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Autopsy and Case Reports
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Perianal Langerhans cell histiocytosis: a rare presentation in an adult male

Asmaa Gaber Abdou; Doha MaherTaie

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Langerhans cell histiocytosis (LCH) is a rare disease characterized by a proliferation of cells that show immunophenotypic and ultrastructural similarities with antigen-presenting Langerhans cells of mucosal sites and skin. LCH in adults is rare, and there are still many undiagnosed/misdiagnosed patients. We describe LCH involvement of the perianal region of a 33-year-old male with a previous history of diabetes insipidus. The differential diagnosis and all the reported cases of LCH of the perianal skin involvement since its description in 1984 till 2016 are discussed. LCH should be considered in the differential diagnosis of perianal ulceration, especially in young patients where topical drug treatment has failed. The history of previous central diabetes insipidus of unknown etiology demands imaging studies in order to rule out central involvement of the disease.


Histiocytosis, Langerhans-Cell, Adult, Skin Diseases


Harmon CM, Brown N. Langerhans cell histiocytosis: a clinicopathologic review and molecular pathogenetic update. Arch Pathol Lab Med. 2015;139(10):1211-4. [PMID:26414464] [].

Weitzman S, Egeler RM. Histiocytic disorders of children and adults: introduction to the problem, overview, historical perspective and epidemiology. In: Weitzman S, Egeler RM, editors. Histiocytic disorders of children and adults. Cambridge: Cambridge University Press 2005. p. 1-13.

Donadieu J, Egeler RM, Pritchard J. Langerhans cell histiocytosis: a clinical update. In: Weitzman S, Egeler RM, editors. Histiocytic disorders of children and adults. Cambridge: Cambridge University Press; 2005. p. 95-129.

Ronceray L, Potschger U, Janka G, Gadner H, Minkov M. Pulmonary involvement in pediatric-onset multisystem Langerhans cell histiocytosis: effect on course and outcome. J Pediatr. 2012;161(1):129-33, 3. [PMID:22284564] [].

Murakami I, Matsushita M, Iwasaki T, et al. Interleukin-1 loop model for pathogenesis of Langerhans cell histiocytosis. Cell Commun Signal. 2015;13(1):13. [PMID:25889448] [].

Shakoei S, Shahidi-Dadras M, Saeedi M, Ayatollahi A. Langerhans cell histiocytosis: an uncommon presentation, successfully treated by thalidomide. Indian J Dermatol Venereol Leprol. 2011;77(5):587-90. [PMID:21860158] [].

Liu YH, Fan XH, Fang K. Langerhans’ cell histiocytosis with multisysteminvolvement in an adult. Clin Exp Dermatol. 2007;32(6):765-8. [PMID:17953644] [].

Cavender PA, Bennett RG. Perianal eosinophilic granuloma resemblingcondylomalatum. Pediatr Dermatol. 1988;5(1):50-5. [PMID:3380762] [].

Bank A, Christensen C. Unusual manifestation of Langerhans’ cell histiocytosis. Acta Med Scand. 1988;223(5):479-80. [PMID:3259788] [].

Moroz SP, Schroeder M, Trevenen CL, Cross H. Systemic histiocytosis: an unusual cause of perianal disease in a child. J Pediatr Gastroenterol Nutr. 1984;3(2):309-11. [PMID:6707852] [].

Kader HA, Ruchelli E, Maller ES. Langerhans’ cell histiocytosis with stool retention caused by a perianal mass. J Pediatr Gastroenterol Nutr. 1998;26(2):226-8. [PMID:9481644] [].

Foster A, Epanoimeritakis M, Moorehead J. Langerhans cell histiocytosis of the perianal region. Ulster Med J. 2003;72(1):50-1. [PMID:12868705]

Usmani GN, Westra SJ, Younes SA. 14 month-old boy with hepatomegaly, perianal lesions, and a bony lump on the forehead. N Engl J Med. 2003;348(17):1692-701. [PMID:12711745] [].

Sabri M, Davie J, Orlando S, Di Lorenzo C, Ranganathan S. Gastrointestinal presentation of Langerhans cell histiocytosis in a child with perianal skin tags: a case report. J Pediatr Gastroenterol Nutr. 2004;39(5):564-6. [PMID:15572902] [].

Field M, Inston N, Muzaffar S, Cruickshank N. Perianal Langerhans cell histiocytosis. Int J Colorectal Dis. 2007;22(9):1141-2. [PMID:16703317] [].

Mango JCC, D’Almedia DG, Magalhaes JP, et al. Perianal Langerhans cell histiocytosis case report and review of literature. Rev Bras Coloproctol. 2007;27:1.

Oguzkurt P, Sarialioglu F, Ezer SS, Ince E, Kayaselcuk F, Hicsonmez A. An uncommon presenting sign of Langerhans cell histiocytosis: focal perianal lesions without systemic involvement. J Pediatr Hematol Oncol. 2008;30(12):915-6. [PMID:19131779] [].

Mittal T, Davis MD, Lundell RB. Perianal Langerhans cell histiocytosis relieved by surgical excision. Br J Dermatol. 2009;160(1):213-5. [PMID:19067696] [].

Akbayram S, Akgun C, Ozen S, et al. A case of Langerhans cell histiocytosis with anal fistula. Kurume Med J. 2009;56(3-4):79-83. [PMID:20505285] [].

Tinsa F, Brini I, Kharfi M, Mrad K, Boussetta K, Bousnina S. Perianal presentation of Langerhans cell histiocytosis in children. Gastroenterol Clin Biol. 2010;34(1):95-7. [PMID:19864102] [].

Kanik A, Elacik K, Yesiloglu S, Turker M, Helvaci M, Ozer E. An unusual presentation of Langerhans cell histiocytosis: perianal lesion. Paediatrics Today. 2014;10(1):43-6. [].

Dere Y, Şahin C, Çelik SY, İlhan Çelik Ö, Kara A, Doğan G. Perianal presentation of Langerhans cell histiocytosis. Zhonghua Pifuke Yixue Zazhi. 2016;2(2):99-101. [].

Marchand I, Barkaoui MA, Garel C, Polak M, Donadieu J. Writing Committee. Central diabetes insipidus as the inaugural manifestation of Langerhans cell histiocytosis: natural history and medical evaluation of 26 children and adolescents. J Clin Endocrinol Metab. 2011;96(9):E1352-60. [PMID:21752883] [].

Zerbini MC, Sotto MN, Campos FP, et al. Indeterminate cell histiocytosis successfully treated with phototherapy. Autops Case Rep. 2016;6(2):33-8. [PMID:27547741] [].








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