Enfermedad de Morbihan: exitoso tratamiento con isotretinoína oral / Morbihan disease: successful treatment with oral isotretinoin

Se presenta a un paciente de 18 años de edad, con antecedentes de hipotiroidismo y acné leve. Consulta por edema facial persistente, a predominio de la mitad superior del rostro, de 7 meses de evolución. El mismo es indoloro, con un leve eritema, principalmente en párpados, y con temperatura local conservada. Se le solicitan estudios complementarios y se lo trata con antihistamínicos sin mejoría. Se toma biopsia de piel y con el diagnóstico de enfermedad de Morbihan se inicia tratamiento con isotretinoína a dosis de 20 mg/día durante seis meses, con respuesta muy favorable (AU)

An 18-year-old male patient with hypothyroidism and mild acne is reported. He consults for a 7-month history of persistent facial edema, predominantly on the upper half of the face. Edema is painless, with eyelids mild erythema and local temperature preserved. Additional tests are requested and treatment with antihistamines is unsuccessful. Skin biopsy is undertaken with Morbihan disease diagnosis. Patient is treated with oral isotretinoin in a daily dose of 20 mg/day for six months, with significant clinical improvement (AU)

Morbihan Disease: A Rare Variant of Rosacea / 대한피부과학회지

Morbihan disease, also known as 'rosacea lymphedema' or 'solid persistent facial edema' was first reported by Degos, in 1957, for describing persistent lymphedema on the upper half of the face in association with rosacea. Although the pathogenesis of Morbihan disease is not established well, two hypotheses were proposed; firstly, the hypothesis of lymphatic obstruction by destruction of vessel supporting connective tissue (e.g. elastin), and secondly, the hypothesis of mast cell-induced fibrosis. Herein, we report a 71-year-old man who had 9-months history of persistent bilateral edema of the eyelids. The histopathologic exam from edematous eyelid revealed marked dermal edema, dilated vascular structure with perivascular mixed cellular inflammatory infiltration. Moreover, focal granulomatous infiltration adjacent to a vascular structure and numerous mast cells were also identified. This case is the first reported case of Morbihan disease in Korean dermatological literature. Furthermore, the histopathologic findings of this case support both of the two hypotheses for development of Morbihan disease.