-
American Journal of Clinical Dermatology May 2021Erythema nodosum is the most common form of panniculitis and is characterized by tender erythematous nodules mainly in the lower limbs on the pretibial area. The exact... (Review)
Review
Erythema nodosum is the most common form of panniculitis and is characterized by tender erythematous nodules mainly in the lower limbs on the pretibial area. The exact cause of erythema nodosum is unknown, although it appears to be a hypersensitivity response to a variety of antigenic stimuli. Although the etiology is mostly idiopathic, ruling out an underlying disease is imperative before diagnosing primary erythema nodosum. Erythema nodosum can be the first sign of a systemic disease that is triggered by a large group of processes, such as infections, inflammatory diseases, neoplasia, and/or drugs. The most common identifiable causes are streptococcal infections, primary tuberculosis, sarcoidosis, Behçet disease, inflammatory bowel disease, drugs, and pregnancy. We propose a diagnostic algorithm to optimize the initial work-up, hence initiating prompt and accurate management of the underlying disease. The algorithm includes an initial assessment of core symptoms, diagnostic work-up, differential diagnosis, and recommended therapies. Several treatment options for the erythema nodosum lesions have been previously reported; nevertheless, these options treat the symptoms, but not the triggering cause. Making an accurate diagnosis will allow the physician to treat the underlying cause and determine an optimal therapeutic strategy.
Topics: Anti-Inflammatory Agents; Diagnosis, Differential; Drug Therapy, Combination; Erythema Nodosum; Humans; Incidence; Skin; Treatment Outcome
PubMed: 33683567
DOI: 10.1007/s40257-021-00592-w -
Frontiers in Immunology 2019Leprosy is an infectious disease caused by the intracellular bacillus that mainly affects the skin and peripheral nerves. One of the most intriguing aspects of leprosy... (Review)
Review
Leprosy is an infectious disease caused by the intracellular bacillus that mainly affects the skin and peripheral nerves. One of the most intriguing aspects of leprosy is the diversity of its clinical forms. Paucibacillary patients are characterized as having less than five skin lesions and rare bacilli while the lesions in multibacillary patients are disseminated with voluminous bacilli. The chronic course of leprosy is often interrupted by acute episodes of an inflammatory immunological response classified as either reversal reaction or erythema nodosum leprosum (ENL). Although ENL is considered a neutrophilic immune-complex mediated condition, little is known about the direct role of neutrophils in ENL and leprosy disease overall. Recent studies have shown a renewed interest in neutrophilic biology. One of the most interesting recent discoveries was that the neutrophilic population is not homogeneous. Neutrophilic polarization leads to divergent phenotypes (e.g., a pro- and antitumor profile) that are dynamic subpopulations with distinct phenotypical and functional abilities. Moreover, there is emerging evidence indicating that neutrophils expressing CD64 favor systemic inflammation during ENL. In the present review, neutrophilic involvement in leprosy is discussed with a particular focus on ENL and the potential of neutrophils as clinical biomarkers and therapeutic targets.
Topics: Animals; Erythema Nodosum; Humans; Leprosy; Neutrophils; Skin; Skin Diseases
PubMed: 30949168
DOI: 10.3389/fimmu.2019.00495 -
Cureus Dec 2021Erythema nodosum is panniculitis that is frequently observed in women aged 18 to 34 years. It usually occurs as an idiopathic condition; however, it may be associated...
Erythema nodosum is panniculitis that is frequently observed in women aged 18 to 34 years. It usually occurs as an idiopathic condition; however, it may be associated with drugs, infections, malignancy, pregnancy, and systemic illnesses. Erythema nodosum presents with the sudden onset of tender, warm, erythematous nodules typically on the ankles, knees, and shins. Although the pathogenesis has not been fully elucidated, evidence supports a delayed type IV hypersensitivity reaction. It is often a clinical diagnosis that does not require a biopsy; appropriate work-up and careful medication history are crucial to identifying an underlying etiology if present. This report describes a woman from Vietnam, a tuberculosis endemic country, who presented with erythema nodosum that was determined to be a sequela of latent tuberculosis. Several studies have demonstrated an association between erythema nodosum and tuberculosis, especially in endemic regions. Summarized data reveals the incidence of tuberculosis-associated erythema nodosum to be six percent; however, when individuals with either secondary erythema nodosum or infection-associated erythema nodosum are evaluated, the incidence of tuberculosis-associated erythema nodosum is 11% or 21%, respectively. Evaluation of erythema nodosum should include a tuberculin or QuantiFERON test, chest roentgenogram, and/or an acid-fast bacilli sputum culture if the diagnosis of tuberculosis is being considered.
PubMed: 35004007
DOI: 10.7759/cureus.20184 -
Journal of General Internal Medicine May 2021
Topics: Erythema Nodosum; Humans; Hypersensitivity
PubMed: 33515194
DOI: 10.1007/s11606-021-06620-z -
Dermatology Online Journal Jun 2002Erythema nodosum is the most frequent clinico-pathological variant of the panniculitides. The disorder is a cutaneous reaction consisting of inflammatory, tender,... (Review)
Review
Erythema nodosum is the most frequent clinico-pathological variant of the panniculitides. The disorder is a cutaneous reaction consisting of inflammatory, tender, nodular lesions, usually located on the anterior aspects of the lower extremities. The process may be associated with a wide variety of diseases, being infections, sarcoidosis, rheumatologic diseases, inflammatory bowel diseases, medications, autoimmune disorders, pregnancy, and malignancies the most common associated conditions. The typical eruption consists of a sudden onset of symmetrical, tender, erythematous, warm nodules and raised plaques usually located on the shins, ankles and knees. Often the lesions are bilaterally distributed. At first, the nodules show a bright red color, but within a few days they become livid red or purplish, and finally they exhibit a yellow or greenish appearance taking on the look of a deep bruise. Ulceration is never seen and the nodules heal without atrophy or scarring. Some clinical variants of erythema nodosum have been described under different names, including erythema nodosum migrans, subacute nodular migratory panniculitis, and chronic erythema nodosum, but probably they are just clinical variants which may all be included within the spectrum of erythema nodosum. Histopathologically, erythema nodosum is the stereotypical example of a mostly septal panniculitis with no vasculitis. The septa of subcutaneous fat are always thickened and variously infiltrated by inflammatory cells that extend to the periseptal areas of the fat lobules. The composition of the inflammatory infiltrate in the septa varies with age of the lesion. In early lesions edema, hemorrhage, and neutrophils are responsible for the septal thickening, whereas fibrosis, periseptal granulation tissue, lymphocytes, and multinucleated giant cells are the main findings in late stage lesions of erythema nodosum. A histopathologic hallmark of erythema nodosum is the presence of the so-called Miescher's radial granulomas, which consist of small, well-defined nodular aggregations of small histiocytes arranged radially around a central cleft of variable shape. Treatment of erythema nodosum should be directed to the underlying associated condition, if identified. Usually, nodules of erythema nodosum regress spontaneously within a few weeks, and bed rest is often sufficient treatment. Aspirin, nonsteroidal anti-inflammatory drugs, such as oxyphenbutazone, indomethacin or naproxen, and potassium iodide may be helpful drugs to enhance analgesia and resolution. Systemic corticosteroids are rarely indicated in erythema nodosum and before these drugs are administered an underlying infection should be ruled out.
Topics: Diagnosis, Differential; Erythema Induratum; Erythema Nodosum; Humans; Prognosis
PubMed: 12165214
DOI: No ID Found -
Annals of the Rheumatic Diseases May 2022Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory...
The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology points to consider for diagnosis and management of autoinflammatory type I interferonopathies: CANDLE/PRAAS, SAVI and AGS.
OBJECTIVE
Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of 'points to consider' to improve diagnosis, treatment and long-term monitoring of patients with these rare diseases.
METHODS
Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates and an allied healthcare professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires and consensus methodology, 'points to consider' to guide patient management were developed.
RESULTS
The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI and AGS.
CONCLUSION
These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treatment and management of patients with CANDLE/PRAAS, SAVI and AGS and aim to standardise and improve care, quality of life and disease outcomes.
Topics: Autoimmune Diseases of the Nervous System; Erythema Nodosum; Fingers; Humans; Nervous System Malformations; Quality of Life; Rheumatology; Skin Diseases
PubMed: 35086813
DOI: 10.1136/annrheumdis-2021-221814 -
Indian Journal of Pharmacology 2022The objective of the study was to assess the efficacy and safety profiles of combined treatment of prednisolone with thalidomide (Gr-A) and prednisolone with clofazimine...
OBJECTIVE
The objective of the study was to assess the efficacy and safety profiles of combined treatment of prednisolone with thalidomide (Gr-A) and prednisolone with clofazimine (Gr. B) in patients with erythema nodosum leprosum (ENL) or type 2 lepra reactions.
MATERIALS AND METHODS
Efficacy of both regimens was assessed on the basis of clinical recovery of recurrent ENL measured by reaction severity score (RSS), Visual Analog Scale (VAS), and recurrence of type 2 lepra reaction. The causality assessment of adverse drug reactions was done using the WHO UMC causality assessment scale.
RESULTS
The average age of patients with recurrent ENL was 42.8 years (male) and 51.8yrs (female) and had mean duration of leprosy and recurrent ENL 2.4 years and 2.09 years, respectively. 80% of nonrecurrence was observed in Gr-A versus 66% in Gr-B. Significant (P < 0.05) lower RSS and VAS was found in both the treatment groups as compared to pretreatment value. The reduction in RSS and VAS was statistically significant (P < 0.05) in Gr-A compared to Gr-B treatment.
CONCLUSION
Thalidomide combination with steroid was found to be more efficacious than clofazimine combination with steroid in the treatment of ENL both the treatment regimens showed few tolerable side effects. Improved strategies for the treatment and management of these reactions need to be developed.
Topics: Adult; Clofazimine; Drug-Related Side Effects and Adverse Reactions; Erythema Nodosum; Female; Humans; Leprostatic Agents; Leprosy, Lepromatous; Male; Prednisolone; Thalidomide
PubMed: 35848688
DOI: 10.4103/ijp.ijp_946_21 -
Reumatismo May 2022The current coronavirus disease 2019 (COVID-19) pandemic is a global challenge with strong medical and socioeconomic implications. Hopes have been placed in the...
The current coronavirus disease 2019 (COVID-19) pandemic is a global challenge with strong medical and socioeconomic implications. Hopes have been placed in the development of various vaccines. As the vaccination campaign is in progress, adverse effects need to be monitored closely. Possible side effects range from minor events to more serious manifestations. In this article, we describe two cases of erythema nodosum (EN) after COVID-19 vaccination in two previously healthy female patients of 59 and 51 years, respectively. Most of the usual etiologies of EN were excluded by laboratory testing. EN was successfully treated with corticosteroids. Remarkably, in the first case, a relapse occurred 48 hours after the second dose of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine. In this case series, we describe two unusual occurrences of EN after vaccination with an mRNA COVID-19 vaccine and a viral vector vaccine, respectively, and we discuss the available related literature.
Topics: COVID-19; COVID-19 Vaccines; Erythema Nodosum; Female; Humans; SARS-CoV-2; Viral Vaccines
PubMed: 35506318
DOI: 10.4081/reumatismo.2022.1475 -
Reumatologia 2016Erythema nodosum is the most common form of panniculitis. It may have many aetiological factors. Erythema nodosum occurs three to five times more often in female... (Review)
Review
Erythema nodosum is the most common form of panniculitis. It may have many aetiological factors. Erythema nodosum occurs three to five times more often in female patients. It appears as erythematous painful rounded nodules, located most often on the anterior surface of the lower extremities, and may be accompanied by systemic symptoms such as fever, malaise and arthralgia. During diagnosis, oncological vigilance should be maintained, because erythema nodosum may be a paraneoplastic symptom. It requires an interdisciplinary approach and exclusion of many underlying causes.
PubMed: 27407284
DOI: 10.5114/reum.2016.60217 -
Journal of Vascular Surgery. Venous and... Mar 2017Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other... (Review)
Review
OBJECTIVE
Chronic wounds are a major cause of morbidity and mortality. Approximately 20% to 23% of nonhealing wounds that are refractory to vascular intervention have other causes, including vasculitis, pyoderma gangrenosum, and other autoimmune diseases. The purpose of this article was to review the literature across medical and surgical specialties with regard to refractory chronic wounds associated with vasculitis and autoimmune diseases and to delineate clinical outcomes of these wounds in response to vascular and other interventions.
METHODS
An electronic search encompassing MEDLINE, PubMed, Cochrane Library, and Scopus was completed using the following search terms: rheumatoid arthritis; systemic sclerosis; systemic lupus erythematosus; antineutrophil cytoplasmic antibody-associated vasculitis; mixed connective tissue disease; antiphospholipid syndrome; pyoderma gangrenosum; thromboangiitis obliterans; cryoglobulinemia; hydroxyurea; sickle cell; atrophie blanche; livedoid vasculitis; cholesterol emboli; calciphylaxis; antiphospholipid antibodies; prothrombotic; combined with the terms: chronic wound and leg ulcer. Full-text articles published in English up to March 1, 2016, that investigated the clinical outcomes of chronic wounds associated with autoimmune diseases were included. Review articles and evaluations of management of chronic wounds were also reviewed. Primary outcomes included in the review were amputation, ulcer healing, reduction in wound size, overall survival, and freedom from reintervention. Owing to the heterogeneity of data reporting among articles, qualitative analysis is also reported.
RESULTS
Vasculitis and autoimmune diseases play a role in 20% to 23% of patients with chronic lower extremity ulcers. Furthermore, patients with autoimmune disease have a significantly high rate of split thickness skin graft failure (50% compared to 97% in patients without autoimmune disease; P = .0002). The management of leg ulcers associated with autoimmune diseases is discussed.
CONCLUSIONS
Autoimmune and vasculitic causes should be considered in patients with chronic wounds who do not respond to appropriate vascular intervention and standard local wound care. A multidisciplinary approach with the involvement of rheumatologists allows investigation for underlying systemic disease and improves clinical outcomes for many of these challenging patients.
Topics: Anemia, Sickle Cell; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antiphospholipid Syndrome; Antirheumatic Agents; Antisickling Agents; Arthritis, Rheumatoid; Autoimmune Diseases; Calciphylaxis; Cardiovascular Agents; Chronic Disease; Cryoglobulinemia; Diagnosis, Differential; Embolism, Cholesterol; Erythema Nodosum; Humans; Hydroxyurea; Leg Ulcer; Panniculitis; Pyoderma Gangrenosum; Steroids; Thromboangiitis Obliterans; Vasculitis; Wound Healing
PubMed: 28214498
DOI: 10.1016/j.jvsv.2016.09.006