Letter to the Editor| Volume 360, ISSUE 4, P419-422, October 2020

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Löfgren Syndrome in Histoplasma Endemic Rural America

      Löfgren syndrome (LS), first described by the Swedish pulmonologist Sven Löfgren in 1946, is one of the most familiar syndromes in medicine. In the original investigative paper, LS represented a specific phenotypic presentation of sarcoidosis and was associated with an excellent long term prognosis.
      • Löfgren S.
      • Erythema N.
      Studies on etiology and pathogenesis in 185 adult cases.
      The onset of LS was characteristically acute with the constellation of the following findings: bilateral hilar lymphadenopathy (BHL) on chest radiography, erythema nodosum (EN), and arthritis or periarthritis, commonly involving the ankle joints.
      • Grunewald J.
      • Eklund A
      Löfgren's syndrome: human leukocyte antigen strongly influences the disease course.
      Subsequently, the presence of EN was determined to be a conditional criterion, as there was a significant difference in incidence between genders. Guidelines have proposed that a confident clinical diagnosis of sarcoidosis can be made without any tissue evidence if LS is the initial presentation.
      Statement on sarcoidosis.
      However, this can be challenging, especially in areas where fungal infections are endemic, and the availability of subspecialty expertise is few and far between. Identification of LS is vital, as most patients can be managed without a commitment to long term systemic steroid therapy and avoidance of complications from such treatment. Genetic studies have further identified that a particular subgroup of patients with LS who are HLA-DRB1×03 positive, to have an even better prognosis.
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