Pictorial CME

Löfgren’s Syndrome

PP Chakraborty* , K Chatterjee**

*Assistant Professor; **Professor and Head of the Department, Department of Medicine, Midnapore Medical College, West Bengal - 721 101. Received : 11.6.2008; Revised : 1.8.2008; Accepted : 4.9.2008

A 34 year old lady presented with high grade fever (101oF-103oF) and tender reddish nodules over extensor aspects of her legs and forearms for one week. She soon developed painful swelling of both ankles and right knee joints. Clinical examination revealed erythema nodosum (EN) (Fig. 1), arthritis of the aforesaid joints (Fig. 2) with some degree of periarticular inflammation.

Complete blood count and baseline biochemistry was normal except for raised ESR (64 mm). ASO titre, rheumatoid factor and ANF were negative. Mantoux test was positive (19 mm with 10TU) (Fig. 3). Chest X-ray documented bilateral hilar lymphadenopathy (BHL) (Fig. 4), which was confirmed with a CT scan thorax. Workup for HIV, tuberculosis, infectious mononucleosis, brucellosis, and leprosy was negative. The serum angiotensin-converting enzyme (ACE) level was 107.3 U/L; (Normal: 8-52U/L). CT guided FNAC from hilar node showed few epithelial like cells with occasional lymphocytes and few giant cells at places suggestive of granulomatous disease. Mycobacterial and fungal cultures of the aspirate did not grow any organism. Transbronchial lung biopsy (TBLB) revealed noncaseating epithelioid cell granulomas with giant cells typical of sarcoidosis. The patient improved significantly following institution of corticosteroids and remission has been maintained for the past six months.

Löfgren’s syndrome is an acute form of sarcoidosis characterized by EN, BHL, and polyarthralgia or polyarthritis. This case fulfilled all the criteria of Löfgren’s syndrome. The interesting feature in this patient is the Mantoux positivity. References in the literature have demonstrated that a positive tuberculin test, while very unusual in sarcoidosis, does not preclude the diagnosis. The incidence of tuberculin positivity in proved cases of sarcoidosis is around 24%1,2 but in India, it may be as high as 40%.3 Moreover, in inactive cases of sarcoidosis especially in cases with an acute course in the beginning conversion of tuberculin skin test may be seen.

References

  1. Gurkan OU, Celik G, Kumbasar O, Kaya A, Alper D. Sarcoidosis in Turkey: 1954-2000. Ann Saudi Med 2004;24:36-9.
  2. Yanardag H, Pamuk ON, Uygun S, Demirci S, Karayel T. Sarcoidosis: child vs adult. Indian J Pediatr 2006;73:143-5.
  3. Gupta SK, Gupta S. Sarcoidosis in India: a review of 125 biopsy-proven cases from eastern India. Sarcoidosis. 1990;7:43-9.
  4. Li S, Liu C. The tuberculin test in intrathoracic sarcoidosis. Zhonghua Jie He He Hu Xi Za Zhi 1998;21:94-7.