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1. Perianal Langerhans cell histiocytosis
Dr Maha Assem Fahmy
Dermatology specialist. Mediclinic Al-Ain UAE
Master of dermatology and andrology
Ain-shams university
Diploma of laser and its application in dermatology
NILES, Cairo university
Board certification in aesthetic medicine AAAM

2. Statement of ethics:
 This presentation was prepared in compliance
with all ethical and confidentiality guidelines
and principles.
 Patient consent for biopsy and photography
was obtained at the time of care.

3. Medical History:
4-yrs-old male child
presented with perianal
lesions 3m duration
progressive course.

4. DD:
 Condyloma accuminata
 Condyloma lata
Child sexual abuse????

5. Biopsy was performed for
medico-legal issues

6. H/P: (200x,400x)
Dermis was infiltrated by sheets of dyscohesive round cells with reniform nuclei
and nuclear in-folding. Also the infiltrate showed eosinophils, lymphocytes, and
plasma cells

7. Pan- CK (-ve):

8. CD1 a, S100 protein

9. Langerhans cell
Histiocytosis

10.  LCH is a rare disease of unknown etiology caused
by clonal proliferation of Langerhans cells.
 LCH its childhood incidence is1/200,000 the
incidence in adults is extremely rare 1/2 million.
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;30:915e6.
Y. Dere et al. Dermatologica Sinica 34 (2016) 99e101

11.  LCH may present with solitary lesions in the
bone or visceral organs, in addition to the
involvement of multiple organ systems.
Tinsa F, Brini I, Kharfi M, Mrad K, Boussetta K, Bousnina S. Perianal presentation of Langerhans cell histiocytosis in
children. Gastroenterol Clin Biol 2010;34:95e7.

12. Imaging and blood work up were done
to roll out systemic involvement
All results were normal

13. Treatment:
Imiqumode 5% was used for 2 weeks
results in complete cure of the lesions.

14.  LCH involving the perianal skin
is rare.
 Searching the Literature (17)
cases were reported.

15. • Shakoei S, Shahidi-Dadras M, Saeedi M, Ayatollahi A. Langerhans cell histiocytosis: an uncommon presentation, successfully treated by thalidomide.
Indian J DermatolVenereol Leprol. 2011;77(5):587-90. PMid:21860158. http://dx.doi.org/10.4103/0378-6323.84064.
• 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.
• Abdou AG, Taie DM. Perianal Langerhans cell histiocytosis: a rare presentation in an adult male. Autops Case Rep [Internet]. 2017;7(3):38-43.
http://dx.doi.org/10.4322/acr.2017.028

16. Take home message
Although case reports are in the base of
scientific evidence hierarches, they provides
resources of unusual information that may lead
advances in clinical practice.
“Always note and record the unusual …..Publish
it. Such information's are always of value”.
(William Osler)