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
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
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
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
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 - 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
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
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
Review Article
Review Articles
Reviewers 2022
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
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
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor - Observation Letter
doi: 10.4103/ijdvl.IJDVL_1004_18
PMID: 32496225

Morbihan disease: Look beyond facial lymphedema

Prince Yuvraj Singh1 , Sukriti Baveja1 , Deepak Vashisht1 , Manish Sharma2 , Prashant Sengupta2
1 Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India
2 Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India

Correspondence Address:
Prince Yuvraj Singh
Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra
Published: 03-Jun-2020
How to cite this article:
Singh PY, Baveja S, Vashisht D, Sharma M, Sengupta P. Morbihan disease: Look beyond facial lymphedema. Indian J Dermatol Venereol Leprol 2020;86:418-421
Copyright: (C)2020 Indian Journal of Dermatology, Venereology, and Leprology


Morbihan disease is characterized by chronic and recurrent pattern of symmetrical facial edema and erythema, over the middle and upper thirds of the face (malar regions, nose, glabella, eyelids and forehead). Its pathogenesis remains largely unknown but the clinical manifestations are presumably caused by derangements in local cutaneous vasculature and imbalance between lymph production and drainage.[1] Some authors also hypothesize that a primary lymph vessel abnormality in the involved skin is the underlying cause. We report a case of this rare entity, in association with primary genital lymphedema.

A 23-year-old male presented with complaints of swelling and redness of the upper eyelids and face, of 7 years duration. The swelling initially started on the upper eyelids and gradually progressed over the next few years to involve the forehead and both cheeks. He denied any pain, itching or worsening of symptoms after sun exposure. There was no history of association with food intake, stress, contact allergy or any visual symptoms. Two years prior to the onset of the facial rash, he was diagnosed with primary penoscrotal lymphedema when he developed progressive penoscrotal swelling causing urinary obstruction and difficulty in retraction of the prepuce. He underwent scrotoplasty and circumcision with relief from urinary symptoms; however, the penoscrotal edema persisted. Dermatological examination revealed erythematous edema with mild scaling involving the forehead, glabella, cheeks and both upper and lower eyelids, causing difficulty in opening his eyes completely [Figure - 1]. There was edema of the penis and scrotum with deformity of the penis [Figure - 2] and increased rugosity of the scrotal skin along with healed scars [Figure - 3]. Dermoscopic examination of the lesions on the face revealed linear vessels and white chrysalis-like structures on a yellow background. Differential diagnoses of Morbihan syndrome, Melkersson–Rosenthal syndrome, sarcoidosis and granuloma faciale were considered. All laboratory investigations including hematologic, biochemical and serological investigations, serum angiotensin converting enzyme levels, chest X-ray and high-resolution computed tomography thorax were normal. Histopathological examination of a punch biopsy done from the forehead revealed sebaceous gland hyperplasia, dermal edema, extravasation of RBCs and perivascular and periadnexal inflammatory infiltrate [Figure - 4] composed of lymphocytes, histiocytes and plasma cells [Figure - 5]. Toluidine blue stain showed an increase in the number of mast cells [Figure - 6]. Dilated lymphatic channels were seen in superficial and deeper dermis, lined by D2-40 positive endothelial cells [Figure - 7]. Histopathological examination of the scrotal skin revealed dermal edema, chronic inflammatory infiltrate and dilated ectatic lymphatics [Figure - 8]. Immunohistochemistry (IHC) with CD-34 staining demonstrated normal arterioles lined by endothelial cells and dilated lymphatic channels [Figure - 9]. Taking into account all the findings, the patient was diagnosed as a case of Morbihan disease. Along with sun-protective measures and a broad-spectrum sunscreen application, he was started on cap isotretinoin 20 mg daily which was later increased to 40 mg daily and was continued for 24 weeks; however, no significant improvement was seen with the therapy.

Figure 1: Erythematous solid edema with mild scaling, involving the face and both eyelids causing difficulty in opening the eyes completely
Figure 2: Edema of the penis and scrotum with deformity of the penis
Figure 3: Increased rugosity of scrotal skin along with healed scar marks
Figure 4: Sebaceous hyperplasia (red arrow) with a dense periadnexal inflammatory infiltrate (blue arrow) (H and E, ×100)
Figure 5: Inflammatory infiltrate comprising lymphocytes, histiocytes and plasma cells (H and E, ×400)
Figure 6: Numerous mast cells (yellow arrows) with prominent metachromatically stained cytoplasmic granules (toluidine blue, ×1000)
Figure 7: On immunohistochemistry, numerous dilated lymphatics (black arrows) lined discontinuous lining of D2-40 positive endothelial cells (IHC D2-40, ×400)
Figure 8: Scrotal biopsy: Dermal edema, chronic inflammatory infiltrate and dilated ectatic lymphatic vessels (yellow circles) (H and E, ×100)
Figure 9: Scrotal biopsy: Immunohistochemical staining showing normal arterioles lined by endothelial cells (black arrow) and numerous dilated lymphatic channels (yellow arrows) (immunohistochemistry CD34, ×400)

Morbihan disease was first described by the French dermatologist Robert Degos in 1957. This condition was first reported from the French region of Morbihan (north-western France); hence, the name, Morbihan disease. Since its description, it has been called by several other names, including solid persistent facial edema, rosaceous lymphedema and morbus Morbihan. Clinically, it presents with chronic and recurrent symmetrical persistent erythema and solid edema of the upper two-thirds of the face.[1] Morbihan disease is predominantly seen in Caucasians and on a literature search; we could find only one Indian patient being reported previously with the disease.[2]

This disease is a likely manifestation of downstream effects from rosacea's recurrent episodes of vasodilation and chronic inflammation. Some authors also hypothesize that it may be caused by an underlying primary lymph vessel abnormality. Ultrasonography and laser Doppler flowmetry demonstrated a preexisting lymphatic drainage defect in the affected skin of one such patient with this condition.[3] Recently, immunohistochemical studies have shown an increase in D2-40 expression in the lymphatic endothelium of the skin affected by Morbihan disease, suggesting that a primary abnormality in lymphangiogenesis may be involved in the pathogenesis.[1]

Histopathological features of Morbihan disease include dermal edema, dilated blood vessels, perifollicular fibrosis and perivascular and perifollicular lymphohistiocytic infiltrates. Rarely, increased mast cell infiltration may be seen and their degranulation may be the underlying cause of collagen deposition and fibrotic reaction seen in Morbihan disease.[4] Sebaceous gland hyperplasia may be observed and rarely, granulomas may also be present. In a case of Morbihan disease, dilated and damaged lymphatics with adjacent epithelioid cell granulomas and lymphatic obstruction were reported by Nagasaka et al.[5] Our patient had histopathological findings consistent with Morbihan disease, which were further reinforced by toluidine blue staining and immunohistochemical staining.

Along with the classical findings of Morbihan disease, our patient had primary genital lymphedema. Genital lymphedema occurs as a result of accumulation of lymph in the superficial lymph vessels between the skin and fascia (Colles fascia in the scrotum and Buck's fascia of the penis). Primary lymphedema occurs due to hypoplasia or aplasia of the lymphatics and is classified as per the age of onset as congenital lymphedema variant (10%–20%) and the praecox variant (80%).[6] Our patient belonged to the latter group with the onset of genital lymphedema around 14 years of age.

Treatment options for Morbihan disease reported in the literature include isotretinoin, systemic antibiotics (tetracyclines, metronidazole), antihistamines (ketotifen), systemic corticosteroids, clofazimine and thalidomide which generally overlap with the management of rosacea.[7] However, at best, these drugs are associated with a reduction of only facial erythema with little or no improvement in facial edema.[7],[8] Although about 15%–20% of patients may not respond to isotretinoin, it is considered the first-line of therapy.

In conclusion, we report a rare case of Morbihan disease in association with primary genital lymphedema. Further research and studies are required to successfully comment on a definite association between this disease and the underlying primary lymphatic vessel abnormality, as hypothesized previously by a few authors.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.


We are thankful to our patient for his support and cooperation.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

Morales-Burgos A, Alvarez Del Manzano G, Sánchez JL, Cruz CL. Persistent eyelid swelling in a patient with rosacea.PR Health Sci J 2009;28:80-2.
[Google Scholar]
Mahajan PM. Solid facial edema as a complication of acne vulgaris. Cutis 1998;61:215-6.
[Google Scholar]
Romiti N. Morbus morbihan: Solid and persistent facial edema and erythema in Portuguese. An Bras Dermatol 2000;75:599-603.
[Google Scholar]
Fujimoto N, Mitsuru M, Tanaka T. Successful treatment of morbihan disease with long-term minocycline and its association with mast cell infiltration. Acta Derm Venereol 2015;95:368-9.
[Google Scholar]
Nagasaka T, Koyama T, Matsumura K, Chen KR. Persistent lymphoedema in Morbihan disease: Formation of perilymphatic epithelioid cell granulomas as a possible pathogenesis. Clin Exp Dermatol 2008;33:764-7.
[Google Scholar]
Namir SA, Trattner A. Transient idiopathic primary penoscrotal edema. Indian J Dermatol 2013;58:408.
[Google Scholar]
Veraldi S, Persico MC, Francia C. Morbihan syndrome. Indian Dermatol Online J 2013;4:122-4.
[Google Scholar]
Ranu H, Lee J, Hee TH. Therapeutic hotline: Successful treatment of Morbihan's disease with oral prednisolone and doxycycline. Dermatol Ther 2010;23:682-5.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections