Purpura hyperglobulinaemica D89.0

Often, an essential gammopathy (MGUS) is present without any signs of underlying disease.

As a result of orthostasis, fresh, non-compressible (glass spatula pressure), fresh red, non-compressible stains appear in batches on dependent areas of skin (especially on the lower legs), even those barely 0.5 cm in size. No itching, no pain. Thus the spots are perceived rather coincidentally. The Rumpel-Leede-sign is positive. Not infrequently, cushion-like oedemas in the ankle area are detected.

In very rare cases a clinically identical clinical picture (recurrent macular vasculitis in hypergammaglobulinemia) can also be accompanied by vasculitic phenomena (Hudson CP et al.1984).

Polyclonal hypergammopathy, possibly anemia, hypalbuminemia. CRP normal; BSG may be accelerated. Platelet count, platelet function and plasmatic coagulation parameters are normal. Thus, no coagulopathies, thrombocytopathies or thrombocytopenia are present.

Non-reactive erythrocyte extravasations in the upper dermis. No signs of leukocytoclastic vasculitis or signs of vascular wall damage as would be typical for CVI.

Because of the clinical picture of progressive pigment purpura, this disease, which is important for differential diagnosis, should be excluded. Polyclonal hypergammopathies also occur in chronic liver diseases

Shear-wise course. This results in a clinical picture that is clinically largely identical to the progressive purpura pigmentosa.

The name "Purpura hyperglobulinaemica" is still a rather uncommon diagnosis for a purpura in MGUS (see also under gammopathy, skin changes)

1. Hudson CP et al (1984) Cutaneous leukocytoclastic vasculitis with hyperglobulinemia and splenomegaly. A variant of hyperglobulinemic purpura of Waldenström. Arch Dermatol 120:1224-1226.
2. Julio A et al (1977) Purpura associated with hypergammaglobulinemia, renal tubular acidosis and osteomalacia.Can Med Assoc J 116:53-58.https://www.ncbi.nlm.nih.gov/pubmed/832220
3. Strauss WG (1959) Purpura hyperglobulinemia of Waldenström; report of a case and review of the literature.N Engl J Med. 1959 Apr 23;260(17):857-60.