Waldenstrom hypergammaglobulinemic purpura as the initial manifestation of pediatric primary Sjogren’s syndrome

Abstract

Hypergammaglobulinemic Purpura of Waldenström (HGPW) is an uncommon benign condition that can be associated with rheumatic diseases.

Objective: To report a clinical case of HGPW as the initial manifestation of primary pediatric Sjögren’s syndrome (SS), an unknown condition in pediatrics, which makes its diagnosis and management challenging.

Clinical Case: A 6-year-old female with symmetrical purpura on lower extremities, residual hyperpigmentation, ankle arthralgia, and recurrent leg edema without other manifestations. Laboratory tests showed eosinophilia, elevated erythrocyte sedimentation rate (ESR), antinuclear antibodies (ANA) titer 1/160 with speckled pattern, and rheumatoid factor (RF) as well as no cytopenia nor complement and urinalysis alterations. Skin biopsy showed leukocytoclastic vasculitis. Prednisone was started with a good response initially, but with recurrence of skin symptoms. After 4 months of treatment, a new analysis showed ANA titer 1/1280 with speckled pattern, positive antibodies against extractable nuclear antigens (ENA) anti-Ro and anti-La, high serum immunoglobulin G (IgG) titers (2,900 mg/dL), hypergammaglobulinemia on serum protein electrophoresis, and normal Schirmer test. Recurrent parotitis was reported and salivary gland biopsy was compatible with SS. HGPW secondary to SS was suspected, therefore, she was treated with prednisone and hydroxychloroquine, then azathioprine and mycophenolate mofetil; she developed corticosteroid dependence and deterioration of her quality of life. She was treated with rituximab without recurrence at one year of follow-up.

Conclusions: HGPW is a rare skin manifestation in pediatrics that can be associated with systemic diseases, thus it requires a comprehensive clinical and laboratory study, especially in diseases such as SS and systemic lupus erythematosus (SLE).