A Rare Case of Vulvar Langerhans Cell Histiocytosis and Review of the Literature


Balci D. D., KUNDAKCI N., Gurel M. S., Yaldiz M., Dogramaci C. A., AKAY B. N.

TURK DERMATOLOJI DERGISI-TURKISH JOURNAL OF DERMATOLOGY, cilt.3, sa.4, ss.97-103, 2009 (ESCI) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 3 Sayı: 4
  • Basım Tarihi: 2009
  • Dergi Adı: TURK DERMATOLOJI DERGISI-TURKISH JOURNAL OF DERMATOLOGY
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus
  • Sayfa Sayıları: ss.97-103
  • Anahtar Kelimeler: Langerhans cell histiocytosis, vulva, ulcer
  • Ankara Üniversitesi Adresli: Evet

Özet

Langerhans cell histiocytosis (LCH) of the vulva is rare. A 32 year-old woman presented with a two year history of non-healing vulvar ulceration and a nine month history of ulcerative nodular lesion in the left inguinal region. Biopsy results of the vulva and inguinal lesions were consistent with LCH. Screening of the body systems revealed hepatosplenomegaly, iliac, inguinal, paraaortic lymph nodes and lung involvement. She was treated with systemic steroid, resulting in complete improvement of vulvar lesion. She also underwent a local excision of left inguinal lesion. Three months later, we found a lesion on her left inguinal region that was consistent with a recurrence and enlargement in the lymph nodes. Therefore, high dose systemic steroid therapy and then a 12-session radyotherapy were given. After the treatment, a complete improvement was obtained. A 1-year of systemic methotrexate therapy was recommended to prevent recurrence of the disease. In women with vulvar ulcer, LCH should be considered in the differential diagnosis.