Efficacy of long-term intralesional triamcinolone in Morbihan's disease and its possible association with mast cell infiltration

Abstract

Morbihan's disease is characterized by chronic persistent facial edema of the upper half of the face, absence of typical diagnostic findings, and refractoriness to treatment. A 44‐year‐old man was diagnosed with Morbihan's disease based on clinical signs and histopathology, which showed dermal edema in upper dermis, discrete lymphocytic infiltrate without granulomatous reaction, and mast cell infiltration. After long‐term therapy with intralesional triamcinolone a remarkable objective and subjective clinical response was observed. Reported cases of Morbihan's disease are reviewed, with respect to their treatment and histopathological findings. Mast cell infiltration has been observed on histopathology in most patients who responded to intralesional triamcinolone, suggesting a possible marker of response. The long‐lasting response seen in our case indicates the efficacy of intralesional triamcinolone in this rare condition.

Morbihan Disease: A Rare Case of Periorbital Bilateral Edema with Histopathological Findings of Chronic Inflammation and Demodex Localization

“…Additionally, antihistamines and diuretics can provide symptomatic relief in some cases. Topical corticosteroids or calcineurin inhibitors can be used to manage localized swelling and inflammation of the facial skin [ 23 ]. In cases in which severe fibrosis and skin changes are present, surgeries such as blepharoplasty or laser therapy can be considered to improve the facial appearance and relieve pressure to the affected tissues [ 7 , 24 ].…”

An Update on the Etiopathogenesis and Management of Morbihan Disease: A Narrative Review

“…Long-term intralesional injection of triamcinolone (every 4 -6 weeks for 4 months) for resistant MD (26). Local lymphedema management as compression therapy, manual lymphatic drainage, and skin care (27,28).…”

“…Long-term intralesional injections of triamcinolone, administered every 4 -6 weeks for 4 months, have been recorded as an effective, safe, and minimally invasive treatment for resistant MD of the upper and lower eyelids, particularly when mast cell infiltration is present (26).…”

Enfermedad de Morbihan. Aspectos clínico-patológicos en 15 pacientes atendidos en el Centro Dermatológico ''Dr. Ladislao de la Pascua''

“…Algunos autores señalan que la histopatología encaja dentro del espectro del linfedema localizado. 9,10,[12][13][14][15] La inmunohistoquímica muestra sobreexpresión de D2-40 por el endotelio linfático lo que refleja el aumento de la linfangiogénesis como parte de la patología de la enfermedad. 15,16 Con respecto al tratamiento, en esta entidad constituye el problema principal, ya que no existe hasta la fecha una terapéutica eficaz.…”

“…También se han utilizado los antibióticos en combinación con triamcinolona intralesional. 13,14 La isotretinoína oral (Figura 4) ha sido uno de los medicamentos más utilizados en los últimos años, observándose periodos de remisión por algunos meses; 8 se ha utilizado en combinación con el ketotifeno y la desloratadina, por su interferencia con la degranulación de los mastocitos, sus propiedades antiinflamatorias y capacidad para disminuir la lipogénesis, de igual manera se ha combinado con deflazacort. 12,16 Algunos consideran a la terapia descongestiva completa (drenaje linfático manual, vendaje compresivo y ejercicios para mejorar el drenaje linfático) (Figura 5) como una opción terapéutica en aquellos pacientes que no se benefician o rechazan el tratamiento farmacológico.…”