OVERVIEW

What is hyperglobulinemic purpura?

Hyperglobulinemic purpura is a rare disease with unclear pathogenesis, but it is associated with excessive production of immunoglobulins.

The condition is characterized by recurrent skin ecchymoses and petechiae with pigmentation, but normal platelet counts.

Hyperglobulinemic purpura predominantly affects women. It is generally a chronic benign condition and not life-threatening, though some cases may be secondary to difficult-to-treat diseases such as multiple myeloma or Sjögren's syndrome.

SYMPTOMS

What are the manifestations of hyperglobulinemic purpura?

The onset is insidious, with small red macules appearing on the skin first, which quickly develop into petechiae and merge into ecchymoses, known as "purpura."

Purpura mainly occurs on the skin of the lower limbs, most severely on the front of the calves and the dorsum of the feet, appearing symmetrically on both sides. There may be mild itching, stinging, or burning sensations occasionally. Purpura often recurs and leaves brown spots, known as "hyperpigmentation," after fading.

Some patients may experience symptoms such as fever, night sweats (excessive sweating during sleep), systemic lymphadenitis with swelling, fatigue, weight loss, and joint or muscle pain.

CAUSES

Why does hyperglobulinemic purpura occur?

The exact pathogenesis is currently unclear. It may be due to a significant increase in plasma globulin caused by various factors, leading to increased vascular fragility and resulting in skin purpura.

What causes hyperglobulinemic purpura?

Acute or chronic infections, malignant tumors, autoimmune diseases, chronic liver diseases, and plasma cell disorders may all lead to hyperglobulinemic purpura. In some cases, no clear cause can be identified.

Underlying these conditions, factors such as alcohol consumption, wearing tight clothing, exposure to excessively hot environments, prolonged standing or walking, and sunlight exposure may trigger purpura episodes.

Is hyperglobulinemic purpura contagious?

No.

DIAGNOSIS

What tests are needed for hyperglobulinemic purpura?

Tests including complete blood count, liver function, coagulation function, erythrocyte sedimentation rate, rheumatoid factor, serum protein electrophoresis, and anti-Ro/SSA antigen antibodies are required.

Patients with hyperglobulinemic purpura may present with:

• Leukopenia and mild anemia;

• Elevated serum globulin;

• Increased erythrocyte sedimentation rate;

• Positive rheumatoid factor;

• Polyclonal increase in γ-globulin;

• Positive anti-Ro/SSA, which may indicate potential progression to Sjögren's syndrome or systemic lupus erythematosus.

What are the skin biopsy findings in hyperglobulinemic purpura patients?

Perivascular lymphocytic inflammation with red blood cell extravasation or leukocytoclastic vasculitis.

What diseases can hyperglobulinemic purpura be easily confused with?

• Macroglobulinemic purpura: Laboratory tests may show increased macroglobulin, decreased coagulation factors, prolonged bleeding time, and positive Bence Jones protein in urine. May be accompanied by Raynaud's syndrome, urticaria, etc.

• Cryoglobulinemic purpura: Purpura appears upon cold exposure, with positive cryoglobulin in blood and accelerated erythrocyte sedimentation rate at room temperature. May be accompanied by cold urticaria, Raynaud's syndrome, livedo reticularis, etc.

TREATMENT

Which department should I visit for hyperglobulinemic purpura?

Hematology.

Does hyperglobulinemic purpura require hospitalization?

Hyperglobulinemic purpura itself can be treated with oral medication and does not require hospitalization. However, if there is a serious underlying condition (such as severe infection, malignant tumor, etc.), hospitalization may be needed to treat the primary disease.

How is hyperglobulinemic purpura treated?

Treatment may include appropriate glucocorticoids, antimalarial drugs, colchicine, indomethacin, azathioprine, chlorambucil, mycophenolate mofetil, and other medications. Plasma exchange may be necessary in some cases. Vitamin E and vitamin C can be used as adjunctive therapy.

What serious consequences can occur if hyperglobulinemic purpura is left untreated?

Hyperglobulinemic purpura can occur alone or secondary to other diseases, which may indicate an underlying serious condition. If ignored and left untreated, it may lead to misdiagnosis, missed diagnosis, or delayed treatment.

Can hyperglobulinemic purpura be cured?

Not necessarily, as it depends on the underlying disease. Hyperglobulinemic purpura can be secondary to various diseases, and the treatment outcomes vary depending on the condition.

Is hyperglobulinemic purpura prone to recurrence?

Hyperglobulinemic purpura may recur repeatedly and leave pigmentation on the skin surface.

DIET & LIFESTYLE

What should patients with hyperglobulinemic purpura pay attention to in daily life?

Hyperglobulinemic purpura tends to recur, so it's important to avoid triggering factors in daily life:

• Quit alcohol;

• Wear loose clothing and avoid tight-fitting garments;

• Use an umbrella when going out, protect the skin from direct sunlight, and avoid exposure to excessively hot environments;

• Avoid prolonged standing or walking.

What dietary precautions should patients with hyperglobulinemic purpura take?

Dietary considerations mainly depend on the underlying disease causing hyperglobulinemic purpura. It is recommended to consult your primary physician.

PREVENTION

Can hyperglobulinemic purpura be prevented?

Since the pathogenesis is unclear, there are currently no preventive methods.