Ann Dermatol.  2019 Apr;31(2):196-200. 10.5021/ad.2019.31.2.196.

Case Series of Morbihan Disease (Extreme Eyelid Oedema Associated with Rosacea): Diagnostic and Therapeutic Approaches

• Kim JE
• Sim CY
• Park AY
• Hong SA
• Park YL
• Jang SY
• Lee SY

• KMID: 2439065
• DOI: http://doi.org/10.5021/ad.2019.31.2.196

Abstract

Morbihan disease (MD) is a rare form of rosacea that presents with chronic erythema and solid oedema on the upper half of the face. A diagnosis of MD can be made only after eliminating diseases that are similar in terms of clinical and histopathological presentation. The cause of MD remains unclear and no standardised treatment is yet available. MD often tends to be recalcitrant to therapies commonly used to treat rosacea, including systemic corticosteroids, antibiotics, isotretinoin, and topical regimens. Thus, surgical interventions have been attempted but most cases have exhibited unsatisfactory responses. We treated six patients with extreme eyelid lymphoedema without any other cutaneous manifestation. Surgical eyelid reduction was performed in all patients, because ptosis and narrowing of the visual field were the major complaints. Histopathological tests revealed various extents of perivascular and perifollicular inflammation, and dermal oedema. After surgery, patients with severe inflammatory cell infiltration (including mast cells) exhibited a tendency toward recurrence. Other patients with severe dermal oedema exhibited better responses to surgical reduction, and thus no recurrence. We propose that MD should be included in the differential diagnosis of persistent, chronic eyelid oedema even if eyelid oedema is the only manifestation; the histological features may aid in the selection of appropriate therapeutic strategies. We suggest that eyelid reduction surgery can be a useful treatment option for MD patients when there is no massive mast cell infiltration.

Keyword

Edema; Eyelids; Rosacea

MeSH Terms

Adrenal Cortex Hormones
Anti-Bacterial Agents
Diagnosis
Diagnosis, Differential
Edema
Erythema
Eyelids*
Humans
Inflammation
Isotretinoin
Mast Cells
Recurrence
Rosacea
Visual Fields
Adrenal Cortex Hormones
Anti-Bacterial Agents
Isotretinoin

Figure

• 

  Fig. 1 Initial clinical presentation of patient 1 to 3. (A~C) Soft, non-pitting oedema without erythema on both eyelids. Clinical presentation after 6 months of surgical reduction of patient 1 to 3. (D~F) All showed marked improvement.

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  Fig. 2 On H&E staining, (A) perifollicular inflammation with minimal oedema of dermis in inflammatory type and (B) mild perifollicular inflammation in oedema type are found (H&E, ×100). (C) Immunohistochemistry (IHC) for CD117 shows the increased number of mast cell in inflammatory group, (D) comparing to those of odedma group (IHC, ×200). (E) The number of CD68-positive histiocyte is increased in inflammatory group than in (F) odema group (IHC, ×200).

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