Morbihan disease

A 24-year-old male came to our clinic with complaints of bilateral eyelid swelling that appeared spontaneously and progressively, 3 months before. No personal pathological history was reported. He had previous diagnoses of angioedema and contact dermatitis, and he was treated with desloratadine (5 mg), and prednisone (20 mg) for 20 days without improvement. Physical examination showed non-pitting edema and bilateral erythema of the eyelids and cheeks (Figure 1). No pain or itching was noticed, and investigations were normal. Histopathological analysis was characterized by dermal edema, nodular infiltrations of histiocytes and perivascular infiltration of lymphocytes, and sebaceous gland hyperplasia. The clinical diagnosis was Morbihan disease. The patient was treated with isotretinoin (20 mg/d) administered after thyroid and lipids profiles, and liver function test were normal. After 6 months of this treatment, the edema disappeared completely.

Figure 1 General appearance of the patient’s face with bilateral distribution of erythematous and non-pitting edema located on the eyelids and cheeks. This image is published with the patient’s consent.

Morbihan disease is a rare complication of rosacea or acne vulgaris characterized by a chronic persistent erythematous solid facial edema that could be related with facial vasoreactivity and inflammatory reaction to Demodex mites; however, its pathogenesis remains imprecise and only a reduced number of clinical cases have been described. This malady has no correlation of systemic clinical manifestations and laboratory abnormalities. The dermatological lesions of this illness persist indeterminately with no propensity to spontaneous disappearance without a specific management occasioning distortion of facial contours and even in ophthalmic impairments. Consequently, it is important to exclude other diseases, such as Melkersson-Rosenthal disease, lupus vulgaris, discoid lupus erythematosus, angioedema, sarcoidosis, dermatomyositis, chronic contact dermatitis, cutaneous leishmaniasis and leprosy. The diagnosis of Morbihan disease is clinical, and the histopathological analysis is non-specific. There are several therapeutic options including antibiotics, corticosteroids, lymphatic drainage, and in case of redundant edematous tissue, the excision with CO₂ laser blepharoplasty; but fortunately, our patient had a good response to isotretinoin that might inhibit the adverse effects of chronic inflammation as the fibroblast proliferation and migration, with the aim of avoiding damage to the dermal matrix (1).

Acknowledgments

Funding: None.

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References

1. Olvera-Cortés V, Pulido-Díaz N. Effective Treatment of Morbihan's Disease with Long-term Isotretinoin: A Report of Three Cases. J Clin Aesthet Dermatol 2019;12:32-4. [PubMed]