Langerhans cell histiocytosis of vulva in adolescent

Langerhans cell histiocytosis (LCH) affecting child vulva alone is a very rare disease. Only 13 cases of primary vulvar LCH have been previously reported in the medical literature. We describe an additional case in which the LCH was confined to the vulva, with review of the literature. A 16.5-year-old girl presented with papulous and ulcerative lesions on her labia majora and minora. The biopsy revealed a typical histopathologic finding consistent with LCH. A metastatic work-up did not reveal any evidence of the disease except on the vulva. Treatment was carried out according to LCH II protocol. The patient was diagnosed with a recurrent disorder in the vulva 8 months after the completion of primary chemotherapy. For this reason, she underwent second line treatment with 2-chlorodeoxyadenosine. Eighteen months after the second line chemotherapy, the patient has no signs of a local or systemic recurrence. Primary LCH of vulva is very unusual, but we have to keep in mind this possibility when an adolescent girl presents with an atypical chronic lesion on the vulva. This patient appears to be the first case of adolescent 16.5 year old having a solely cutaneous lesion of the vulva

[1]  J. Starý,et al.  Treatment of recurrent Langerhans cell histiocytosis in children with 2-chlorodeoxyadenosine , 2006, Leukemia & lymphoma.

[2]  R. Kodet.  Outcome of Treatment of Langerhans Cell Histiocytosis according to the LCH II Protocol , 2005 .

[3]  K. Takehara,et al.  Localised vulva Langerhans cell histiocytosis. , 2004, European journal of dermatology : EJD.

[4]  M. Deavers,et al.  Isolated vulvar Langerhans cell histiocytosis: report of two cases. , 2004, International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists.

[5]  K. McClain,et al.  Efficacy of continuous infusion 2‐CDA (cladribine) in pediatric patients with Langerhans cell histiocytosis , 2004, Pediatric blood & cancer.

[6]  P. Ramirez,et al.  Vulvar Langerhans cell histiocytosis: a case report and review of the literature. , 2003, Gynecologic oncology.

[7]  A. Tefferi,et al.  2-Chlorodeoxyadenosine therapy for disseminated Langerhans cell histiocytosis. , 2003, Mayo Clinic proceedings.

[8]  J. Pretto,et al.  Successful treatment of Langerhans cell histiocytosis with 2‐chlorodeoxyadenosine , 2003, Respirology.

[9]  L. M. Lima,et al.  Synthesis and anti-inflammatory activity of phthalimide derivatives, designed as new thalidomide analogues. , 2002, Bioorganic & medicinal chemistry.

[10]  J. Moodley,et al.  Isolated Langerhans Cell Histiocytosis of the Vulva: A Case Report , 2001, The journal of obstetrics and gynaecology research.

[11]  A. Chu,et al.  Langerhans cell histiocytosis of the skin. , 1998, Hematology/oncology clinics of North America.

[12]  J. Malpas.  Langerhans cell histiocytosis in adults. , 1998, Hematology/oncology clinics of North America.

[13]  H. Gadner,et al.  Current therapy for Langerhans cell histiocytosis. , 1998, Hematology/oncology clinics of North America.

[14]  J. Shupack,et al.  Rediscovering thalidomide: a review of its mechanism of action, side effects, and potential uses. , 1996, Journal of the American Academy of Dermatology.

[15]  T. Cerny,et al.  Erfolgreiche Behandlung eines Falls von kutaner Langerhanszell-Granulomatose mit 2-Chlorodesoxyadenosin und Thalidomid , 1995, Der Hautarzt.

[16]  Z. Argényi,et al.  Histiocytosis X of the vulva with a confusing clinical and pathologic presentation. A case report. , 1995, The Journal of reproductive medicine.

[17]  J. Saurat,et al.  [Successful treatment of a case of cutaneous Langerhans cell granulomatosis with 2-chlorodeoxyadenosine and thalidomide]. , 1995, Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete.

[18]  H. Gadner,et al.  Treatment of Langerhans cell histiocytosis--evolution and current approaches. , 1994, The British journal of cancer. Supplement.

[19]  L. Thomas,et al.  Successful treatment of adult's Langerhans cell histiocytosis with thalidomide. Report of two cases and literature review. , 1993, Archives of dermatology.

[20]  J. Blaauwgeers,et al.  Langerhans cell histiocytosis of the vulva. , 1993, European journal of obstetrics, gynecology, and reproductive biology.

[21]  J. Dietl,et al.  Primary Langerhans cell histiocytosis of the vulva. , 1993, Gynecologic and obstetric investigation.

[22]  P. Duray,et al.  Langerhans cell histiocytosis of the female genital tract , 1991, Cancer.

[23]  P. Pizzo,et al.  Principles and Practice of Pediatric Oncology , 1989 .

[24]  R. O’Toole,et al.  Pure cutaneous histiocytosis X of the vulva. , 1984, Obstetrics and gynecology.

[25]  V. Ferrans,et al.  The histiocytoses. , 1983, Pathology annual.

[26]  L. Lichtenstein.  Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schüller-Christian disease as related manifestations of a single nosologic entity. , 1953, A.M.A. archives of pathology.

[27]  Margaret G. Smith,et al.  LXXXIV.—CUTANEOUS MANIFESTATIONS OF CHRONIC (IDIOPATHIC) LIPOIDOSIS (HAND-SCHÜLLER-CHRISTIAN DISEASE): REPORT OF FOUR CASES, INCLUDING AUTOPSY OBSERVATIONS , 1939 .