Morbihan diseaseThe Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York
Stephanie W Hu MD, Maria Robinson MD, Shane A Meehan MD, David E Cohen MD
Dermatology Online Journal 18 (12): 27
Morbihan disease, which consists of solid facial edema, is a rare complication of rosacea, a common cutaneous disorder in middle-aged individuals. The characteristic features of Morbihan disease are its chronic course, typical clinical picture, lack of specific laboratory and histopathologic findings, and refractoriness to therapeutic measures. Since its initial description in 1957, only a small number of cases have been reported in the dermatologic literature. We report a 54-year-old man who developed a two-year duration of erythema and edema that affects the upper and mid face, with accentuation in the periorbital region. Patch tests excluded an allergic contact dermatitis and histopathologic investigation showed small, nodular clusters of epithelioid cells in the dermis that were consistent with sarcoidal granulomata. A diagnosis of Morbihan disease was made owing to the combination of clinical and histopathologic findings. Therapeutic options for the disease remain unsatisfactory and treatments reported in the literature include systemic glucocorticoids, oral tetracyclines, thalidomide, isotretinoin, ketotifen, and clofazimine. Our patient failed a six-to-seven months course of minocycline prior to presentation and has since experienced improvement on gradually-increasing doses of isotretinoin.
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A 54-year-old man presented to the Dermatology Faculty Group Practice at the New York University Langone Medical Center for evaluation of a two-year history of erythema and edema of the cheeks and periorbital region. Beginning in May, 2009, the patient noted swelling of the right side of the face that subsequently generalized to involve the left side. He denied pruritus or other symptoms preceding or accompanying the onset of the lesions. There was no history of trauma. In May, 2009, the patient began a new construction job that required using fire-rated plywood. He experienced exposure to appreciable amounts of woodworking dust. The patient denied a history of acne vulgaris or rosacea, although he did report more frequent exposure of the right side of the face to the sun. He denied allergies to medications or seasonal allergens. Prior to presentation, he was evaluated by two dermatologists, one of whom believed that the patient had rosacea. The other gave him a short course of prednisone, which provided no improvement. The patient also underwent a six-to-seven months trial of minocycline, which also did not prove beneficial. The patient was evaluated by an allergist and skin tests were negative.
A review of systems was negative. The patient was otherwise healthy and had no other past medical or surgical history. He took no medications other than those prescribed for this condition. The patient also denied the use of cigarettes or recreational drugs but did drink socially.
A punch biopsy was obtained from a representative area of erythema and edema on the right cheek.
The upper and mid face were involved with erythema, non-pitting and non-tender edema, and a woody, smooth-surfaced, non-scaling induration. These findings were particularly prominent in the periorbital region. Telangiectases, inflammatory papules, pustules, and rhinophyma were not present.
A complete blood count, complete metabolic panel, liver function tests, iron panel, thyroid stimulating hormone, erythrocyte sedimentation rate, rheumatoid factor, vitamin B12, folate, and vitamin D levels were normal. Total cholesterol, LDL cholesterol, and cholesterol/HDL ratio were elevated to 251, 189, and 5.3, respectively. Patch tests to the North American Standard Series as well as a specialized panel to plastics and glues were negative.
Within the dermis, there are nodular aggregates of epithelioid histiocytes and admixed lymphoctes.
Rosacea, which is a common cutaneous disease that affects middle-aged individuals, presents with a variety of clinical manifestations. The facial convexities are often impacted, with the primary clinical features comprising frequent flushing, persistent erythema, telangiectases, papules, and pustules . It is a chronic condition of facial vasoreactivity and an inflammatory response to Demodex mites that reside within the large sebaceous follicles in the center of the face has been implicated in its pathogenesis. The degree of involvement is highly variable and the standard classification system established by the National Rosacea Society in 2002 included four subtypes of rosacea: erythematotelangiectatic, papulopustular, phymatous, and ocular .
Morbihan disease is a rare complication of acne vulgaris or rosacea with histopathologic features of granulomatous rosacea. It is considered a part of the spectrum of rosacea and a variant [3, 2], which is characterized by non-caseating epithelioid granulomas in biopsy specimens. Clinically, Morbihan disease consists of solid facial edema that may occur in any stage of the disease, which includes relatively mild rosacea and the absence of any other symptoms of the disease [4, 5, 6, 7]. In 1957, the disease was first reported as a distinct entity by Robert Degos who described a chronic persistent erythema and edema of the upper one-half of the face, with accentuation in the periorbital tissues, forehead, glabella, nose, and cheeks. This refractory condition also has been referred to as lymphedematous rosacea or persistent solid facial edema. More severe cases of this entity have presented with soft tissue swelling and resultant distortion of the midline of the face and cheeks . The induration may be accompanied by erythema. Similar changes have been reported in Melkersson-Rosenthal syndrome.
The pathogenesis of Morbihan disease remains unclear. Histopathologic features include a granulomatous dermatitis with a periadnexal distribution that consists mainly of lymphocytes and histiocytes . Because this is a rare disease, the histopathologic features of the persistent lymphedema have only been documented in a few case reports, which include perifollicular fibrosis and perivascular and perifollicular infiltration of lymphocytes, histiocytes, and neutrophils in association with stromal edema. In a 24-year-old woman with lymph vessel dysplasia and progressive edema of her legs since her second year or life, a diagnosis of Morbihan disease was made when she also developed progressive facial edema . The clinical and histopathologic findings were consistent with rosacea and lymph vessel dysplasia. In another case, the authors postulated that the marked perilymphatic granulomas and intralymphatic histiocytosis that were observed in the biopsy specimen might account for the persistent lymphedema as a consequence of lymphatic obstruction . Others have hypothesized that the chronic inflammatory process, which accompanies the disease, leads to the destruction of supporting connective tissue that surrounds dermal lymphatics, in particular elastin around dermal vessels. Over time, there may be loss of vessel wall integrity with resultant transudation of fluids and subsequent development of lymphedema [6, 7, 10].
Owing to the clinical appearance of the entity and its histopathologic findings, it is also crucial to exclude other conditions, such as orofacial granulomatosis, sarcoidosis, Hansen disease, lupus vulgaris, cutaneous leishmaniasis, pseudolymphoma, foreign-body granuloma, granuloma faciale, and discoid lupus erythematosis . In our patient with an occupational history of construction and exposure to woods, dusts, glues, and other work-related materials, extensive patch tests were performed to eliminate an allergic contact dermatitis. With negative results on the standard as well as specialized panels and with histopathologic confirmation of perifollicular sarcoidal granulatomata, a diagnosis of Morbihan disease was made.
The cutaneous lesions of Morbihan disease persist indefinitely with no tendency to spontaneous involution without treatment. Because the persistent facial edema may lead to visual impairment in severe cases , it is critical to control disease activity. Therapeutic regimens, such as X-irradiation, lymphatic massage, interferon gamma injections, antihistamines, and high-dose antibiotics often are unsatisfactory . Oral prednisolone and oral metronidazole have been reported as successful treatments, whereas thalidomide has apparently failed to control the disease. Promising results in some patients have been achieved by a systemic combination therapy of isotretinoin (0.1 to 0.2 mg/kg/day) with either ketotifen (1-2 mg/day) [14, 15] or clofazimine . Excision of redundant edematous tissue may be an alternative and one group has reported good cosmetic results with CO2 laser blepharoplasty . This treatment produced marked improvement of visual impairment and no recurrence during a six-month follow-up in a patient with right lower and upper eyelid swelling and erythema. Our patient failed a course of minocycline, but was started on isotretinoin initially at 20 mg daily, with subsequent gradual increases to 30 mg daily, 60 mg daily, and finally 80 mg daily. After approximately four months of therapy, he has experienced improvement, with decreases in both erythema and edema of the affected regions of the face.
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