Coagulation activation in autoimmune bullous diseases

Area of Science:

• Dermatology
• Hematology
• Immunology

Background:

• Autoimmune blistering skin disorders, pemphigus vulgaris (PV) and bullous pemphigoid (BP), differ in inflammatory intensity.
• Inflammation can activate coagulation pathways in various diseases.
• Understanding coagulation activation in BP and PV is crucial for assessing thrombotic risk.

Purpose of the Study:

• To evaluate local and systemic coagulation activation in patients with bullous pemphigoid and pemphigus vulgaris.
• To compare coagulation markers and tissue factor presence in active and remittent stages of BP and PV.
• To determine if coagulation activation correlates with disease activity and thrombotic risk in these autoimmune conditions.

Main Methods:

• Studied 20 BP patients, 23 PV patients, and 10 healthy controls.
• Measured plasma prothrombin fragment F1+2 and D-dimer using enzyme-immunoassays.
• Assessed tissue factor (TF) presence immunohistochemically in skin biopsies.

Main Results:

• Significantly elevated plasma F1+2 and D-dimer levels were observed in active BP, but not in active PV.
• Coagulation marker levels normalized during remission for both BP and PV.
• Tissue factor immunoreactivity was present in active BP skin but absent in active PV and control skin, with higher scores in active BP.

Conclusions:

• Bullous pemphigoid is associated with systemic coagulation activation, unlike pemphigus vulgaris.
• The presence of tissue factor in active BP skin suggests a role in disease pathogenesis and potential thrombotic risk.
• These findings indicate an increased thrombotic risk in BP patients compared to PV patients, supported by clinical observations of thrombotic complications.