Persistent Facial Edema

presented by David Elpern MD,

Williamstown, Massachusetts, USA

on March 21, 2004

Dermatologist, The Skin Clinic, Williamstown, Massachusetts, USA

 
Name
D.B,,50-year-old white woman
Duration
1.5 years
Distribution
Face
History

This woman has a history of mild rosacea for many years. Over the past 1.5 years she has developed fixed facial edema. Her problem list includes asthma, breast cancer, hypothyroidism, "arthritis", manic depressive psychosis, irritable bowel syndrome and rosacea. She is a fair historian.

In the past few years she has taken omeprazole, clonazepam, tramadol, tamoxifen, quetiapine fumarate, lithium, hydrochlorthiazide, and gabapentin. She has used 1% hydrocortisone cream to the affected area at least daily for > 10 years for "rosacea." She has no medical insurance and takes the the above medications intermittently at present (when she can afford them!!).
Physical Examination
Pertinent findings on forehead and upper face. There is marked erythema and woody edema of forehead and malar eminences. Her lips look a bit puffy, too. There are a few erythematous papules. This did not improve when went off topical hydrocortisone.

Images

The last image was the picture of the patient before her problem began.

Laboratory Data
CBC, Blood chemistry and UA normal
Histopathology

There is marked edema of the reticular dermis and a superficial and deep perivascular lymphoplasmacytic inflammatory infiltrate. This histologic pattern is non-specific.
Diagnosis
Persistent and Solid Facial edema
Comments and References

Is this Morbihan's Disease? What role do you think ten years of hydrocortisone cream plays in the genesis of her problem? Do you have any therapeutic suggestions? D.B. is anxious to have her old face back.

References

1. Holzle E, Jansen T, Plewig G. [Morbihan disease--chronic persistent erythema and edema of the face] Hautarzt. 1995 Nov;46(11):796-8.
[Article in German] Hautklinik und Poliklinik, Universitats Krankenhauses, Eppendorf, Universitat Hamburg.

Morbihan's disease was first reported as a distinct entity in 1957 by Degos, describing a chronic persistent erythema and oedema of the upper half of the face. Such conditions have been noted in the literature designated as
chronic lymphoedema or solid persistent facial oedema in acne or rosacea. The characteristic features are a chronic course, a typical clinical picture, lack of specific laboratory and histological findings and refractoriness to
therapeutic measures.

2. Harvey DT, Fenske NA, Glass LF. Rosaceous lymphedema: a rare variant of a common disorder. Cutis. 1998 Jun;61(6):321-4.
Division of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine 33612, USA.

Rosaceous lymphedema is considered to be a rare and disfiguring variant of acne rosacea. Cases remain difficult to treat and can challenge afflicted patients both cosmetically and psychologically. We describe an unusual presentation of rosaceous lymphedema and review the differential diagnosis of persistent
facial edema.

Comments from Members

Yelva Lynfield MD, Cedarhurst, New York, USA on Mar 21, 2004

Poor woman! All those medical and psychiatric problems plus a complication of rosacea that is visible to everyone! Please give her some doxycycline and let us know whether it helps.

Jairo Messa MD, Manizales, Columbia on Mar 21, 2004

I saw a case in past with good response to isotretinoin

Julian Manzur MD, Havana, Cuba on Mar 21, 2004

Very interesting`patient. I have never seen a patient with rosaceous lymphedema. Sure, she needs all our efforts to improve

Khaled El-hoshy MD, Troy, Michigan,USA on March 22, 2004

Consider Melkersson-Rosenthal syndrome; SVC syndrome; Rosacea/acne facial edema.
?trial of furosemide. Check thyroid.

Sunil Dogra MD, Dept. of Dermatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India on Mar 23, 2004

It was indeed an interesting case but challanging from the therapy point of view. I will like to treat such case with low dose oral isotretinoin for a long period. Low dose oral prednisolone for short period may also be considered in the initial period to reduce inflammation and edema.

Farid Youssef MD, Cairo, Egypt on Mar 25, 2004

A very interesting case. I never saw one like it before will be great to see the effect of ketotifen 1-2mg /day in combination with isotretinoin 0.2-0.5mg/kg/day for 4-5 months. Reported to work in Morbihan's disease.

Nidal Dabbour MD, Saudi Arabia on Mar 26, 2004

Hydroxychloroquine 200mg twice daily was effective in a young man with same clinico-pathological presentation

Abir Saraswat MD, Lucknow, India on March 30, 2004

The underlying cause for her rosacea may be revealed if the treatment history is probed a little more. Her list of (intermittent) medications does not include any drug for her asthma. If she has been on high dose inhaled/ low dose oral steroids for it, that may be the reason, rather than off-and-on hydrocortisone cream. As regards treatment, isotretinoin may be best, but will she be able to afford it without insurance? Bipolar disorder may also be a (controversial) contraindication.

Caroline Koblenzer M.D., Professor of Dermatology and Psychiatry, University of Pennsylvania, Philadelphia, PA, USA on October 1, 2004

I would think that this is a dead-ringer for Morbihan's disease, and I too would think in terms of isotretinoin. What about protopic ? and also facial massage? Does she see a psychiatrist at all? I guess not, given her lack of insurance. Regular visits to a therapist would of course be helpful, in terms of living with the disfigurement. I don't know what the prognosis is----I guess we'll have to wait for the next Blue Journal, where I expect to see therapy and prognosis discussed, in Part 2 of the CME article !!---