Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
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 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
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:

Case Letter
ARTICLE IN PRESS
doi:
10.25259/IJDVL_1174_2021

A case of Renbök phenomenon in a patient with alopecia areata and nevus flammeus

Department of Dermatology, Peking University People’s Hospital, Xicheng District, Beijing, China
Corresponding author: Dr. Cheng Zhou, Department of Dermatology, Peking University People’s Hospital, Xicheng District, Beijing, China. chengzhou@live.cn
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: Zhang W, Li X, Chen B, Zhang J, Zhou C. A case of Renbök phenomenon in a patient with alopecia areata and nevus flammeus. Indian J Dermatol Venereol Leprol doi: 10.25259/IJDVL_1174_2021

Sir,

Alopecia areata is a common condition characterised by sudden onset of non-scarring patchy hair loss. Alopecia areata is considered as an organ-specific, T cell-dependent autoimmune disease with a genetic background. The exact aetiopathology remains unclear. It is presumed that alopecia areata results from the collapse of the immune privilege of the anagen hair follicles with the subsequent assault on the follicle bulb by CD8+ T lymphocytes. The term Renbök phenomenon was first described in 1991 by Happle et al.1 They reported retained hair growth localised to psoriasis plaques in patients with co-existing psoriasis and alopecia areata. Subsequently, many other diseases displaying Renbök phenomenon have been reported. Here, we report a case of severe alopecia areata with extensive involvement of the scalp showing retained hair growth in co-existing nevus flammeus. This case might provide further evidence for the occurrence and potential aetiopathology of the Renbök phenomenon in alopecia areata.

A 20-year-old Chinese woman presented with hair loss for eight years. She had patchy hair loss eight years ago, which was diagnosed as alopecia areata and resolved spontaneously. However, diffuse hair loss started suddenly three years ago without any trigger and gradually progressed to alopecia universalis. She was treated with oral prednisone 20 mg once daily and intralesional betamethasone once a month for three months, with minimal hair regrowth. Interestingly, throughout the course of the disease, the hair on a born nevus flammeus on her occipital scalp was mildly involved. She had a past history of allergic rhinitis. Her family history was noncontributary. Physical examination revealed an extensive loss of hair on her scalp with a SALT score of 85 per cent [Figure 1a]. Nevus flammeus on the occipital area was covered with a fair density of terminal hairs [Figure 1b]. A dermoscopic examination of the clinically spared area revealed reticular vessels and terminal hairs [Figure 1c].

Figure 1a:: Extensive alopecia areata
Figure 1b:: Obvious terminal hair growth within the area of a nevus flammeus on the occipital scalp
Figure 1c:: Dermoscopy under polarized light (CBS-907; 50× magnification) of the clinically spared area showing reticular vessels and terminal hairs

There have been several case reports and studies describing alopecia areata sparing nevus flammeus, which suggest a possible association. A case-control study showed that the prevalence of nuchal nevus flammeus was significantly increased in patients with alopecia areata, especially in severe and chronic forms.2 However, the pathophysiological mechanism of the Renbök phenomenon for nevus flammeus in alopecia areata is still unknown.

Nevus flammeus is a disorder caused by genetic mosaicism, which may explain the sparing of hair loss on nevus flammeus.3 The mosaic skin might express altered immunomodulatory proteins in protected skin which are distinct from that in overlapping inflammatory skin.4 Previous findings have illustrated that the Th1 alopecia areata inflammatory response and the Th17 psoriatic inflammation could oppose each other presumably through changing local cytokine milieu.4 The dominant inflammatory profile might ultimately determine the final cytokine phenotype. In this case, extensive hair loss of alopecia areata spared the occipital area with nevus flammeus, suggesting that the hair follicles within the nevus lesion could reverse its vulnerability to the autoimmune reaction initiated by T cells.

Nevus flammeus is also a congenital vascular or capillary malformation. The persistent lesions in adults can show ectasia of subpapillary capillaries.2 Vascular endothelial growth factor has also been proved to play an important role in the development of nevus flammeus which may promote angiogenesis and vasodilatation.5 Interestingly, vascular endothelial growth factor produced by hair follicles is significantly reduced in the skin of alopecia areata patients, resulting in a loss of vascular support to the affected scalp tissue.6 Therefore, in this patient vascular endothelial growth factor production within the nevus flammeus may increase vascular support and protect itself from hair loss. Diphenylcyclopropenone, also known as diphencyprone, which is currently considered effective contact immunotherapy for severe alopecia areata, has been proven to upregulate vascular endothelial growth factor in hair follicle keratinocytes of alopecia areata patients.7 The study suggests that there are different factors regulating vascular endothelial growth factor expression, like epidermal growth factor, transforming growth factor-α and transforming growth factor-β, the proinflammatory cytokine tumour necrosis factor-α, interleukin-1β and interleukin-6.7 It could be postulated that some cytokines also play a critical role in the hair follicle keratinocytes of an alopecia areata -sparing nevus flammeus, which may be involved in the immune reaction to some extent.

In conclusion, we report a case of Renbök phenomenon in extensive alopecia areata with retained hair growth in co-existing nevus flammeus and discussed the possible pathophysiological mechanism of this phenomenon. Further study of this sparing phenomenon may shed new light on underlying mechanisms involved in alopecia areata, potentially leading to the development of new therapeutic strategies.

Declaration of patient consent

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

Financial support and sponsorship

This study was supported by the National Natural Science Foundation of China (No. 82073459).

Conflict of interest

There are no conflicts of interest.

References

  1. , . The Renbök phenomenon: An inverse Koebner reaction observed in alopecia areata. Eur J Dermatol. 1991;2:39-40.
    [Google Scholar]
  2. , , , , , . The association between nuchal nevus flammeus and alopecia areata: A case-control study. Dermatology. 2005;211:334-7.
    [CrossRef] [PubMed] [Google Scholar]
  3. . Alopecia areata universalis sparing nevus flammeus. Dermatology. 2005;210:227-8.
    [CrossRef] [PubMed] [Google Scholar]
  4. , , . Renbok phenomenon and contact sensitization in a patient with alopecia universalis. Arch Dermatol. 2010;146:422-5.
    [CrossRef] [PubMed] [Google Scholar]
  5. , , , , , . The expression of vascular endothelial growth factor and its receptors in port-wine stains. Otolaryngol Head Neck Surg. 2008;139:560-4.
    [CrossRef] [PubMed] [Google Scholar]
  6. , , , . Loss of vascular endothelial growth factor in human alopecia hair follicles. J Invest Dermatol. 1995;104:18S-20S.
    [CrossRef] [PubMed] [Google Scholar]
  7. , , , , , . Expression of vascular endothelial growth factor, apoptosis inhibitors (survivin and p16) and CCL27 in alopecia areata before and after diphencyprone treatment: An immunohistochemical study. Br J Dermatol. 2004;150:940-8.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
702

PDF downloads
42
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections