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
Images in Dermatology
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
Media and news
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
Images in Dermatology
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
Media and news
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:

Letter To Editor
2004:70:5;313-314
PMID: 17642648

Granulomatous cheilitis evolving into Melkersson-Rosenthal syndrome with bilateral facial palsy

K Muhammed, G Nandakumar, Sumi Thomas
 Department of Dermatology and Venereology, Medical College, Kozhikode, Kerala - 673 008, India

Correspondence Address:
K Muhammed
Kunnummal House, Koroth School Road, Vatakara - 673 101, Kerala
India
How to cite this article:
Muhammed K, Nandakumar G, Thomas S. Granulomatous cheilitis evolving into Melkersson-Rosenthal syndrome with bilateral facial palsy. Indian J Dermatol Venereol Leprol 2004;70:313-314
Copyright: (C)2004 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by the classical triad of recurrent swelling of the lips and/or face, fissured tongue (lingua plicata) and relapsing peripheral facial nerve paralysis.[1] However, many patients do not manifest all signs of the triad. Melkersson in 1928 first described labial edema in association with recurrent facial palsy. Rosenthal in 1930 emphasized the role of genetic factors and added scrotal tongue to the syndrome. Monosymptomatic or oligosymptomatic forms in which only one or any two features of the triad are present are common.

The most frequent monosymptomatic form is granulomatous cheilitis (GC),[2] described by Meischer in 1945, which is defined as painless chronic isolated enlargement of one or both lips due to granulomatous inflammation with a recurrent to gradually persistent course. Histopathologic examination reveals focal noncaseating epithelioid cell granulomas with lymphocytes and plasma cells. The lymph nodes may also show granulomatous inflammation. The origin of GC/MRS is obscure and the treatment is notoriously difficult. Among the numerous postulated theories are genetic factors, chronic infectious odontogenic foci, autoimmune mechanisms, allergic reactions and local disturbances of the autonomic nervous system.[1] Traditional therapies include systemic or intralesional steroids,[3] metronidazole,[4] clofazimine,[5] minocycline and surgical methods. Other treatment options include thalidomide, sulfasalazine, erythromycin, azathioprine and cyclosporine.

A 46-year-old female school teacher presented to the dermatology department of Medical College Hospital, Calicut with complaints of recurrent episodes of asymptomatic swelling of the upper lip of three years′ duration. The swelling used to subside with treatment, but there was no complete remission. She did not have any systemic complaints. On examination the patient had a diffuse swelling of her upper lip with a firm rubbery consistency [Figure - 1].

With a provisional diagnosis of granulomatous cheilitis, we investigated the patient. Her blood biochemistry, urinalysis and chest X-ray were normal. Biopsy from the lesion showed non-caseating epithelioid cell granulomas [Figure - 2]. The patient was started on oral metronidazole (400 mg) twice daily. Her lip swelling partially subsided with treatment.

About six months later, she suddenly developed difficulty in closing her left eye and deviation of the angle of mouth to the right. On examination she was found to have left sided lower motor neuron (LMN) facial palsy [Figure - 3]. Her tongue appeared slightly larger than normal and showed superficial grooves on the anterior aspect. With these two new findings, the patient exhibited the complete triad of Melkersson-Rosenthal syndrome.

The facial palsy was managed with systemic steroids and physiotherapy. Oral clofazimine (100 mg) thrice daily along with intralesional triamcinolone acetonide (10 mg/ml) every three weeks were also started for the lip swelling. The facial palsy improved significantly and the lip swelling also subsided almost completely. Six months later, she again presented with relapse of right LMN facial palsy and was managed with systemic steroids and physiotherapy.

Clinically MRS may manifest as the classic triad, in an incomplete form, or with sequential appearance of the clinical findings. The onset of the disease is usually in middle age with no gender predominance. In a large series orofacial involvement was the predominant feature; it occurred in all 36 patients and was the presenting sign in 15 (42%).[2] The neurological features may precede the attacks of edema by months or years or may develop later and while initially intermittent, may occasionally become permanent.[1] Recurrent edema can be periorbital or vulval (vulvitis granulomatosa). In our patient, the labial edema responded partially to metronidazole and significantly to clofazimine and intralesional steroids,[3] but treatment did not prevent the occurrence of facial palsy. This case report highlights the interesting evolution of granulomatous cheilitis to full fledged MRS with bilateral facial palsy. The presence of the complete triad in a single patient is rare, being reported in only 10-20% of cases, and bilateral facial palsy in MRS is even rarer.[6]

References
1.
Robert RM, Wolfgang W, Natalie Z, Kaddu S, Soyer HP, El Shahrawi CL, et al. Granulomatous cheilitis and Borrelia burgdorferi: Polymerase chain reaction and serologic studies in a retrospective case series of 12 patients. Arch Dermatol 2000;136:1502-6.
[Google Scholar]
2.
Greene RM, Rogers RS 3rd. Melkersson-Rosenthal Syndrome: A review of 36 patients. J Am Acad Dermatol 1989;21:1263-70.
[Google Scholar]
3.
Perez-Calderon R, Gonzalo-Garijo MA, Chaves A, de Argila D. Chelitis granulomatosa of Melkersson-Rosenthal syndrome: Treatment with intralesional corticosteroid injections. Allerg Immunopathol 2004;32:36-8.
[Google Scholar]
4.
Kano Y, Shiohara T, Yagita A. Treatment of recalcitrant cheilitis granulomatosis with metronidazole. J Am Acad Dermatol 1992;27:629-30.
[Google Scholar]
5.
Arbiser JL, Moschella SL. Clofazimine: A review of its medical uses and mechanisms of action. J Am Acad Dermatol 1995;32:241-7.
[Google Scholar]
6.
Lopez Gonzalez R, Bragado Alonso A, Delgado Vicente S, Rodriguez Prieto MA. Melkersson-Rosenthal syndrome, about one case with bilateral facial paralysis. An Med Interna 2004;21:255-6.
[Google Scholar]

Fulltext Views
2,945

PDF downloads
547
Show Sections