Extrathoracic sarcoidosis with giant splenomegaly and cranial osteolysis

Isolated extrathoracic sarcoidosis is a rare entity. We present a 25-year old woman with recurrent episodes of fever of unknown origin in the 10 years previously, with a new episode of fever with hepatosplenomegaly, abdominal adenopathies and cranial osteolysis without pulmonary or mediastinal affectation. With the suspicion of linfoproliferative disease we have resected the spleen with a weight of 1500 g. and multiple non caseous granulomata also in the liver and adenopathies. The clinical presentation is compatible with an atypical sarcoidosis without thoracic affectation and with two rare associated clinical manifestation namely giant splenomegaly and sarcoidosis of the skull.