Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Images
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Snippets
Special Article
Specialty Interface
Studies
Study Letter
Study Letters
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
Therapy Letters
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Net Letter
ARTICLE IN PRESS
doi:
10.25259/IJDVL_204_2023

Langerhans cell histiocytosis presenting as perianal ulcers

Department of Dermatology and Venereology, Gastroenterology and Human Nutrition, AIIMS New Delhi, India
Department of Gastroenterology and Human Nutrition, AIIMS New Delhi, India
Corresponding author: Dr. Sujay Khandpur, Dermatology and Venereology, AIIMS, New Delhi, India. khandpursujay@gmail.com
Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Sharma A, Mondal A, Aggarwal A, Khandpur S. Langerhans cell histiocytosis presenting as perianal ulcers. Indian J Dermatol Venereol Leprol. doi: 10.25259/IJDVL_204_2023

Dear Editor,

Langerhans cell histiocytosis (LCH) is a rare disease characterised by clonal proliferation of CD1a+ Langerhans cells that infiltrate into various organs, causing tissue damage. Though most commonly present in children less than four years of age, LCH is seen in all age groups. It is more often multisystemic (MS-LCH) in adults (28–69%), most commonly involving the bone (50–66%) followed by the lungs (61.7%) and skin (15–50.5%). 1,2 The cutaneous presentation of LCH is highly variable and includes erythematous scaly papules with or without petechiae, papulovesicles, plaques, nodules, erosions and ulcerations most commonly distributed on seborrheic and flexural areas. We present a rare manifestation of LCH in the form of perianal and perineal ulceration with concomitant anal and colonic ulceration.

A 21-year-old man was referred to the dermatology outpatient department in view of severely tender symmetric perianal ulcers for seven months. The patient had presented to the gastroenterology department three months ago and undergone a palliative diversion loop sigmoid colostomy in view of recalcitrant faecal leakage per anally as well as tenesmus and significant mucoid discharge for the past three years. He had no other areas of skin involvement, systemic symptoms, weight loss, or bone pain. A pituitary adenoma was detected on magnetic resonance imaging three years ago during evaluation of symptoms of excessive urination and thirst, which had completely resolved post-resection. Examination revealed symmetric, well-defined, severely tender, indurated and ulcerated plaques with a peripheral raised, hyperpigmented border over the natal cleft and perineal area, extending to the medial aspect of the gluteal region and inguinal folds [Figure 1]. It was associated with a foul-smelling, sticky, yellowish discharge. There was no locoregional lymphadenopathy. Routine evaluation, including complete blood count, liver and renal function tests, were within normal limits. Evaluation for his gastrointestinal complaints revealed a long segment thickening involving the rectum and recto-sigmoid junction on the contrast enhanced computed tomography (CECT) abdomen that was metabolically active on positron emission tomography (PET-CT). These findings were suggestive of an inflammatory disease, with possibility of Crohn’s disease or infective colitis. A colonoscopy revealed multiple rectal ulcers and sinuses. Histopathology from the colonic ulcer showed villous adenomatous polypoid tissue with focal low-grade dysplasia and squamous epithelium with subepithelial, dense, acute on chronic inflammation. There were multiple eosinophilic abscesses as well as numerous cells with nuclear grooving. In immunohistochemistry (IHC), these cells were CD1a+, langerin+ and S-100+, suggestive of LCH. On the basis of the above clinical features and evaluation, differential diagnoses of ulcerated perianal LCH, metastatic Crohn’s disease, pyoderma gangrenosum and pyoderma vegetans were considered. Histopathological examination of a biopsy taken from the ulcer edge revealed a large collection of Langerhans cells in the papillary dermis [Figure 2], admixed with lymphocytes, histiocytes and plasma cells, along with mild pigment incontinence. IHC was positive for CD1a & langerin, confirming perianal cutaneous LCH. Serum and urine osmolality, and bone marrow aspiration were normal. CT abdomen had not revealed any hepatosplenomegaly. Patient was referred to medical oncology, and was initiated on a regimen of vinblastine 14 mg (6 mg/m2) IV weekly and prednisolone 95 mg (40 mg/m2) daily. However, he developed severe diabetic ketoacidosis within three weeks and expired despite supportive care.

Figure 1:
Well-defined, symmetric, indurated and ulcerated plaques with peripheral raised, hyperpigmented border over the natal cleft, extending to medial buttocks.
Figure 2a:
Skin biopsy from ulcerated plaque showing irregular acanthosis and papillomatosis with moderately dense inflammation at one end in the papillary dermis, extending to the deeper dermis (Haematoxylin and Eosin, 40x).
Figure 2b:
Higher magnification shows collection of Langerhans cells (white arrows) admixed with lymphocytes, histiocytes and plasma cells (Haematoxylin and Eosin, 400x).

This patient was a case of adult MS-LCH involving the skin, gastrointestinal tract and likely pituitary gland with atypical cutaneous manifestations in the form of perianal and perineal ulceration. Perianal and anal involvement in LCH is rare. A recent literature review by Hamdan et al. identified 15 published reports of adult perianal LCH. 3 The mean age was 38 years, with a male predominance of 73% (11/15). Most of these patients presented with ulcerated to vegetative, indurated plaques with raised borders, similar to our case. The latent period for diagnosis ranged from four months to five years. Eighty percent of these patients had multisystem involvement, with gastrointestinal tract involvement in 13.3% (2 of the 15 cases) which is otherwise rarely seen (0.9%). 4 There was a preceding history of diabetes insipidus in one-third of the patients, a known harbinger of LCH. 5 Perianal LCH in paediatric age group has also been reported in 8 cases between 1984 and 2016, 75% (6/8) of whom had bony lesions and one case also had gastrointestinal tract involvement. 6 Perianal LCH may mimic Crohn’s disease with respect to both perianal and colonic ulcers that show chronic inflammation with crypt distortion and lympho-histiocytic infiltrate. 7 A long list of differentials gets elicited in such cases, and so a high index of suspicion is required to exclude the possibilities of conditions like Crohn’s disease, cutaneous tuberculosis, extramammary Paget’s disease, and atypical herpes. LCH should be considered in the list of differentials for perianal ulcerative lesions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

References

  1. , , , , , . Langerhans cell histiocytosis in adults. Report from the International registry of the histiocyte society. Eur J Cancer. 2003;39:2341-8.
    [CrossRef] [PubMed] [Google Scholar]
  2. , . Langerhans’-cell histiocytosis in 58 adults. Eur J Med Res. 2004;9:510-51.
    [PubMed] [Google Scholar]
  3. , , . Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: A case report and review of the literature. J Med Case Rep. 2021;15:357.
    [CrossRef] [PubMed] [PubMed Central] [Google Scholar]
  4. , , , , , . [A clinicopathological analysis of 160 cases of adult Langerhans cell histiocytosis] Zhonghua Xue Ye Xue Za Zhi. 2015;36:135-9. [Chinese]
    [CrossRef] [PubMed] [PubMed Central] [Google Scholar]
  5. , , , , , . Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatr Blood Cancer. 2004;43:594-9.
    [CrossRef] [PubMed] [Google Scholar]
  6. , . Perianal Langerhans cell histiocytosis: A rare presentation in an adult male. Autops Case Rep. 2017;7:38-43.
    [CrossRef] [PubMed] [PubMed Central] [Google Scholar]
  7. , , , , . Langerhans cell histiocytosis presenting as Crohn’s disease: A case report. Int J Colorectal Dis. 2018;33:1501-4.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
919

PDF downloads
29
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections