Hypergammaglobulinemic Purpura of Waldenstorm is one of the uncommon conditions with purpura and is often associated with collagen vascular disease. It is difficult to treat and sometimes needs anti CD 20 molecules for resistant cases.
We present a case of 20 years old female who came to us in June 2008 with recurrent history of rash since last 1 year over both legs, rash was precipitated on prolonged standing and disappeared with rest in 2 - 3 days and was associated with pain she also complained of dryness of mouth and dryness of eyes and received steroids for last 1 year but showed no improvement. There was no history of fever, joint pains, photosensitivity. On admission she was conscious, oriented afebrile, pulse was 76/min, BP 120/76 mmHg. Systemic examination was normal. She had maculopapular rash over lower limbs which was non blanch able. Investigations showed Hemoglobin 9.9 mg%, TLC 3800/cumm, ESR 98 mm in 1st hour, CRP was positive, Renal and Liver function were within normal limits. Further skin biopsy showed leucocytoclastic vasculitis, in view of biopsy report CT scan was done to look for primary cause it revealed mediastinal lymphadenopathy, endoscopic ultrasound FNAC done revealed granulomatous changes with focal necrosis, montoux test was 25 mm and TB quantiferon gold test was positive. So patient was diagnosed as having tubercular mediastinal lymphadenopathy with leucocytoclastic vasculitis and was started on 4 drugs antitubercular drugs. She was subsequentely followed up as outpatient, after 8 weeks she came with similar complaints so she underwent further test. Her Rheumatoid factor was positive. ANA was positive in fine speckled pattern, ENA profile by BINDAZYME IMMUNOASSAY showed high titre of SSA/RO, SSB/La [
Hypergammaglobulinemic purpura of Waldenström is characterized by hypergammaglobulinemia, recurring purpura, an elevated erythrocyte sedimentation rate, and the presence of rheumatoid factor indicative of circulating immune complexes. There is an association with autoimmune diseases, especially Sjögren’s syndrome [