maricopa County, Arizona, a highly endemic region for coccidioidomycosis. All clinic visits and subset analyses for patients with International Classification of Diseases, Tenth Revision codes for pneumonia (J18.*) or erythema nodosum (L52) during 2018-2024 were included. Tabulated were daily frequencies of visits, pneumonia and erythema nodosum coding, coccidioidal testing, and test results. Banner Urgent Care Services' counts of monthly coccidioidomycosis diagnoses were compared with those of confirmed coccidioidomycosis cases reported to Maricopa County Department of Public Health. RESULTS: Monthly frequencies of urgent care coccidioidomycosis diagnoses strongly correlated with public health coccidioidomycosis case counts (r = 0.86). Testing frequency for coccidioidomycosis correlated with overall pneumonia frequency (r = 0.52). The proportion of pneumonia due to coccidioidomycosis varied between <5% and >45% within and between years. Coccidioidomycosis was a common cause of erythema nodosum (65%; 95% confidence interval, 45%-67%) and independent of pneumonia. Over half of Banner Urgent Care Services' coccidioidomycosis diagnoses were coded for neither pneumonia nor erythema nodosum. CONCLUSION: Data provided by the coccidioidomycosis dashboard can assist urgent care practitioners in knowing when coccidioidomycosis is prevalent in the community. Patients with exposure to endemic coccidioidomycosis who develop erythema nodosum or pneumonia should routinely be tested for coccidioidomycosis. Data from private health care organizations can augment surveillance of diseases important to public health.

Erythema nodosum in northern Finland between 1996 and 2019: A register-based study.

Erythema nodosum (EN) is seen at any age with varying and often unidentified etiology. We studied the etiology and characteristics of EN in Northern Finland. Medical records of all patients with a diagnosis code for EN between 1996 and 2019 from Oulu University Hospital were retrieved and analyzed. There were in total 142 EN cases with a female predominance (n = 112, 72.9%). The mean age of the patients was 35.9 years. There were five cases diagnosed with EN in those younger than 2 years of age. Almost one third had EN nodules in multiple anatomical locations. In addition to skin findings, systemic symptoms were common (81.0%), and seen more often in men (p < 0.05). In children and adolescents, the most common etiological factors were gastroenteritis caused by 'Yersinia, Salmonella or Campylobacter', followed by inflammatory bowel diseases and hormonal contraception. Bacterial infections were the most common etiological factor among adults. In 28.2% of the cases there was no identified causative factor. In this study, EN was seen surprisingly often in small children. Etiological factors varied markedly among different age groups and symptoms differed between the sexes in adults. These aspects should be taken into account when diagnosing EN patients.

Erythema Nodosum Leprosum Reaction Masquerading as Rheumatoid Arthritis.

Erythema nodosum leprosum is a type 3 hypersensitivity reaction that often presents with transient eruptions of red papules, plaques, and nodules. A 52-year-old female presented with multiple joint pain that was being treated as rheumatoid arthritis (RA), but through clinical examination, she was found to have Hansen's disease with a type 2 reaction. Hence, the importance of a thorough clinical examination is a must for the timely and correct diagnosis of patients suffering from Hansen's disease.