Rahul Sharma (Editor)

Erythema nodosum

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ICD-10
  
L52

DiseasesDB
  
4462

eMedicine
  
derm/138

ICD-9-CM
  
695.2, 017.1

MedlinePlus
  
000881

Erythema nodosum

Specialty
  
Dermatology, rheumatology

Erythema nodosum (EN), also known as Subacute Migratory Panniculitis of Vilanova and Piñol, is an inflammatory condition characterised by inflammation of the fat cells under the skin, resulting in tender red nodules or lumps that are usually seen on both shins. It can be caused by a variety of conditions, and typically resolves spontaneously within 30 days. It is common in young people between 12–20 years of age.

Contents

Pre-eruptive phase

The first sign of erythema nodosum is often flu-like symptoms. It may involve a pyrexia, weakness and arthralgia.

Eruptive stage

It is characterized by the appearance of dermo-hypodermic rounded nodules which may be bright red or purplish, bilaterally symmetrical, located on the extensor surface of the legs. They are movable relative to the deep plane and skin. They generally disappear in 1–6 weeks without sequelae.

Erythema nodosum is characterised by red nodules that are tender and warm. Nodules occur under the skin, classically in front of the shins. Nodules may occur anywhere there is fat under the skin, including the thighs, arms, trunk, face, and neck.

Erythema nodosum may occur concurrently with fever, malaise, and joint pain and inflammation. Nodules vary from 1–10 cm in diameter, and may coalesce to form large areas of hardened skin.

As the nodules age, they become bluish purple, brownish, yellowish, and finally green, similar to the color changes that occur in a resolving bruise. The nodules usually subside over a period of 2–6 weeks without ulceration or scarring.

Less common variants of erythema nodosum include:

  • Ulcerating forms, seen in Crohn's disease
  • Erythema contusiforme, an erythema nodosum lesion with subcutaneous haemorrhage
  • Chronic erythema nodosum, more likely to be unilateral and migratory.
  • Erythema nodosum migrans, (also known as subacute nodular migratory panniculitis of Vilanova and Piñol), a form of erythema nodosum with lesions that spread centrifugally with central clearing, and a form of chronic erythema nodosum.) is a form of septal panniculitis that is much less common than acute erythema nodosum.
  • Lofgren's syndrome, a form of acute sarcoidosis with erythema nodosum, bilateral hilar adenopathy and fever, often accompanied by joint symptoms.
  • Causes

    EN is associated with a wide variety of conditions, including:

    Idiopathic

    In about 30–50% of cases, the cause of EN is unknown.

    Infection
  • Streptococcal infection which, in children, is by far the most common precipitant,
  • Primary infection of Tuberculosis
  • Mycoplasma pneumoniae
  • Histoplasma capsulatum
  • Yersinia
  • Epstein-Barr virus
  • Coccidioides immitis (Valley fever)
  • Cat scratch disease
  • Autoimmune disorders, including
  • Inflammatory bowel disease (IBD)
  • Behçet's disease
  • Sarcoidosis
  • Pregnancy
    Medications, including
  • Sulfonamides
  • Penicillins
  • Oral contraceptives
  • Bromides
  • Hepatitis B vaccination
  • Cancer, including
  • Non-Hodgkins lymphoma (NHL)
  • Carcinoid tumours
  • Pancreatic cancer
  • EN may also be due to excessive antibody production in lepromatous leprosy leading to deposition of immune complexes.

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

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

    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.

    Diagnosis

    Erythema nodosum is diagnosed clinically. A biopsy can be taken and examined microscopically to confirm an uncertain diagnosis. 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.

    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. The ESR is typically high, the C-reactive protein elevated, and the blood showing an increase in white blood cells.

    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.

    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. Treatment should focus on the underlying cause. Symptoms can be treated with bedrest, leg elevation, compressive bandages, wet dressings, and nonsteroidal anti-inflammatory agents (NSAIDs). NSAIDs are usually more effective at the onset of EN versus with chronic disease.

    Potassium iodide can be used for persistent lesions whose cause remains unknown. Corticosteroids and colchicine can be used in severe refractory cases. Thalidomide has been used successfully in the treatment of Erythema nodosum leprosum, and it was approved by the U.S. FDA for this use in July 1998.

    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.

    About 15 percent of patients with inflammatory bowel disease develop erythema nodosum.

    Eponym

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

    References

    Erythema nodosum Wikipedia