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Table 1 Cases of perianal Langerhans cell histiocytosis

From: Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature

Author, year Age in years, sex History Perianal lesion morphology Perianal lesion symptoms Perianal lesion treatment Intervala Systemic involvement Systemic treatment Treatment outcome Survival (follow-up time in months)
Abdou et al. 2017 [2] 33 M Polydipsia (4 years), perianal lesions (2 years) Perianal ulcerative plaque with raised edges oozing pus Pain Antibiotics and steroids DI (no brain imaging obtained) Methotrexate
Mansour et al. 2017 [5] 32 M Polydipsia (10 years), Smoking Cutaneous infiltration of anal sphincter, eroded ulcerative plaques over the anal orifice Pain, bleeding and purulent discharge None, only systemic 0 Lungs fibrosis; rectal tumor on colonoscopy, DI (no brain imaging obtained) Vinblastine, prednisone, gemcitabine Anal and colonic lesions, disappeared, lung fibrosis remained stable Yes (6)
Gul et al. 2017 [6] 36 M Anal fissure, two rectal surgeries _ Pain and pressure Local radiotherapy 9 months after perianal lesions Thyroid Vinblastine, prednisone, total thyroidectomy Yes (6)
Bank et al. 1988 [7] 18 M Itchy scalp lesion (2 years) Ulceration with purulent secretions Antibiotics 2 months after perianal lesions Lung fibrosis and emphysema, bilateral (BL) pneumothorax, DI (no brain imaging obtained) Vinblastine, prednisone, topical mustargen, lung subsegmental resection Persistent scalp and perianal lesions Yes (8)
Dere et al. 2016 [8] 45 F Perianal wound (1 year) 3-cm ulcerovegetant purulent mass Pressure sensation Topical steroids 0 Femur and tibial lesions Methotrexate (MTX) Skin lesion healed with MTX Yes (1 M)
Chauffaille et al. 1998 [9] 31 F Polydipsia (3 years), vulvar ulcers (1 year) Granulomatous perianal and vulvar ulceration 0 Femurs, skull, shoulder, forearm lesions, liver, oral lesions, DI, hypothalamic tumor on brain MRI
Mittal et al. 2009 [10] 45 M Perianal ulcerations (4 M) Painful perianal ulcerations Nitrogen mustard, steroids, pentostatin. Then, surgical excision with APR and proctectomy Prior to perianal lesions Bone and lung No recurrence of skin lesion post excision Yes (36)
Foster et al. 2003 [11] 19 M Perianal lesions (2 years), NSGY procedure Two flat sessile perianal lesions on each buttock Surface bleeding Surgical excision 11 years prior perianal lesions Extradural cranial mass invading cranial fossa on head computed tomography (CT) Craniotomy for mass excision. Prednisone, vincristine, mercaptopurine No recurrence of skin lesions and cranial mass Yes (48)
Shahidi et al. 2011 [12] 20 M Perianal wounds (1 year) Well demarcate erythematous plaque Difficulty in defecation Antibiotics and steroids None None Thalidomide Lesion shrunk and became painless Yes (6)
Waters et al. 2015 [13] 32 M Perianal ulcerations (10 years), multiple excisions, and biopsies Obliteration of anal verge by scar tissue Pain and pressure Laparoscopic diverting colostomy None None None
Madnani et al. 2011 [14] 38 F Polydipsia (8 years), vulvar ulcers (5 years) Demarcated, indurated ulcer labia minora extending to the perianal area Antibiotics multiple biopsies 0 Liver, bone, DI, mass in brain ventricle on brain MRI Etoposide, 6-mercaptopurine, prednisolone Bone pain disappeared, and skin lesions healed Yes (120)
Conias et al. 1998 [15] 24 M Scalp scaling and external auditory canal discharge and crusting (4 years) Ulcerated hemorrhagic plaque extending to the scrotum (pain and bleeding) Potassium permanganate, topical steroids, local radiotherapy None None Cladribine Scalp and ear lesions disappeared. Perianal lesions recurred and responded to local radiotherapy Yes (12)
Field et al. 2007 [13] 70 M Perianal pain and discharge (4 M), prostate CA 2-cm ulcerated lesion Rectal bleeding, pain, mucous discharge Steroids, potassium permanganate After the perianal lesions Tibial lesion
Broekaert et al. 2007 [17] 57 F Polydipsia (2 years), inguinal and perianal ulcers (1 year) Ulcerations with red raised border None, only systemic 0 DI, thickened infundibulum on brain MRI Thalidomide Skin lesions healed; cerebral lesion remained stable Yes (24)
Roeb et al. 2012 [18] 69 M Perianal fistula, eczema, abdominal pain (4 years) Perianal pain Steroids 0 Colon (mucosal lesions throughout) Perianal eczema and abdominal pain improved
This patient presentation 50 M Polydipsia (3 years), perianal ulcers and pruritis ani (1 year) Excoriated and erythematous, multiple superficial lacerations Pruritus and pain Silver sulfadiazine, zinc oxide None DI (no masses on brain MRI) None None Yes
  1. –, data not reported; Intervala, interval time between LCH lesions pathological diagnosis and systematic manifestation; APR, abdominal perineal resection; DI, diabetes insipidus; MTX, Methotrexate; NSGY, Neurosurgery; CT, Computed Tomography