الداء النشواني والجلد
Abstract
يصنف الداء النشواني الجهازي إلى بدئي، ثانوي، وعائلي. ويكون الداء النشواني الجهازي البدئي AL ، إما بدئياً، أو مرافقاً للورم النقوي العديد. وتتألف فيه المادة النشوانية من أضداد مناعية خفيفة السلسلة Light- chain. 1,2 وحسب ترسب هذه المادة على أنسجة الجسم تحدث الأذية الوظيفية، وأكثر الأجهزة إصابةً الكلية، القلب، الأعصاب المحيطية، الجهاز العصبي اللاإرادي والرئة.3
إن التظاهرات الجلدية موجودة في الداء النشواني الجهازي البدئي، ويعتقد ان المادة النشوانية تترسب على الجلد حتى في غياب العلامات الجلدية، وهي غالباً متعلقة بالنزوف الجلدية وهي حبرية، أو كدمية، التي تنتج عن المادة النشوانية في جدران الأوعية الجلدية، أما باقي العلامات الجلدية فهي قليلة الشيوع، وهنا تكمن أهمية تسجيل حالتين نقدمهما في هذه الدراسة المصغرة عن العلامات الجلدية في الداء النشواني الجهازي البدئي AL.4,5
Systemic Amyloidosis is classified into primary, secondary and familial. Primary systemic amyloidosis (AL-Amyloidosis) may be idiopathic or myeloma-associated. It is the amyloidosis composed of immunoglobulin light chains. It involves kidney, heart, liver, peripheral nerves, autonomic nervous system and sometimes lungs. Skin involvement may be seen in AL Amyloidosis. Cutaneous manifestation depends upon the site of amyloid deposition. Skin involves other than those related to intracutaneous haemorrhage manifesting in the form of petechiae, purpura and ecchymoses due to infiltration of blood vessel walls by amyloid deposits that is not very common. Here we are presenting two cases of AL amyloidosis with atypical skin involvement.
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