Clinicopathological and Immunohistochemical Study of 14 Cases of Morbihan Disease: An Insight Into Its Pathogenesis
Jose L Ramirez-Bellver1, Yosmar C Pérez-González2,3, Ko R Chen4
1Dermatologist, Department of Dermatology, Fundación Jiménez Diaz, Madrid, Spain.
Introduction:
Morbihan disease (MORD) is rare with only 45 clear-cut cases previously reported. Histopathologic findings are supposed to be nonspecific. We report 14 patients and review the previous cases.
Objectives:
To characterize the clinicopathologic findings, outcomes, and immunophenotype of MORD.
Material And Methods:
Inclusion criteria were a clinical picture of persistent, nonpitting edema affecting the mid and or upper third of the face and histopathological findings fitting previous reports and exclusion of other entities.
Results:
The majority of our patients were males (71.5%) with a male/female ratio of 10/4. The mean age when diagnosed was 58.8 years. Eyelids and forehead were the most frequently involved areas. Two of the patients presented previous rosacea. Most constant histopathological findings were lymphatic vessel dilatations in the upper dermis and the presence of mast cells (7.5 in 10 high-power field as a mean). Mild edema was also present in most of the cases. Granulomas were found in 7 of the cases, and immunostaining with CD68 and CD14 only revealed an additional case.
Conclusions:
MORD occurs more in middle-aged males, not associated with rosacea and mostly affects eyelids and forehead. Granulomas are not mandatory for the diagnosis. Histopathology of MORD fits within the spectrum of localized lymphedema.
Insights
Morbihan disease (MORD) is a rare condition affecting middle-aged males, characterized by facial edema, primarily of the eyelids and forehead. Histopathology reveals lymphatic vessel dilatations and mast cells, fitting within localized lymphedema.
Area of Science:
- Dermatology
- Pathology
Background:
- Morbihan disease (MORD) is a rare condition with limited reported cases.
- Previous histopathologic findings were considered nonspecific.
- This study presents 14 new cases and reviews existing literature.
Purpose of the Study:
- To define the clinicopathologic features of Morbihan disease.
- To investigate patient outcomes.
- To characterize the immunophenotype associated with MORD.
Main Methods:
- Inclusion criteria: persistent, non-pitting facial edema (mid/upper face) and compatible histopathology.
- Exclusion of other potential diagnoses.
- Review of 14 patient cases and previous literature.
Main Results:
- MORD predominantly affects middle-aged males (71.5%) with a mean age of 58.8 years.
- Eyelids and forehead are the most common sites of edema.
- Key histopathologic findings include lymphatic vessel dilatations and mast cell presence; granulomas are not consistently observed.
Conclusions:
- Morbihan disease primarily affects middle-aged males, with eyelids and forehead being the most affected areas.
- Rosacea is not a significant associated factor.
- Histopathology aligns with localized lymphedema, and granulomas are not essential for diagnosis.
