Hypergammaglobulinemic purpura of Waldenström

Hypergammaglobulinaemic Purpura Associated with IgG Subclass Imbalance and Recurrent Infection

“…HP is an extremely rare disorder in childhood [3], characterised by recurrent purpura, polyclonal hypergammaglobulinaemia and elevated ESR [1,2]. The purpura is usually bilateral, symmetrical, starting on the feet and legs, and may extend to involve the thighs, buttocks, trunk and occasionally upper extremities [1,4].The cutaneous eruptions usually appear as petechial scattered lesions which later become con¯uent, with residual hyperpigmentation [1].…”

“…Hypergammaglobulinaemic purpura (HP) is a rare chronic relapsing disorder characterised by recurrent purpuric skin rash, polyclonal hypergammaglobulinaemia, elevated erythrocyte sedimentation rate (ESR) and rheumatoid factor (RF) positivity [1,2]. It is divided into a primary and a secondary type, associated with underlying disease such as systemic autoimmune rheumatic diseases (systemic lupus erythematosus, Sjo Ègren syndrome) [1,3,4].…”

Purpuras

“…44 L'évolution se fait vers une pigmentation rési-duelle. Des symptômes sont fréquemment associés au moment des poussées : prurit, sensation de brûlure superficielle, oedème.…”

Mycophenolate mofetil treatment of a patient with hyperglobulinemic purpura

“…1-5 HGPW may occur as primary form or secondary to an associated disorder, most commonly an autoimmune disease. 1,2,[5][6][7][8][9] Previously, multiple therapies including oral corticosteroids, 1,10 antimalarials, 1 colchicine, 1,10 indomethacin, 1,10 azathioprine, 1 chlorambucil, 5 and plasmapheresis 11,12 have had variable results. Mycophenolate mofetil (MMF), an antimetabolite with immunosuppressive properties, has been reported in the treatment of several cutaneous diseases, such as autoimmune blistering disorders, psoriasis, cutaneous Crohn's disease, and connective tissue disorders.…”

“…[1][2][3][4][5]10 Associated symptoms on the affected areas such as burning, stinging, pruritus, and edema have been commonly reported. [2][3][4][5]8,11 Less commonly, other symptoms such as fever, night sweats, generalized lymphadenopathy, malaise, weight loss, myalgias, and arthralgias have been seen. [2][3][4] Alcohol consumption, tight-fitting clothes, heat exposure, standing or walking for prolonged periods of time, and sun exposure have been reported as precipitating factors.…”