Erythema nodosum

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Erythema nodosum
Other namesSubacute migratory panniculitis of Vilanova and Piñol
ENlegs.JPG
Erythema nodosum in a person who had recently had streptococcal pharyngitis
Specialty Dermatology, rheumatology

Erythema nodosum (EN) is an inflammatory condition characterized by inflammation of the fat cells under the skin, resulting in tender red nodules or lumps that are usually seen on both shins. [1] It can be caused by a variety of conditions, and typically resolves spontaneously within 30 days. [2] It is common in young people aged 12–20 years.

Contents

Signs and symptoms

Pre-eruptive phase

The first signs of erythema nodosum are often flu-like symptoms such as a fever, cough, malaise, and aching joints. Some people also experience stiffness or swelling in the joints and weight loss. [3]

Eruptive stage

Erythema nodosum is characterised by 1–2-inch (25–51 mm) nodules (rounded lumps) below the skin surface, usually on the shins. These subcutaneous nodules can appear anywhere on the body, but the most common sites are the shins, arms, thighs, and torso. Each nodule typically disappears after around two weeks, though new ones may continue to form for up to six or eight weeks. [3] A new nodule usually appears red and is hot and firm to the touch. The redness starts to fade and it gradually becomes softer and smaller until it disappears. Each nodule usually heals completely without scarring over the course of about two weeks. [3] [4] Joint pain and inflammation sometimes continue for several weeks or months after the nodules appear. [5]

Less common variants of erythema nodosum include:

Causes

EN is associated with a wide variety of conditions.

Idiopathic

About 30–50% of EN cases are idiopathic (of an unknown cause). [9]

Infection

Infections associated with EN include: [5]

Autoimmune

Autoimmune disorders associated with EN include: [6]

Immune-mediated/Idiopathic granulomatous diseases

Pregnancy

Pregnancy may be associated with EN. [6]

Medications

Medications associated with EN include: [6] [9] [15]

Cancer

Cancers associated with EN include: [6]

EN may also be due to excessive antibody production in lepromatous leprosy leading to deposition of immune complexes. [17]

There is an association with the HLA-B27 histocompatibility antigen, which is present in 65% of patients with erythema nodosum. [18]

A useful mnemonic for causes is SORE SHINS (Streptococci, OCP, Rickettsia, Eponymous (Behçet), Sulfonamides, Hansen's Disease (Leprosy), IBD, NHL, Sarcoidosis. [19]

Pathophysiology

Erythema nodosum is probably a delayed hypersensitivity reaction to a variety of antigens. Although circulating immune complexes have been demonstrated in patients with inflammatory bowel disease, they have not been found in idiopathic or uncomplicated cases. [20]

Diagnosis

Erythema nodosum is diagnosed clinically. A biopsy can be taken and examined microscopically to confirm an uncertain diagnosis. [4] Microscopic examination usually reveals a neutrophilic infiltrate surrounding capillaries that results in septal thickening, with fibrotic changes in the fat around blood vessels. A characteristic microscopic finding is radial granulomas, well-defined nodular aggregates of histiocytes surrounding a stellate cleft. [5]

Additional evaluation should be performed to determine the underlying cause of erythema nodosum. This may include a full blood count, erythrocyte sedimentation rate (ESR), antistreptolysin-O (ASO) titer and throat culture, urinalysis, intradermal tuberculin test, and a chest x-ray. [21] The ESR is typically high, the C-reactive protein elevated, and the blood showing an increase in white blood cells. [4]

The ESR is initially very high and falls as the nodules of erythema nodosum. The ASO titer is high in cases associated with a streptococcal throat infection. A chest X-ray should be performed to rule out pulmonary diseases, in particular sarcoidosis and Löfgren syndrome. [4]

Treatment

Erythema nodosum is self-limiting and usually resolves itself within 3–6 weeks. A recurring form does exist, and in children, it is attributed to repeated infections with streptococcus. [17] Treatment should focus on the underlying cause. Symptoms can be treated with bed rest, leg elevation, compressive bandages, wet dressings, and nonsteroidal anti-inflammatory agents (NSAIDs). [5] NSAIDs are usually more effective at the onset of EN versus with chronic disease. [22]

Potassium iodide can be used for persistent lesions whose cause remains unknown. Corticosteroids and colchicine can be used in severe refractory cases. [23] [24] Thalidomide has been used successfully in the treatment of Erythema nodosum leprosum, [25] and it was approved by the U.S. FDA for this use in July 1998. [26] According to a 2009 meta-analysis, there is some evidence of benefit for both thalidomide and clofazimine in the treatment of erythema nodosum leprosum. [27]

Epidemiology

Erythema nodosum is the most common form of panniculitis. It is most common in the ages of 20–30, and affects women 3–6 times more than men. [4]

Eponym

The term, Subacute Migratory Panniculitis of Vilanova and Piñol, was named after the two Catalan dermatologists who provided a brief description and explanation of the disease, Xavier Montiu Vilanova (1902–1965) and Joaquin Aguade Piñol (1918–1977), in 1954, and was named in 1956. [28] [29]

Related Research Articles

<span class="mw-page-title-main">Sarcoidosis</span> Medical condition

Sarcoidosis is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly affected are the eyes, liver, heart, and brain, though any organ can be affected. The signs and symptoms depend on the organ involved. Often, no, or only mild, symptoms are seen. When it affects the lungs, wheezing, coughing, shortness of breath, or chest pain may occur. Some may have Löfgren syndrome with fever, large lymph nodes, arthritis, and a rash known as erythema nodosum.

<span class="mw-page-title-main">Panniculitis</span> Medical condition

Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue. Symptoms include tender skin nodules, and systemic signs such as weight loss and fatigue.

<span class="mw-page-title-main">Neurosarcoidosis</span> Medical condition

Neurosarcoidosis refers to a type of sarcoidosis, a condition of unknown cause featuring granulomas in various tissues, in this type involving the central nervous system. Neurosarcoidosis can have many manifestations, but abnormalities of the cranial nerves are the most common. It may develop acutely, subacutely, and chronically. Approximately 5–10 percent of people with sarcoidosis of other organs develop central nervous system involvement. Only 1 percent of people with sarcoidosis will have neurosarcoidosis alone without involvement of any other organs. Diagnosis can be difficult, with no test apart from biopsy achieving a high accuracy rate. Treatment is with immunosuppression. The first case of sarcoidosis involving the nervous system was reported in 1905.

<span class="mw-page-title-main">Clofazimine</span> Medication

Clofazimine, sold under the brand name Lamprene, is a medication used together with rifampicin and dapsone to treat leprosy. It is specifically used for multibacillary (MB) leprosy and erythema nodosum leprosum. Evidence is insufficient to support its use in other conditions though a retrospective study found it 95% effective in the treatment of Mycobacterium avium complex (MAC) when administered with a macrolide and ethambutol, as well as the drugs amikacin and clarithromycin. However, in the United States, clofazimine is considered an orphan drug, is unavailable in pharmacies, and its use in the treatment of MAC is overseen by the Food and Drug Administration. It is taken orally.

<span class="mw-page-title-main">Lupus pernio</span> Medical condition

Lupus pernio is a chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis. The name "lupus pernio" is a misnomer, as microscopically this disease shows granulomatous infiltration and does not have features of either lupus nor pernio.

<span class="mw-page-title-main">Lupus vulgaris</span> Human disease

Lupus vulgaris are painful cutaneous tuberculosis skin lesions with nodular appearance, most often on the face around the nose, eyelids, lips, cheeks, ears and neck. It is the most common Mycobacterium tuberculosis skin infection. The lesions may ultimately develop into disfiguring skin ulcers if left untreated.

<span class="mw-page-title-main">Febrile neutrophilic dermatosis</span> Medical condition

Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.

<span class="mw-page-title-main">Löfgren syndrome</span> Medical condition

Löfgren syndrome is a type of acute sarcoidosis, an inflammatory disorder characterized by swollen lymph nodes in the chest, tender red nodules on the shins, fever and arthritis. It is more common in women than men, and is more frequent in those of Scandinavian, Irish, African and Puerto Rican heritage. It was described in 1953 by Sven Halvar Löfgren, a Swedish clinician. Some have considered the condition to be imprecisely defined.

<span class="mw-page-title-main">Prurigo nodularis</span> Medical condition

Prurigo nodularis (PN), also known as nodular prurigo, is a skin disease characterised by pruritic (itchy) nodules which usually appear on the arms or legs. Patients often present with multiple excoriated lesions caused by scratching. PN is also known as Hyde prurigo nodularis, Picker's nodules, atypical nodular form of neurodermatitis circumscripta, lichen corneus obtusus.

Id reactions are types of acute dermatitis developing after days or weeks at skin locations distant from the initial inflammatory or infectious site. They can be localised or generalised. This is also known as an 'autoeczematous response' and there must be an identifiable initial inflammatory or infectious skin problem which leads to the generalised eczema. Often intensely itchy, the red papules and pustules can also be associated with blisters and scales and are always remote from the primary lesion. It is most commonly a blistering rash with itchy vesicles on the sides of fingers and feet as a reaction to fungal infection on the feet, athlete's foot. Stasis dermatitis, allergic contact dermatitis, acute irritant contact eczema and infective dermatitis have been documented as possible triggers, but the exact cause and mechanism is not fully understood. Several other types of id reactions exist including erythema nodosum, erythema multiforme, Sweet's syndrome and urticaria.

Septal panniculitis is a condition of the subcutaneous fat affecting the layer of adipose tissue that lies between the dermis and underlying fascia, of which there are two forms: acute erythema nodosum and chronic erythema nodosum.

<span class="mw-page-title-main">Fungal folliculitis</span> Inflammation of hair follicles due to fungal infection

Majocchi's granuloma is a skin condition characterized by deep, pustular plaques, and is a form of tinea corporis. It is a localized form of fungal folliculitis. Lesions often have a pink and scaly central component with pustules or folliculocentric papules at the periphery. The name comes from Domenico Majocchi, who discovered the disorder in 1883. Majocchi was a professor of dermatology at the University of Parma and later the University of Bologna. The most common dermatophyte is called Trichophyton rubrum.

Granuloma multiforme is a cutaneous condition most commonly seen in central Africa, and rarely elsewhere, characterized by skin lesions that are on the upper trunk and arms in sun-exposed areas. It may be confused with tuberculoid leprosy, with which it has clinical similarities. The condition was first noted by Gosset in the 1940s, but it was not until 1964 that Leiker coined the term to describe "a disease resembling leprosy" in his study in Nigeria.

<span class="mw-page-title-main">Nakajo syndrome</span> Medical condition

Nakajo syndrome, also called nodular erythema with digital changes, is a rare autosomal recessive congenital disorder first reported in 1939 by A. Nakajo in the offspring of consanguineous parents. The syndrome can be characterized by erythema, loss of body fat in the upper part of the body, and disproportionately large eyes, ears, nose, lips, and fingers.

Jacob Sheskin, sometimes written as Sheskin Jacob was a Russian-born Israeli physician best known for his 1964 serendipitous discovery that thalidomide can be used as a treatment for leprosy at Hadassah University in Jerusalem.

Weber–Christian disease is a cutaneous condition characterized by recurrent subcutaneous nodules that heal with depression of the overlying skin.

Mosuke Murata was a Japanese dermatologist and was the designator of erythema nodosum leprosum (ENL)(1912), the type 2 lepra reaction.

<span class="mw-page-title-main">Behçet's disease</span> Inflammatory disorder

Behçet's disease (BD) is a type of inflammatory disorder which affects multiple parts of the body. The most common symptoms include painful sores on the mucous membranes of the mouth and other parts of the body, inflammation of parts of the eye, and arthritis. The sores can last from a few days, up to a week or more. Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness. Often, the symptoms come and go.

<span class="mw-page-title-main">Skin manifestations of sarcoidosis</span> Medical condition

Sarcoidosis, an inflammatory disease, involves the skin in about 25% of patients. The most common lesions are erythema nodosum, plaques, maculopapular eruptions, subcutaneous nodules, and lupus pernio. Treatment is not required, since the lesions usually resolve spontaneously in two to four weeks. Although it may be disfiguring, cutaneous sarcoidosis rarely causes major problems.

References

  1. ↑ "Erythema Nodosum". Johns Hopkins Medicine. Retrieved 12 June 2019.
  2. ↑ Pedro-Pons, Agustín (1968). Patología y Clínica Médicas (in Spanish). Vol. 6 (3rd ed.). Barcelona: Salvat. p. 193. ISBN   978-84-345-1106-4.
  3. 1 2 3 "Information for Families Erythema nodosum". Great Ormond Street Hospital for Children NHS Foundation Trust. GOSH NHS Foundation Trust. April 2012. Ref: 2012F1224. Archived from the original on 2017-04-19. Retrieved 2017-04-19.
  4. 1 2 3 4 5 Fitzpatrick, Thomas B. (2005). Fitzpatrick's color atlas and synopsis of clinical dermatology (5th ed.). New York: McGraw-Hill. p. 148. ISBN   978-0-07-144019-6.
  5. 1 2 3 4 Schwartz, R. A.; Nervi, S. J. (2007). "Erythema nodosum: A sign of systemic disease". American Family Physician. 75 (5): 695–700. PMID   17375516.
  6. 1 2 3 4 5 Gilchrist, Heidi; Patterson, James W. (2010). "Erythema nodosum and erythema induratum (nodular vasculitis): Diagnosis and management". Dermatologic Therapy. 23 (4): 320–7. doi: 10.1111/j.1529-8019.2010.01332.x . PMID   20666819. S2CID   39695627.
  7. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN   978-1-4160-2999-1.[ page needed ]
  8. 1 2 William D. James; Timothy G. Berger; Dirk M. Elston (2011). Andrews' Diseases of the skin : clinical dermatology (11th ed.). [London]: Saunders/ Elsevier. p. 488. ISBN   978-1-4377-0314-6.
  9. 1 2 Mert, Ali; Ozaras, Resat; Tabak, Fehmi; Pekmezci, Salih; Demirkesen, Cuyan; Ozturk, Recep (2009). "Erythema Nodosum: An Experience of 10 Years". Scandinavian Journal of Infectious Diseases. 36 (6–7): 424–7. doi:10.1080/00365540410027184. PMID   15307561. S2CID   13123205.
  10. ↑ Laborada, Jennifer; Cohen, Philip R (2021). "Tuberculosis-Associated Erythema Nodosum". Cureus. 13 (12): e20184. doi: 10.7759/cureus.20184 . PMC   8723782 . PMID   35004007.
  11. ↑ Renault, Cybèle A.; Ernst, Joel D. (2015). "Mycobacterium leprae (Leprosy)". Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. pp. 2819–2831.e2. doi:10.1016/B978-1-4557-4801-3.00252-6. ISBN   978-1-4557-4801-3.
  12. ↑ Budden, Heidi; Ulloa-Gutierrez, Rolando; Dobson, Simon; Scheifele, David (August 2007). "Erythema Nodosum, Mycobacterium avium Lymphadenitis, and a 15-mm Mantoux Test". Pediatric Infectious Disease Journal. 26 (8): 764–765. doi: 10.1097/INF.0b013e31807a2fe1 . PMID   17848899.
  13. ↑ Areias, E; García E Silva, L (1984). "[Erythema nodosum and Crohn's disease]". Medicina Cutanea Ibero-latino-americana. 12 (6): 489–95. PMID   6152299.
  14. ↑ Katta, Rajani (15 April 2002). "Cutaneous sarcoidosis: a dermatologic masquerader". American Family Physician. 65 (8): 1581–1584. PMID   11989634.
  15. ↑ Bohn, S; Büchner, S; Itin, P (1997). "Erythema nodosum: 112 cases. Epidemiology, clinical aspects and histopathology". Schweizerische Medizinische Wochenschrift. 127 (27–28): 1168–76. PMID   9324739.
  16. ↑ Rogerson, S J; Nye, F J (1990). "Hepatitis B vaccine associated with erythema nodosum and polyarthritis". BMJ. 301 (6747): 345. doi:10.1136/bmj.301.6747.345. PMC   1663612 . PMID   2144199.
  17. 1 2 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon C. (2009). Robbins and Coltran Pathological Basis of Disease. Saunders/Elsevier. pp. 372–3. ISBN   978-1-4160-4930-2.
  18. ↑ Cunha, Burke A. (1999). Infectious disease pearls. Philadelphia: Hanley & Belfus. p. 105. ISBN   978-1-56053-203-3.
  19. ↑ Dr Kevin McCarthy, Cork, Ireland or DIPOUT: Drugs (e.g. sulfonamides), Infections (e.g. strep), Pregnancy, OCP, Ulcerative colitis, TB[ full citation needed ]
  20. ↑ Nguyen, Geoffrey C.; Torres, Esther A.; Regueiro, Miguel; Bromfield, Gillian; Bitton, Alain; Stempak, Joanne; Dassopoulos, Themistocles; Schumm, Philip; Gregory, Federico J.; Griffiths, Anne M.; Hanauer, Stephen B.; Hanson, Jennifer; Harris, Mary L.; Kane, Sunanda V.; Orkwis, Heather Kiraly; Lahaie, Raymond; Oliva-Hemker, Maria; Pare, Pierre; Wild, Gary E.; Rioux, John D.; Yang, Huiying; Duerr, Richard H.; Cho, Judy H.; Steinhart, A. Hillary; Brant, Steven R.; Silverberg, Mark S. (2006). "Inflammatory Bowel Disease Characteristics Among African Americans, Hispanics, and Non-Hispanic Whites: Characterization of a Large North American Cohort". The American Journal of Gastroenterology. 101 (5): 1012–23. doi:10.1111/j.1572-0241.2006.00504.x. PMID   16696785. S2CID   7720460.
  21. ↑ García-Porrúa, Carlos; González-Gay, Miguel A.; Vázquez-Caruncho, Manuel; López-Lazaro, Luis; Lueiro, Mercedes; Fernández, Maria L.; Alvarez-Ferreira, Javier; Pujol, Ramón M. (2000). "Erythema nodosum: Etiologic and predictive factors in a defined population". Arthritis & Rheumatism. 43 (3): 584–92. doi: 10.1002/1529-0131(200003)43:3<584::AID-ANR15>3.0.CO;2-6 . PMID   10728752.
  22. ↑ "Erythema Nodosum". The Lecturio Medical Concept Library. 24 July 2020. Retrieved 21 July 2021.
  23. ↑ Mat, C.; Yurdakul, S; Uysal, S; Gogus, F; Ozyazgan, Y; Uysal, O; Fresko, I; Yazici, H (2005). "A double-blind trial of depot corticosteroids in Behcet's syndrome". Rheumatology. 45 (3): 348–52. doi: 10.1093/rheumatology/kei165 . PMID   16263779.
  24. ↑ Yurdakul, Sebahattin; Mat, Cem; Tüzün, Yalçin; Özyazgan, Yilmaz; Hamuryudan, Vedat; Uysal, Ömer; Şenocak, Mustafa; Yazici, Hasan (2001). "A double-blind trial of colchicine in Behçet's syndrome". Arthritis & Rheumatism. 44 (11): 2686–92. doi: 10.1002/1529-0131(200111)44:11<2686::AID-ART448>3.0.CO;2-H . PMID   11710724. S2CID   19523919.
  25. ↑ Silverman, W. A. (2002). "The Schizophrenic Career of a 'Monster Drug'". Pediatrics. 110 (2): 404–6. doi:10.1542/peds.110.2.404. PMID   12165600.
  26. ↑ Rouhi, Maureen (20 June 2005). "THALIDOMIDE". Chemical & Engineering News Archive. 83 (25): 122. doi:10.1021/cen-v083n025.p122.
  27. ↑ Van Veen, Natasja HJ; Lockwood, Diana NJ; van Brakel, Wim H; Ramirez Jr, Jose; Richardus, Jan Hendrik (8 July 2009). "Interventions for erythema nodosum leprosum". Cochrane Database of Systematic Reviews (3): CD006949. doi:10.1002/14651858.CD006949.pub2. PMID   19588412.
  28. ↑ Subacute Nodular Migratory Panniculitis (Vilanova Disease) at eMedicine
  29. ↑ Mascaró, J. M. (1978). "In memoriam Joaquin Piñol Aguadé, 1918--1977". Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und Verwandte Gebiete. 29 (11): 613–4. PMID   363649.
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