Langerhans cell histiocytosis with anorectal involvement: a rare manifestation of adult disease

Gastrointestinal Langerhans cell histiocytosis with unifocal, single‐system involvement in adults: Cases report and literature review

“… 1 Whereas, patients with multi‐system LCH predominantly need chemotherapy. 17 On the other hand, the prognosis of single‐system LCH is better than multi‐system LCH. The data of adult LCH is limited and standardized protocols are sparse, and thus most treatments are based on the pediatric experience.…”

“…Individuals with unifocal, single‐system involvement do not require systemic therapy, other than local excision or observation 1 . Whereas, patients with multi‐system LCH predominantly need chemotherapy 17 . On the other hand, the prognosis of single‐system LCH is better than multi‐system LCH.…”

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

“…Patients were initially treated with antibiotics and corticosteroids, and systemic chemotherapy was started if the lesions were refractory. Three patients had surgical interventions: perianal lesion excision, diverting colostomy due to anal obliteration after a 10-year history of LCH ulcerations, and an abdominal perineal resection (APR) due to refractory perianal lesions [ 10 , 11 , 13 ]. Interestingly, six (40%) patients were in their thirties, two (13%) in their teens, two (13%) in their twenties, two (13%) in their forties, one (6%) in his sixties, and one (6%) in his seventies.…”

“…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.…”

“…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.…”