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Löfgren syndrome
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Löfgren syndrome

Contributors: Benjamin L. Mazer MD, MBA
Other Resources UpToDate PubMed

Synopsis

Löfgren syndrome is a constellation of symptoms that is nearly always suggestive of acute sarcoidosis. The primary symptoms are erythema nodosum and hilar lymphadenopathy. Arthritis, uveitis, and fever also frequently occur. Primary tuberculosis can rarely present with hilar lymphadenopathy and erythema nodosum. The simultaneous presence of all the typical features of Löfgren syndrome, however, is highly specific for sarcoidosis.

Löfgren syndrome predominantly occurs in female patients of Northern European descent. Unlike other manifestations of sarcoidosis, Löfgren syndrome usually resolves within 2 years and rarely relapses. NSAIDs are the typical treatment, with immunosuppression sometimes required.

Codes

ICD10CM:
D86.86 – Sarcoid arthropathy

SNOMEDCT:
238676008 – Lofgrens syndrome

Differential Diagnosis & Pitfalls

Differential diagnosis depends on presenting symptoms.

See Erythema nodosum for a differential of skin findings, and also consider:
  • Lupus panniculitis
  • Ecchymoses
  • Acute febrile neutrophilic dermatosis
  • Fat necrosis
  • Drug-induced Erythema nodosum (eg, oral contraceptives)
  • Inflammatory bowel disease (eg, Crohn disease, Ulcerative colitis)
  • Fungal infections (eg, Histoplasmosis)
  • Bacterial infections (eg, Cellulitis, Yersinia enterocolitica infection)
Hilar lymphadenopathy:
  • Cat-scratch disease
  • Malignancy (particularly Lymphoma, Lung cancer, metastasis)
  • Fungal infection (eg, Histoplasmosis, Coccidioidomycosis)
  • Histiocytic diseases
  • Staphylococcus aureus pneumonia
  • Human immunodeficiency virus primary infection
  • Tuberculosis
  • Systemic lupus erythematosus
  • Pneumoconiosis (eg, Silicosis, Asbestosis)
Arthritis:
  • Rheumatoid arthritis
  • Reactive arthritis
  • Adult onset Still disease
  • Systemic lupus erythematosus
  • Psoriatic arthritis
  • Ankylosing spondylitis
  • Other connective tissue diseases
  • Septic arthritis
  • Osteoarthritis
  • Trauma
  • Avascular necrosis
  • Tendonitis
  • Bursitis
  • Meniscal disease
Uveitis:
  • HLA-B27 autoimmune diseases (eg, inflammatory bowel disease [Ulcerative colitis, Crohn disease], Ankylosing spondylitis, Psoriasis)
  • Behçet syndrome
  • Viral infection (eg, herpes viruses [Herpes simplex virus keratitis, Herpes zoster ophthalmicus]),
  • Bacterial infections (eg, Ocular syphilis, mycobacteria)
  • Toxoplasmosis
  • Fungal infections (eg, Cryptococcosis)
  • Acute angle-closure glaucoma
  • Conjunctivitis (eg, Viral conjunctivitis, Bacterial conjunctivitis, Allergic conjunctivitis)
  • Scleritis (eg, Diffuse scleritis)
  • Episcleritis (eg, Diffuse episcleritis)
  • Ocular foreign body
  • Corneal abrasion

Best Tests

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References

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Last Updated:05/26/2016
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Löfgren syndrome
A medical illustration showing key findings of Löfgren syndrome : Fever, Hilar lymphadenopathy, Malaise, Migrating polyarthralgia, Arthralgia
Copyright © 2024 VisualDx®. All rights reserved.