Erdheim Chester disease: A case with cutaneous involvement

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Erdheim Chester disease: A case with cutaneous involvement [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2025; 59(1): 22-24 | DOI: 10.4274/turkderm.galenos.2024.34682

Erdheim Chester disease: A case with cutaneous involvement

Sera Nur Y�cesoy¹, G�ll� Gencebay², Ay�e Mine �nenerk³, Tumay Ak⁴, Yusuf Demir¹, �pek Sertbudak³, �vg� Ayd�n �lgen³, Zekayi Kutlubay¹
¹�stanbul University-Cerrahpa�a, Cerrahpa�a Faculty of Medicine, Department of Dermatology, �stanbul, T�rkiye
²Bezmialem University Faculty of Medicine, Department of Dermatology, �stanbul, T�rkiye
³�stanbul University-Cerrahpa�a, Cerrahpa�a Faculty of Medicine, Department of Pathology, �stanbul, T�rkiye
⁴�stanbul University-Cerrahpa�a, Cerrahpa�a Faculty of Medicine, Department of Internal Medicine, �stanbul, T�rkiye

Erdheim-Chester disease (ECD) is a rare multisystem disease characterized by the proliferation of non-Langerhans histiocytes. It is characterized by excessive production and accumulation of histiocytes in multiple tissues and organs. Sites of involvement may include long bones, skin, eyes, lungs, brain, pituitary gland, and additional tissues and organs. The disease course varies depending on the organ and degree of involvement. In this case report, we presented the case of a 54-year-old female patient diagnosed with ECD, manifesting with squamous, enduring plaque lesions on the skin.

Keywords: Erdheim Chester disease, skin, immunosuppressant, BRAF

Erdheim Chester hastal��: Deri tutulumu olan bir olgu

Sera Nur Y�cesoy¹, G�ll� Gencebay², Ay�e Mine �nenerk³, Tumay Ak⁴, Yusuf Demir¹, �pek Sertbudak³, �vg� Ayd�n �lgen³, Zekayi Kutlubay¹
¹�stanbul �niversitesi-Cerrahpa�a, Cerrahpa�a T�p Fak�ltesi Deri ve Z�hrevi Hastal�klar Anabilim Dal�
²Bezmialem Vak�f �niversitesi, Deri ve Z�hrevi Hastal�klar Anabilim Dal�
³�stanbul �niversitesi-Cerrahpa�a, Cerrahpa�a T�p Fak�ltesi Patoloji Anabilim Dal�
⁴�stanbul �niversitesi-Cerrahpa�a, Cerrahpa�a T�p Fak�ltesi Dahiliye Anabilim Dal�

Erdheim-Chester hastal�� (ECH) Langerhans h�cre d�� histositlerin proliferasyonu ile seyreden nadir bir multisistem hastal��d�r. Birden fazla doku ve organda a��r� histiyosit �retimi ve birikimi ile karakterizedir. Tutulum b�lgeleri aras�nda uzun kemikler, deri, g�z, akci�erler, beyin, hipofiz bezi ve/veya ek doku ve organlar yer alabilir. Hastal�k seyri organ tutulumu ve tutulum derecesine ba�l� olarak de�i�kenlik g�sterir. Bu olgu raporunda, deride skuam�z end�re plak lezyonlarla seyreden ECH tan�s� konmu� 54 ya��nda bir kad�n olguyu ele ald�k.

Anahtar Kelimeler: Erdheim Chester hastal��, deri, imm�nos�presan, BRAF

Sera Nur Y�cesoy, G�ll� Gencebay, Ay�e Mine �nenerk, Tumay Ak, Yusuf Demir, �pek Sertbudak, �vg� Ayd�n �lgen, Zekayi Kutlubay. Erdheim Chester disease: A case with cutaneous involvement. Turkderm-Turk Arch Dermatol Venereol. 2025; 59(1): 22-24

Corresponding Author: Sera Nur Y�cesoy, T�rkiye

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