Hypergammaglobulinemic purpura of Waldenstrom - Skin
Hypergammaglobulinemic purpura can be primary (idiopathic) or secondary to an underlying disease. Associated diseases include autoimmune connective tissue disorders, most frequently Sjögren's syndrome and occasionally systemic lupus erythematosus or rheumatoid arthritis, and rarely hematologic malignancies, such as monoclonal gammopathy, lymphoma, or multiple myeloma. The onset of hypergammaglobulinemic purpura often precedes the underlying disease by many years with some associated diseases developing 10 or more years later. The presence of anti-Ro/SS-A and anti-La/SS-B antibodies may predict a higher likelihood of developing an associated autoimmune connective tissue disorder.
D89.0 – Polyclonal hypergammaglobulinemia
190809001 – Waldenstrom hypergammaglobulinemic purpura
- Henoch-Schönlein purpura (HSP) – most commonly affects children with a characteristic clinical tetrad of palpable purpura, abdominal pain, arthritis, and hematuria and typical histopathologic findings of leukocytoclastic vasculitis with IgA deposition in blood vessel walls.
- Cutaneous small vessel vasculitis – classic presentation is palpable purpura, systemic symptoms (especially gastrointestinal, renal, respiratory or joint) and laboratory abnormalities relating to underlying causes such as autoimmune disorders, drugs, infection, or malignancy, and typical histopathologic findings of leukocytoclastic vasculitis.
- Pigmented purpuric dermatoses – purpura more copper-brown in color ("cayenne pepper" appearance), males more frequently affected, and no associated underlying disease.
- Purpuric contact dermatitis – rare disorder resulting from allergy to textile dyes and resins; patch testing will reveal responsible allergens.
- Mycosis fungoides – can appear as pigmented purpura-like eruption with typical histopathologic findings including epidermotropism of atypical lymphocytes, Pautrier's microabscesses, and presence of clonal T-cell receptor gene rearrangement.
- Idiopathic thrombocytopenic purpura (ITP) – more petechiae and purpura tend to be smaller in size with characteristic history of bleeding and low platelet count in setting of normal bone marrow and absence of disease or medication associated with thrombocytopenia.
- Thrombotic thrombocytopenic purpura (TTP) – more petechiae and purpura tend to be smaller in size with characteristic clinical pentad of thrombocytopenia, microangiopathic hemolytic anemia (presence of schistocytes on peripheral blood smear), renal failure, neurologic deficits, and fever.
- Scurvy – characterized by dietary history of inadequate vitamin C intake, perifollicular purpura, corkscrew hairs, spongy bleeding gums, specific radiologic features relating to vitamin C deficiency, and low fasting ascorbic acid levels; more frequently seen in underdeveloped countries.