Persistent erythema and edema of the midthird and upper aspect of the face (morbus morbihan): evidence of hidden immunologic contact urticaria and impaired lymphatic drainage.

Background: A persistent erythema and edema of the midthird and upper aspect of the face, which bears some resemblance to Melkersson-Rosenthal syndrome and rosaceous lymphedema, has been characterized as morbus morbihan (MM) by French dermatologists. The disease of yet unknown cause starts with recurrent facial edema of short duration, which ultimately leads to persistent swelling after a period of weeks or months.

Methods: We recruited 6 patients with MM and acquired their history, routine blood tests, and individual UV light tolerability. To check for contact allergies the allergen patch test and the open epicutaneous patch test were performed. To objectify the skin conditions laser Doppler flowmetry and 20-MHz ultrasound were used. Five patients with similar symptoms, but with definitely transient facial erythema and edema caused by proven contact urticaria on cosmetics served as a comparison group.

Results: In all patients, routine blood tests and UVA/UVB light tests showed no pathologic results. Observations of 6 patients with MM revealed the common feature of a clinically relevant immunologic contact urticaria caused by various cosmetic ingredients, which could be diagnosed in all of them. Delayed resorption of the acute edema and prolonged inflammation were shown by laser Doppler flowmetry and 20-MHz ultrasound in the affected skin areas in patients with MM after induction of immunologic contact urticaria by a cosmetic ingredient. Strict avoidance of cosmetics yielded a remarkable clinical benefit in the follow-up examinations.

Conclusions: We conclude that recurrent and possibly subclinical inflammation caused by immunologic contact urticaria in conjunction with a locally pre-existing lowered lymphatic drainage plays a crucial role in the evolution of MM.