Rare manifestations of refractory extrapulmonary sarcoidosis

Abstract

Sarcoidosis is a multisystemic disease, characterised by histopathologic presence of non-necrotising granulomas, primarily affecting the lungs (>90%). We present three cases predominantly characterised by uncommon extrapulmonary manifestations of sarcoidosis, where only one had pulmonary involvement. We describe three female patients, between the second and third decades of life, whose sarcoidosis was a diagnostic challenge due to their atypical extrapulmonary manifestations, from which we highlight: livedo reticularis and painful subcutaneous nodules with uncommon localisation, size and histology, being the first reported case of extensive subcutaneous nodules triggered by intramuscular penicillin; extensive symptomatic and refractory osseous involvement; and dispersed erythema nodosum affecting the entire body surface. All three patients required third-line treatment (antitumour necrosis factor agents) to achieve significant clinical and imagiological improvement. Through this case series, we highlight the importance of considering the rare and atypical presentations of sarcoidosis to avoid diagnostic delays and serious repercussions on the patient's prognosis.

Keywords: Biological agents; Rheumatology.

Figure 1

Livedo reticularis on both distal lower extremities.

Figure 2

MRI of the lumbosacral spine with multiple diffuse lesions on vertebral bodies and voluminous L5-S1 disc herniation (white arrow).

Figure 3

18F-fluorodeoxyglucose positron emission tomography/CT with adenopathies and multiple axial and appendicular hypermetabolic areas.

Figure 4

Diffuse erythema nodosum on the left lower limb (A) and abdomen (B).