Table 1B: Demographic, clinical and histological characteristics of patients with Langerhans cell histiocytosis in vulva

Table 1B: Demographic, clinical and histological characteristics of patients with Langerhans cell histiocytosis in vulva
Author	Age (Years)	Type of skin lesion	Affected site	Inmunohistochemistry exam	Systemic commitment	Treatment	Response	Outcome (months)
Triantafyllidou et al^[1]	52	Papule	Labia minora	NI	No	Local excision	CR	NED (10)
Hwang C et al^[2]	1	Papule	Labia majora	S100 (+) CD1a (+)	No	Topical corticosteroid	NI	NI
Hussein M.R.A ^[3]	8	Papule	Vulva	S100 (+) CD1a (+)	No	NI	NI	NI
Pan Z et al ^[4]	49	Ulcer	Clitoris	S100 (+) CD1a (+) HMB45 (-)	No	RT	CR	NED (5)
Bongiorno M et al^[5]	32	Ulcer	Vulva	S100 (+) CD1a (+)	Osseous/Pituitary/Lungs	CT(Vinblastin) + PREDNIS	CR	NED (22)
Tran D.T et al^[6]	19	Papule	Vulva	S100 (-) CD1a (-) CD68 (+)	Osseous/Pituitary	Clobetasol + CT(Vincristin/cytosine-arabinoside)+PREDNIS + MTX	NI	NI
Beneder C. et al^[7]	49	Papule	Vulva	S100 (+) CD1a (+)	No	Local excision + RT	CR	NED (51)
Dewan M et al^[8]	35	Ulcer	Vulva	S100 (+) Birbeck granules (+)	No	Local excision	NI	NI
Liu Y.H et al^[9]	32	Ulcer	Labia majora	S 100(+) CD1a (+) CD68(+)	Osseous/Pituitary/Gingiva	CT (Ciclophosphamide + etoposide) + PREDNIS	NI	NI
Mottl H et al^[10]	16	Vesicle	Vulva	S100 (+) CD1a (+)	No	Local excision + CT (Vinblastin) + PREDNIS + Cladribine	CR	NED (18)
Mlyncek M et al^[11]	63	Ulcer	Vulva	S100 (+) CD1a (+)	No	Radical vulvectomy	CR	NED (12)
Venizelos I.D et al^[12]	64	Ulcer	Labia minora	S100 (+) CD1a (+) Vimentin (+)	No	Partial Vulvectomy + RT	CR	NED (22)
Ishigaki H et al^[13]	65	Ulcer	Vulva	S100 (+) CD1a (+), Birbeck granules	No	Local excision	CR	NED (12)
Padula A et al ^[14]	31	Nodules/ulcer	Labia minora	S-100 (+) CD1A (+)	No	Radical Vulvectomy + RT	CR	NED (19)
	52	Ulcer	Clitoris	S-100 (+) CD1A (+)	Osseous	RT + CT (Vinblastin + 6 mercaptopurine)	NI	NI
Powell J.L^[15]	2	NI	NI	NI	Osseous/Bone marrow/Spleen	Local excision + CT(Vinblastin)	CR	NED (180)
Dietrich J.E et al^[16]	41	Ulcer	Vulva	NI	Pituitary	Local excision + RT + MTX	NI	NI
	29	NI	Vulva	NI	No	Radical vulvectomy +RT+ PREDNIS + Clobetasol cream 0.05%	NR	REC (NI)
Santillan A et al^[17]	33	Nodules	Vulva	S100 (+) CD1a (+) HMB45 (-)	No	Radical Vulvectomy + RT + Thalidomide	CR	NED(12)
Ruiz A. M.A et al^[18]	49	Nodules	Vulva	S-100 (+) CD1a	No	Thalidomide + clobetasol	NR?	NI
Rizvi R.M et al^[19]	41	Ulcer	Labia minora	S-100 (+), CD-68 (+)	No	Simple vulvectomy	CR	NED (1.5)
Solano T et al^[20]	40	Ulcer	Vulva	S-100 (+), CD1a (+)	No	Local excision + QT (Vincristina)	CR	NED (18)
Prignano F et al^[21]	64	Ulcer	Vulva	CD1a (+) Birbeck granules	Pituitary	CT (Vinblastin)	NI	NI
Khawali C. et al^[22]	31	Ulcer	Vulva	NI	Pituitary/hepatic	CT + hormonal replacement (desmopressin + levothyroxine)	NI	NI
Savell V. et al^[23]	76	NI	Vulva	NI	No	CT (Vinblastin + Vincristin)	CR	NED (9)
Takata M. et al^[24]	62	Ulcer	Vulva	CD1a, CD4 y DLA-DR (+) S100(+), Vimentin (+), Birbeck granules	Osseous	Local excision + RT	NI	NI
Voelklein K. et al^[25]	36	Ulcer	Vulva	S100 (+)	No	RT	NI	NI
Wong K.K et al^[26]	13	Ulcer	Vulva	S-100 (+)	Osseous/ Pituitary	CT (Vincristine) + PREDNIS	NI	REC (NI)
Axiotis C. A. et al^[27]	85	NI	Vulva	NI	No	Topical corticosteroid	NR	REC (NI)
Sang Y.H ^[28]	26	Ulcer	Labia majora	NI	Pituitary	RT	CR	REC (6)
Otis C.N et al^[29]	2	Ulcer	Vulva	S100 (+)	Osseous	CT (Vinblastin)	NR	REC (NI)
NED: No evidence of disease, NI: No information; NR: No response; MTX: methotrexate, REC: Recurrent; CR: complete response CT: Chemotherapy; PREDNIS: Prednisolone; RT: Radiotherapy; MTX: Methotrexate

^{^[1]}Triantafyllidou O., Giannakopoulos K., Pergialiotis V., Simou M., Lagkadas A., Alexandrou P. Pure vulvar Langerhans cell histiocytosis: A case report and literature review. European Journal of Gynaecological Oncology 2009 30:6 (691-694).

^{^[2]} Hwang C, Kim YJ, Seo YJ, Park JK, Lee JH, Lee Y. Isolated Langerhans Cell Histiocytosis of the Vulva in an Infant. PediatrDermatol 2009(6):751

^{^[3]} Hussein MRA. Skin-limited Langerhans' cell histiocytosis in children. Cancer Invest 2009 06;27(5):504-511

^{^[4]} Pan Z, Sharma S, Sharma P. Primary Langerhans cell histiocytosis of the vulva: Report of a case and brief review of the literature. Indian Journal of Pathology and Microbiology 2009(1)

^{^[5]}Bongiorno MR, Pistone G, de Giorgi V, Aricò M. Clinical and immunohistochemical evaluation of the vulvar Langerhans cell histiocytosis. Dermatologic Therapy 2008 11/02;21:S15-S20

^{^[6]} Tran DT, Wolgamot GM, Olerud J, Hurst S, Argenyi Z. An 'eruptive' variant of juvenile xanthogranuloma associated with langerhans cell histiocytosis. J CutanPathol 2008 10;35 Suppl 1:50-54

^{^[7]}Beneder C, Kuhn A, ImObersteg J, Beer K, Fleischmann A, Mueller MD. Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature. Gynecological Surgery 2008(2):165

^{^[8]}Dewan M, Al-Ghamdi A, Zahrani MB. Lessons to be learned--Langerhans' cell histiocytosis. J R SocPromot Health 2008 01;128(1):41-46

^{^[9]} Liu, Y.H, Fang K, Fan XH. Langerhans' cell histiocytosis with multisystem involvement in an adult. ClinExpDermatol 2007 / 11 / 01 /;32(6):765-768

^{^[10]}Mottl H, Rob L, Stary J, Kodet R, Drahokoupilova E. Langerhans cell histiocytosis of vulva in adolescent. International Journal of Gynecological Cancer 2007(2):520

^{^[11]}Mlynček M, Uharček P, Durčanský D. Vulvar Langerhans’ cell histiocytosis: a case report. ActaObstetGynecolScand 2006 06/01; 2018/09;85(6):753-755

^{^[12]} Venizelos ID, Mandala E, Tatsiou ZA, Acholos V, Goutzioulis M. Primary langerhans cell histiocytosis of the vulva. Int J GynecolPathol 2006 01;25(1):48-51

^{^[13]}Ishigaki H, Hatta N, Yamada M, Orito H, Takehara K. Localised vulva Langerhans cell histiocytosis. Eur J Dermatol. 2004 Nov-Dec;14(6) 412-414.

^{^[14]}Padula A, Medeiros LJ, Silva EG, Deavers MT. Isolated vulvar Langerhans cell histiocytosis: report of two cases. Int J GynecolPathol 2004 07;23(3):278-283

^{^[15]} Powell, J.L., Otis CN, Ramirez, P.T. Commenting on "vulvar langerhans cell histocytosis: A case report and review of the literature" by Santillan et al. 91:241-246 [2] (multiple letters). GynecolOncol 2004 / 01 / 01 /;93(3):719

^{^[16]} Dietrich JE, Edwards C, Laucirica R, Kaufman RH. Langerhans cell histiocytosis of the vulva: two case reports. J Low Genit Tract Dis 2004 04;8(2):147-149

^{^[17]}Santillan A, Montero AJ, Kavanagh JJ, Liu J, Ramirez PT. Vulvar Langerhans cell histiocytosis: a case report and review of the literature. GynecolOncol 2003 10;91(1):241-246

^{^[18]} Ruiz Beguerie, J. Fernández, J. Stringa MF, Anaya, J. Vulvar Langerhans cell histiocytosis and thalidomide: an effective treatment option. Int J Dermatol 2017 / 03 / 01 /;56(3):324-326

^{^[19]} Rizvi R.M., Nasreen C., Jafri N. Histiocytosis X of the vulva. JPMA. The Journal of the Pakistan Medical Association 2002 52:9 (430)

^{^[20]} Solano T, España A, Sola J., López G. Langerhans' cell histiocytosis on the vulva. Gynecologic Oncology 2000 78:2 (251-254)

^{^[21]}Prignano F, Domenici L, Carli P, Pimpinelli N, Romagnoli P. Langerhans cell histiocytosis of the vulva: an ultrastructural study. UltrastructPathol 1999 03/19;23(2):127-132

^{^[22]}Khawali C., Silva M.R.D., Faiçal S. Hypothalamic Diabetes Insipidus and panhypopituitarism due to Langerhans cell histiocytosis in an adult. Endocrinologist 1999 9:1 (61-63)

^{^[23]}Savell V, Hanna R, Benda JA, Argenyi ZB. Histiocytosis X of the vulva with a confusing clinical and pathologic presentation. A case report. J Reprod Med. 1995 Apr;40(4) 323-326.

^{^[24]}Takata M, Taniguchi A, Imai T, Hirone T, Nonomura A, Fukui Y. An Adult Case of Histiocytosis X with a Vulvar Ulcer and Multiple Bone Lesions. The Journal of Dermatology 1994 04/01; 2019/03;21(4):259-263

^{^[25]}Voelklein K., Horny H. Prymary Langerhans Cell Histiocytosis of the vulva. Gynecol. Obstet. Invest. 1993; 36(3): 189-90.

^{^[26]} Wong K.K., Lin H.P., Loo L.M. Histiocytosis X and vulvar ulceration International Journal of Gynecology and Obstetrics 1992 39:2 (131-134)

^{^[27]}Axiotis C., Merino M., Dura y P. Langerhans cell Histiocytosis of the female genital trae t. Cancer. 1991; 67: 1650-1660.

^{^[28]} Sang Y.H., Choi I.C., Jun J.B., Kim D.W., and Chung S.L. Histiocytosis-X with chronic weeping ulcers in the anogenital areas. Annals of Dermatology 1990 2:2 (128-131)

^{^[29]} Otis C.N., Fischer R.A., Johnson N., Kelleher J.F., Powell J.L. Histiocytosis X of the vulva: A case report and review of the literature Obstetrics and Gynecology 1990 75:3 II (555-558)