Oculocutaneous Albinism, Platelet Storage Pool Disease, and Progressive Lupus Nephritis

Systemic lupus erythematosus (SLE) was seen in a patient with oculocutaneous albinism and platelet storage pool disease (Hermansky Pudlak syndrome). Although the patient had severely compromised platelet function, lupus nephritis developed nonetheless, which progressed to end-stage renal disease. The role of platelets in the pathogenesis of the nephritic process is discussed, and it is concluded that release of platelet granular constituents, particularly serotonin, is not required for the microvascular damage of SLE.