Morbihan disease: successful treatment with slow‐releasing doxycycline monohydrate

Chaidemenos, Georgios; Apalla, Zoe; Sidiropoulos, Theodoros

doi:10.1111/jdv.14521

Cited by 5 publications

(4 citation statements)

References 7 publications

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“…However, in the case of our female patient, thalidomide showed poor efficacy and was subsequently discontinued. Chaidemenos et al (5) recommended that long-term Minocycline monotherapy may be appropriate when histology sections reveal significant mastocyte infiltration. They observed that doxycycline administered in doses of 200 mg/day for 4-12 months in five patients was effective, but short-duration treatment was irreflective whether mastocyte infiltrated or not.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Morbihan disease treated with tofacitinib successfully

Chi

Cai

2023

Front. Immunol.

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IntroductionTo date, there is no standard treatment for Morbihan disease. Several studies have reported that Morbihan disease responds well to systemic corticosteroids (prednisone and prednisolone), systemic antibiotics (tetracyclines), antihistamines (ketotifen) and surgical therapy (Lymphaticovenous anastomosis). To our knowledge, Tofacitinib, as a Janus-activated kinase (JAK) inhibitor, plays a vital role in the treatment of inflammatory and autoimmune disorders. Therefore, Tofacitinib may be a promising medical option for patients with Morbihan disease.Case PresentationThe first case involves a 43-year-old Chinese man who presented a 12-month history of progressive painless swelling of the left upper eyelid. According to the skin biopsy, perivascular dermal edema with dilatation of lymphatic vessels and telangiectasia was observed, accompanied by mixed lymphocyte infiltrate, including histiocytes, plasma cells, and a few eosinophils. The second case involves a Chinese female patient who presented a 2-year history of progressive left-sided facial edema, which was eventually diagnosed as Morbihan disease. The skin biopsy revealed lymphocyte infiltration in the superficial vessels of dermis and some accessories. Based on patients’ clinical presentation, skin biopsy results, and exclusion of differential diagnoses such as systemic lupus erythematosus (SLE), they were diagnosed with Morbihan disease. They were both treated with Tofacitinib (5mg, po twice daily).OutcomesPatient 1 underwent a trial of Tofacitinib at a dosage of 5 mg twice daily for one month, with notable improvement. His edema and erythema present on the left face were alleviated. Patient 1 reduced the dosage of Tofacitinib by half (5mg, once daily) and continued using it for 5 months. During the 6-month follow-up, the facial erythema in the patient subsided, and there was a noticeable improvement in the swelling of the left eyelid compared to before. Patient 2, her lesions gradually improved after one-week treatment. She received a one-month treatment of Tofacitinib, and during the subsequent six-month follow-up, there was no evidence of eruption recurrence.ConclusionWe present the first cases of two patients receiving short-term Tofacitinib as therapy for Morbihan disease and retrieving huge succession. Tofacitinib may be a promising oral alternative for patients with Morbihan disease. However, its safety and efficacy require further assessment through clinical trials.

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Section: Discussionmentioning

confidence: 99%

Case Report: Morbihan disease treated with tofacitinib successfully

Chi

Cai

2023

Front. Immunol.

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“…As características histopatológicas do linfedema persistente só foram documentadas em alguns relatos de casos, que incluem fibrose perifolicular e infiltrado perivascular e perifolicular de linfócitos, histiocitos e neutrófilos em terreno edematoso. 16 Chaidemenos et al 17 Segundo Carney et al, 26 o uso de corticosteroides sistêmicos não foi satisfatório: a prednisolona 50 mg/dia por um mês foi ineficaz, prednisolona 20 mg/dia (duração não disponível) mostrou apenas efeito transitório e um curso curto de corticosteroide não forneceu nenhuma melhoria.…”

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“…Chaidemenos et al17 utilizaram doxiciclina 200 mg/dia por dois meses com melhora clínica do paciente, com posterior dose de manutenção por 6 meses com doxiciclina 40 mg/ dia. Após 51 meses da finalização do tratamento, o paciente mantinha remissão completa do quadro.Okubo et al 25 descreveram quatro casos clínicos de Síndrome de Morbihan.…”

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Síndrome de Morbihan: relato de caso e revisão bibliográfica

et al. 2018

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Síndrome de Morbihan é uma rara entidade, mais comum na terceira ou quarta década de vida. Afeta principalmente caucasianos adultos de ambos os sexos, apresentando incidência maior nas mulheres. É considerada uma forma de rosácea tendo como sinônimos linfedema rosáceo e edema facial persistente sólido. Sua patogênese não é completamente conhecida. Clinicamente caracterizada pelo aparecimento lento de eritema e edema sólido nos dois terços superiores da face, com acentuação na região periorbital, frontal, glabelar, nasal e bucinadora. Neste relato, apresentamos um caso de uma paciente do sexo feminino com linfedema de face, com hipótese diagnóstica inicial de Síndrome Linfocitária de Jessner. Após acompanhamento clínico e exame anatomopatológico confirmou-se o diagnóstico de Síndrome de Morbihan. O tratamento proposto foi doxiciclina em monoterapia com obtenção de sucesso terapêutico.

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“…Oral isotretinoin, antihistamines, and tetracycline, associated with facial massage to improve drainage, are the most useful reported treatments. 2,4,[6][7][8][9] We describe the case of a 28-year-old man, who presented permanent severe edema and erythema of the periorbital region, nose, and cheeks, more evident on the right side of the face, since 7 years (Figure 1A). The patient has been treated for years with topical medications for seborrheic dermatitis without clinical benefit.…”

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Usefulness of in vivo reflectance confocal microscopy in Morbihan syndrome

Rizzo

Fiorani

Lazzeri

et al. 2021

Skin Research and Technology

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Morbihan syndrome is a rare dermatological condition characterized by chronic persistent erythema and firm edema of the face accompanied by telangiectasia, papules, or pustules. [1][2][3] It is considered a complication of acne or rosacea.Lesions do not regress spontaneously without treatment, causing permanent swelling, distortion of facial contours, and visual impairment in severe cases. 3 The pathogenesis is not yet well known; however, according to most authors, a chronic cutaneous disorder, like rosacea or acne, can cause vascular dysregulation, fibrosis, and obstruction of lymphatic vessels. 1,4 A biopsy should be performed to rule out other conditions as sarcoidosis, Hansen's disease, systemic lupus erythematosus, cutaneous leishmaniasis, facial granuloma, and Melkersson-Rosenthal syndrome. 2-5 Histopathological examination

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Morbihan disease: successful treatment with slow‐releasing doxycycline monohydrate

Cited by 5 publications

References 7 publications

Case Report: Morbihan disease treated with tofacitinib successfully

Case Report: Morbihan disease treated with tofacitinib successfully

Síndrome de Morbihan: relato de caso e revisão bibliográfica

Usefulness of in vivo reflectance confocal microscopy in Morbihan syndrome

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