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

Table 1A: Demographic, clinical and histological characteristics of patients with Langerhans cell histiocytosis in vulva
Author	Age (Years)	Type of skin lesion	Affected site	Immunohistochemistry exam	Systemic commitment	Treatment	Response	Outcome (months)
Shah PR et al ^[1]	54	Papule	Labia majora	S100 (+) CD1a (+)	No	Thalidomide + Tacrolimus + MTX	NI	NI
Jorgensen EM et al ^[2]	8	Ulcer	Vulva	S100 (+) CD1a (+)	Osseous	CT(Vinblastin) + PREDNIS	CR	NED (12)
Wieland R et al ^[3]	26	Papule	Labia minora	S100 (+) CD1a (+)	No	Local excision	CR	NED (23)
	67	Vesicle	Labia majora	CD1a (+)	No	Local excision + hydrocortisone cream	CR	NED (130)
	31	Papule	Cervix	NI	No	Hysterectomy	CR	NED (54)
Nic DE et al ^[4]	16	Ulcer	Vulva	S100 (+) CD1a (+)	Pituitary	CT(Cytarabine) + PREDNIS	NI	NI
Ruiz BJ et al ^[5]	56	Plaque	Vulva	S100 (+) CD1a (+)	No	Thalidomide + Clobetazol	CR	NED (2)
Zudaire T et al ^[6]	59	Plaque/ Ulcer	Labia minora	S100 (+) CD1a (+)	Osseous/ Skin	CT(Cytarabine)	NI	NI
Baser S et al ^[7]	3	Plaque	Vulva	NI	Osseous/Liver	CT(Vinblastine) + PREDNIS	CR	NED (8)
Gordon MS et al ^[8]	22	NI	Vulva	S100(+) CD1a (+) CD68(+)	Pituitary	PREDNIS	NI	NI
Martínez CD et al ^[9]	26	Ulcer	Vulva	S100(+) CD1a (+) CD68 (+) Vimentin (+)	Lung	Local excision + topical corticosteroid	CR	NED (96)
Ikeya S et al ^[10]	20	Nodules	Vulva	S100(+) CD1a (+) Birbeck Granules	Pituitary	Cladribine + PREDNIS +	NI	NI
Pedrosa AFB et al ^[11]	43	Ulcer	Clitoris	S100 (+) CD1a (+)	Skin/ Gingiva	PREDNIS	NI	NI
Rasool S et al ^[12]	76	Plaque	Labia minora/ Clitoris	S 100(+) CD1a (+) CD68(+) Birbeck Granules (+)	Skin	Topical corticosteroid	NI	NI
Sun N et al ^[13]	28	Ulcer	Vulva	S100(+) CD1a (+) Vimentin (+)	No	Interferon + PREDNIS + MTX	CR	NED (18)
Khoummane N et al ^[14]	47	NI	Vulva	S100 (+) CD1a (+)	No	Local excision	NI	NI
Singh M et al ^[15]	50	Ulcer	Vulva	S100 (+) CD1a (+)	No	RT + Thalidomide	NI	NI
Ibrahim I et al ^[16]	33	Nodules	Vulva	NI	No	Radical vulvectomy + RT + Thalidomide + Lenalidomide	NI	REC (NI)
El Fekih N et al ^[17]	20	Ulcer	Vulva	S100 (+) CD1a (+)	Osseous/Pituitary	RT + topical corticosteroid	NI	NI
Ibrahim IF et al ^[18]	43	Nodules	Vulva	NI	No	Local excision + RT + Thalidomide + Lenalidomide	NI	REC (NI)
Chang JC et al^[19]	68	Plaque	Labia majora	S100 (+) CD1a (+)	No	Topical corticosteroid	CR	NED (6)
EL-Safadi S et al^[20]	59	Ulcer	Vulva	S100 (+) CD1a (+)	No	Radical Vulvectomy + RT + MTX +Thalidomide	NI	NED (31)
Jiang W et al^[21]	46	Papule	Labia minora	S100(+) CD1a (+) Vimentin (+)	No	Local excision + CT(Vinblastin) + PREDNIS	CR	NED (40)
	40	Ulcer	Labia majora	S100(+) CD1a (+) Vimentin (+)	No	Local excision + CT(Vinblastin) + PREDNIS	CR	NED (36)
	23	Plaque	Cervix	S100(+) CD1a (+) Vimentin (+)	No	Thalidomide + CT (Vinblastin) + PREDNIS + Hysterectomy	CR	NED (12)
Márques F et al^[22]	1	Condyloma	Perineum	S100 (+) CD1a (+)	No	CT (Vinblastin) + PREDNIS	CR	REC (6)
Fernández L.B et al ^[23]	60	Plaque	Vulva	S100 (+) CD1a (+)	Skin	Dexamethasone + Thalidomide	VR	NED (NI)
Foley S et al ^[24]	62	Nodule	Labiaminora	S100 (+) CD1a (+)	No	Clobetazol propionate	CR	NED (36)
Simons M et al^[25]	33	Condyloma	Labia majora	S100 (+) CD1a (+) Vimentina (+)	No	Local excision + Tacrolimus + RT	CR	REC(11)
Brazeal T et al^[26]	65	Rash	Vulva	S 100(+) CD1a (+) CD68(-)	Skin/ Lungs	NI	NI	NI
NED: No evidence of disease, NI: No information; NR: No response; REC: Recurrent; CT: Chemotherapy; PREDNIS: Prednisolone; RT: Radiotherapy; MTX: Methotrexate

^{^[1]} Shah PR, Pitch MA, Scott G, Mercurio MG. Primary Langerhans Cell Histiocytosis of the Vulva in a Postmenopausal Woman: Response to Treatment with Oral Methotrexate. J Low Genit Tract Dis 2018 04;22(2):169-170

^{^[2]} Jorgensen EM, Chen PP, Rutter S, Cron JA. Vulvar Lesions in an 8-Year-Old Girl: Cutaneous Manifestations of Multisystem Langerhans Cell Histiocytosis. J PediatrAdolescGynecol 2018 04;31(2):153-155

^{^[3]} Wieland R, Flanagan J, Everett E, Mount S. Langerhans cell histiocytosis limited to the female genital tract: A review of literature with three additional cases. GynecolOncol Rep 2017 08/26;22:4-8

^{^[4]}NicDhonncha E, Clowry J, Field S. Persistent Vulval Symptoms in a 16-Year-Old Girl. PediatrDermatol 2017 07;34(4):484-485

^{^[5]} 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

^{^[6]}Zudaire T, Guarch R, Valcayo A, García K, Resano MÁ, Requena D, et al. Primary Langerhans Cell Histiocytosis of the Vulva: Case Report and Review of the Literature. Int J GynecolPathol 2017 03;36(2):111-114, 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)

^{^[7]} Baser S, Kaman A, Zaimoglu I, Gayretli-Aydin Z, Aydin-Teke T, Kaygusuz U, et al. A case of Langerhans cell histiocytosis mimicking child abuse. Turk J Pediatr 2016;58(6):675-678

^{^[8]} Gordon MS, Gordon MB. Occult Langerhans Cell Histiocytosis Presenting with Papillary Thyroid Carcinoma, a Thickened Pituitary Stalk and Diabetes Insipidus. Case Reports in Endocrinology 2016

^{^[9]}Martínez-Castillón D, Sanz-Cardiel A, Gilaberte-Calzada Y, Borderías-Clau L, Vera Álvarez J, Ramón Y Cajal,J.M. Langerhans cell Histiocytosis of the vulva. Rev ClinEsp 2015 01/20;215(1):e5-e7

^{^[10]}Ikeya S, Tokura Y, Yagyu T. Langerhans cell histiocytosis presenting as a nodule beneath the clitoral hood. J Dermatol 2014 / 02 / 01 /;41(2):175-176

^{^[11]}Pedrosa AFB, Lisboa C, Ferreira O, Azevedo F, Bettencourt H. Primary Langerhans cell histiocytosis of the vulva: acitretin as a glucocorticoid-sparing agent. Int J Dermatol 2014(4):294

^{^[12]}Rasool S, Johnston GA, Bamford M. A cutaneous presentation of a rare condition. ClinExpDermatol 2014 03;39(2):248-249

^{^[13]} Sun N, Cao D, Zhao Q, LI W. Langerhans Cell Histiocytosis on the Vulva: A Case Report and Review of the Literature. Reproduction and Contraception 2014(2):123

^{^[14]}Khoummane N, Guimeya C, Lipombi D, Gielen F. Vulvar Langerhans cell histiocytosis: a case report. Pan Afr Med J 2014 06/06;18:119-119

^{^[15]} Singh M, Lewis F, Robson A, Child F. A Case of Vulval Ulceration. JAMA Dermatology 2014 03;150(3):325

^{^[16]} Ibrahim I, Naina H. Successful Treatment of Recurrent Langerhans' Cell Histiocytosis of the Vulva with Lenalidomide. international journal of gynecological cancer 2013;23(8)

^{^[17]} El Fekih N, Kamoun I, Jones M, Remmeh S, Zéglaoui F, Ben Slama C, et al. Histiocytosis X revealed by diabetes insipidus and skin lesions. Am J Dermatopathol 2013 07;35(5):606-608

^{^[18]} Ibrahim IF, Naina HVK. Treatment of recurrent Langerhans cell histiocytosis of the vulva with lenalidomide. JCO 2013 05/20; 2019/03;31(15):e16555-e16555

^{^[19]} Chang JC, Blake DG, Leung BV, Plaza JA. Langerhans cell histiocytosis associated with lichen sclerosus of the vulva: case report and review of the literature. J CutanPathol 2013 02;40(2):279-283

^{^[20]} El-Safadi S, Dreyer T, Oehmke F, Muenstedt K. Management of adult primary vulvar Langerhans cell histiocytosis: review of the literature and a case history. European Journal of Obstetrics and Gynecology 2012(2):123

^{^[21]} Jiang W, Li L, He Y, Yang K. Langerhans cell histiocytosis of the female genital tract: a literature review with additional three case studies in China. ArchGynecolObstet 2012 01;285(1):99-103

^{^[22]} Marques F, Sousa H, Cruz A, Constantino C, Paiva M, Lacerda A, et al. Uncommon Presenting Signs of Langerhans Cell Histocytosis in Children. Actapediátrica 2011;100:55-55

^{^[23]}Fernandes LB, Guerra JG, Costa MB, Paiva IG, Duran FP, Jaco DN. Langerhans cells histiocytosis with vulvar involvement and responding to thalidomide therapy - Case report. An Bras Dermatol 2011;86(4):78-81

^{^[24]} Foley S, Panting K, Bell H, Leonard N, Franks A. Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids. Australas J Dermatol 2011 02;52(1):e8-e14

^{^[25]}Simons M, Nieuwenhof VD, Van DA, Bulten J, De Hullu JA. Case report: A patient with lichen sclerosus, Langerhans cell histiocytosis, and invasive squamous cell carcinoma of the vulva. ObstetGynecol 2010;203:e7-e10

^{^[26]}Brazeal T, DiCaudo D, Weber M, Warschaw K. Multisystem Langerhans cell histiocytosis in an adult presenting with vulvar lesions. J Am AcadDermatol 2010;62(3):AB29-AB29