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Case Reports
. 2024 Jan 15;16(1):e52317.
doi: 10.7759/cureus.52317. eCollection 2024 Jan.

Löfgren Syndrome: A Mosaic of Sarcoidosis Phenotypes

Francisca Martins  1 , Miguel Martins  1 , Rui Malheiro  1
Affiliations
Case Reports

Löfgren Syndrome: A Mosaic of Sarcoidosis Phenotypes

Francisca Martins et al. Cureus. .

Abstract

Sarcoidosis is an autoimmune multisystemic granulomatous disease with an unknown etiology. Löfgren syndrome (LS), an infrequent initial presentation of acute sarcoidosis, is characterized by the classic triad of acute arthritis, erythema nodosum (EN), and bilateral hilar lymphadenopathy (BHL). The presence of this triad offers high diagnostic specificity for sarcoidosis, eliminating the need for a confirmatory biopsy. Typically, LS follows a predictable, self-limiting clinical course. However, atypical presentations require early suspicion and closer monitoring. This case report highlights an unusual clinical manifestation of LS, marked by an incomplete presentation with acute panniculitis and joint lesions in the absence of EN. Acute sarcoidosis should be considered among the differential diagnoses when these clinical manifestations are present, and chest radiography should be performed to rule out BHL. In atypical cases, the disease course becomes less predictable, as exemplified in our case, where recurrence of the disease may occur, necessitating consistent monitoring.

Keywords: acute sarcoidosis; löfgren syndrome; panniculitis; sarcoid arthritis; subcutaneous sarcoidosis.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Photograph sent by our patient a day before her hospital admission, documenting the bilateral ankle panniculitis, a skin-colored subcutaneous swelling in lateral view of the right foot (a) and both feet (b).
Figure 2
Figure 2. Grayscale ultrasound showing hypoechoic subcutaneous tissue (asterisks), decreased echogenicity of the dermis, and increased size and echogenicity of the fatty lobules (triangles) of the subcutaneous tissue.
Figure 3
Figure 3. A PA chest x-ray revealing the classic pattern of sarcoidosis: 123 sign or Garland's triad. The characteristics of this sign include mediastinal enlargement, usually right paratracheal (1) and bilateral hilar nodes, on the right (2) and left (3), due to lymphadenopathy.
PA, posteroanterior
Figure 4
Figure 4. Contrast thoracic CT, in coronal (a) and transverse (b) plane, revealing a classic 123 sign with bilateral hilar (2 and 3) and paratracheal (1) lymphadenopathy, with no other abnormalities.
CT, computed tomography
Figure 5
Figure 5. (a) Microscopic view of a granuloma (G), highlighting immune cells such as macrophages and lymphocytes in distinctive violet hues following Giemsa staining; (b) microscopic view of a granuloma structure within a cell block sample, visualized after hematoxylin and eosin staining, showcasing the distinctive cellular arrangement and morphology of a non-caseating granuloma (G), on a background of lymphocytes (arrows).
Biopsy slides’ images were provided to the Pathology Department team at the Portuguese Oncology Institute, Lisbon.
See this image and copyright information in PMC

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