Jerković Gulin, Sandra

Ljubojević Hadžavdić, Suzana

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118Morbihan Disease – An Old and Rare Entity Still  Difficult to TreatActa Dermatovenerol Croat                              2020;28(2):118-119                              LETTER TO THE EDITORMorbihan disease (MD), also known as Morbihan syndrome, “solid persistent facial edema and erythe-ma”, “rosacea lymphedema”, and “solid facial edema in acne”, is a rare and often unrecognizable entity, that presents with a slow occurrence of persistent lymph-oedema of the upper two-thirds of the face (1,2). A 30-year-old woman presented to our Depart-ment with persistent, asymptomatic face edema and erythema lasting for 18 months. She was previously treated for rosacea with doxycycline (100 mg/day for four months) without improvement. Dermatologi-cal examination revealed erythematous, nonpitting, solid edema located on the mid-forehead, nose, and cheeks with sparse erythematous papules and pus-tules on the entire face including the chin and com-edones, papules, and pustules on the back (Figure 1 and Figure 2). She was otherwise healthy and was not taking any medication. Laboratory tests with immunological tests and Quantiferon test together with MRI of the orbits, chest X-ray, chest high-reso-lution computed tomography, cranial X-ray, and ab-dominal ultrasound were all within normal limits. Histopathology revealed dermal edema, perivascu-lar and peri-adnexal lymphohistiocytic infiltrate, and sebaceous gland hyperplasia. Based on the typical clinical picture, histopathological findings, and the exclusion of several differentials the diagnosis of MD was established. The patient was treated with oral isotretinoin (20 mg/day for eight months) without re-gression of solid edema and erythema on the face but with complete regression of acne on the trunk. She was started on oral corticosteroids (prednisolone, 20 mg/day for two months followed by reduction of the dose over three months), again with only slight short transient improvement and rapid relapse of facial erythema and edema. The patient refused any other suggested treatment. We treated our patient for a to-tal 2 years and followed up for 5 years. Figure 1. Erythematous, nonpitting, solid edema on the mid-forehead, nose, and cheeks with sparse erythematous papules and pustules. Figure 2. Comedones, papulopustular acne on the back.ACTA DERMATOVENEROLOGICA CROATICA119ACTA DERMATOVENEROLOGICA CROATICAThe pathogenesis of MD is still unknown. It is con-sidered a clinical variety or a complication of rosacea or acne which does not tend to regress spontaneous-ly. It is believed that chronic inflammation in patients with MD is due to acne or rosacea causing structural damage to blood and lymph vessels (1,3,4). However, cases of MD without previous history of rosacea and acne have been reported supporting the distinct dis-ease theory (3,4). Edema and erythema are localized on the upper half of the face affecting the forehead, glabella, eyelids, nose, and cheeks. Although the symptoms may come and go, MD usually does not improve without treatment (5). Several therapeutic options have been reported, although there is no es-tablished standard treatment for MD. Reported ther-apy includes short-term oral isotretinoin (0.5 mg/kg/day), long-term oral isotretinoin (40-80 mg/day, 10-24 months), long-term doxycycline, combination of sys-temic corticosteroids and antibiotic (prednisolone 20 mg/day for 2 weeks and doxycycline 200 mg/day for 12 weeks), slow-releasing doxycycline monohydrate (40 mg/day for 6 months), long-term minocycline (50 mg/day for 4 months), and a combination of both oral retinoid and ketotifen (isotretinoin 0.7 mg/kg/day for 4 months, ketotifen 2 mg/day for 4 months) (1,2,6,8). The disease is frequently recalcitrant to therapy, and only several cases of successfully treated patients with MD have been reported (1,2,4, 6-8).We presented a patient with characteristic fea-tures of MD, which is a persistent, cosmetically dis-turbing condition, unfortunately mostly refractory to therapeutic measures. References:1. Smith LA, Cohen DE. Successful long-term use of oral isotretinoin for the management of Morbi-han disease: a case series report and review of the literature. Arch Dermatol. 2012;148:1395-8.2. Chaidemenos G, Apalla Z, Sidiropoulos, T. Mor-bihan disease: successful treatment with slow-releasing doxycycline monohydrate. J Eur Acad Dermatolol Venereol. 2018 Feb;32:e68-9. 3. Renieri G, Brochhausen C, Pfeiffer N, Pitz S. Chro-nisches Lidödem assoziiert mit Rosazea (Morbus Morbihan): differenzialdiagnostische Schwierig-keiten und Therapieoptionen. Klin Monbl Augen-heilkd. 2011;228:19-24.4. Kuhn-Régnier S, Mangana J, Kerl K, Kamarachev J, French LE, Cozzio A, et al. A report of two cases of solid facial edema in acne. Dermatol Ther. 2017;7:167-74.5. Kabuto M, Fujimoto N, Honda S, Tanaka T. Suc-cessful treatment with long-term use of mino-cycline for Morbihan disease showing mast cell infiltration: A second case report. J Dermatol. 2015;42:827-8.6. Fujimoto N, Mitsuru M, Tanaka T. Successful treat-ment of Morbihan disease with long-term mino-cycline and its association with mast cell infiltra-tion. Acta Derm Venereol. 2015;95:368-9.7. Eber AE, Mlacker S, Verne S, Griffith R, Ledon J, Perper M, et al. Morbihan Disease complicated by dermatosis neglecta: an unique presentation. J Cutan Pathol. 2017;44:470-3.8. Okubo A, Takahashi K. Akasaka T, Amano H. Four cases of Morbihan disease successfully treated with doxycycline. J Dermatol. 2017;44:713-6.Sandra Jerković Gulin1, Suzana Ljubojević Hadžavdić2 1Department of Dermatology and Venereology, General Hospital Sibenik, Sibenik, Croatia2Department of Dermatology and Venereology, University Hospital Center Zagreb, University of Zagreb School of Medicine, Zagreb, CroatiaCorresponding author: Associate Professor Suzana Ljubojević Hadžavdić, MD, PhDDepartment of Dermatology and Venereology, University Hospital Center Zagreb, University of Zagreb School of MedicineŠalata 410000 Zagreb,Croatiasuzana.ljubojevic@gmail.comReceived: March 10, 2018Accepted: May 15, 2020Letter to the editor Acta Dermatovenerol Croat  2020;28(2):118-119

Morbihan Disease – An Old and Rare Entity Still Difficult to Treat

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