Beş aylık bebekte deri tutulumu ile saptanan langerhans hücreli histiyositoz: olgu sunumu

Langerhans hücreli histiyositoz, histiyositlerden köken alan ve patogenezitam olarak bilinmeyen, aktive Langerhans hücrelerinin klonal çoğalması ilekarakterize, nadir görülen bir hastalıktır. Çok farklı klinik prezentasyonlarlakarşımıza çıkan bu hastalık, çoğunlukla çocukluk yaş grubunda görülür. Ensık kemik, kemik iliği ve ikinci sıklıkla da deri tutulumu gösteren langerhanshücreli histiyositoz, tutulum yerleri ve klinik prezentasyonlarına göre dörtalt gruba ayrılır; Letterer-Siwe hastalığı, Hand-Schüller-Christian hastalığı,eozinofilik granülom ve konjenital Self-Healing Retikulohistiyositoz. Kesintanı langerhans hücresinin elektron mikroskopik olarak gösterilmesi veyaimmünohistokimyasal olarak CD1a yüzey antijeninin gösterilmesi ile konur.Bu yazıda seboreik dermatit tanısı ile uzun dönem tedavi almış birlangerhans hücreli histiyositoz olgusu sunulmuştur.

Langerhans cell histiocytosis manifested with cutaneous findings, in a five-months-old baby: case report

Langerhans cell histiocytosis is a rare disease, characterized by a clonal proliferation of activated Langerhans cells. The disease has many different clinical presentations and occurs mostly in childhood. Skin involvement is the second most common involvement of langerhans cell histiocytosis after the bone and bone marrow. The disease has four subgroups according to the clinical manifestation and organ involvement; Letterer- Siwe, Hand-Schüller-Christia, eosinophilic granuloma and congenital self- healing langerhans cell histiocytosis. Accurate diagnosis is made by showing langerhans cells by electron microscopy or by detecting CD1a surface antigen by immunohistochemistry. We describe a case of langerhans cell histiocytosis that were falsely diagnosed and treated as seborrheic dermatititis.

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