Bone and Soft-Tissue Disorders
= DISSEMINATED LIPOGRANULOMATOSIS
Histo: foam cell granulomas; lipid storage of neuronal tissue (accumulation of ceramide + gangliosides)
- hoarse weak cry; subcutaneous + periarticular granulomas
- swelling of extremities; generalized joint swelling
- intermittent fever, dyspnea; lymphadenopathy
- capsular distension of multiple joints (hand, elbow, knee)
- juxtaarticular bone erosions from soft-tissue granulomas
- subluxation / dislocation
- disuse / steroid deossification
Prognosis: death from respiratory failure within 2 years