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The Journal of Clinical and Aesthetic Dermatology logoLink to The Journal of Clinical and Aesthetic Dermatology
. 2023 Oct;16(10):22–30.

Diagnosis and Treatment of Morbihan’s Disease: A Practical Approach based on Review of the Literature

Mireille MD van der Linden 1, Bernd WM Arents 2, Esther J van Zuuren 3,✉
PMCID: PMC10617896  PMID: 37915334

Abstract

Background

Morbihan’s disease (MD), also known as solid persistent facial edema, solid facial lymphedema or rosacea lymphedema, is a rare condition.

Objective

Despite existing case reports and literature reviews, clinical guidance for diagnosis and management is lacking. This review aims to provide comprehensive information on the etiology, differential diagnoses, diagnostics, and management of MD.

Methods

PubMed was searched up to April 2023 for relevant studies on MD with no language restriction. Furthermore, references were checked of found reports.

Results

A comprehensive overview of the literature and clinical guidance for MD. We found 95 studies involving 166 patients (118 male, 46 female and 2 gender unreported) evaluating management options, categorized into: isotretinoin (16 studies), isotretinoin plus antihistamines (8), isotretinoin plus corticosteroids (8), antibiotics (13), antibiotics plus corticosteroids (7), surgical debulking (10) and miscellaneous/combination treatments (43). Some studies contributed to two categories. Treatment with isotretinoin as monotherapy or combined with antihistamines, doxycycline or minocycline as well as surgical procedures demonstrated mostly satisfactory results, although recurrences were common. Longer treatment duration, of at least 6 to 12 months, is recommended for pharmacological treatments. Adding systemic or intralesional corticosteroids to previous treatments may be beneficial. Manual lymph drainage seems to contribute to satisfying result.

Limitations

This is not a systematic review and randomized controlled trials are lacking.

Conclusion

Diagnosis of MD is based on specific clinical features and excluding diseases with similar appearance. Prolonged treatment is often necessary to obtain satisfactory results, which might be limited to a partial and/or temporary response.

Keywords: Morbihan, solid facial lymphedema, solid persistent facial edema

Morbihan’s disease, also known as solid persistent facial edema, solid facial lymphedema or rosacea lymphedema, is a rare condition which may develop spontaneously without a tendency to resolve.1–4 First described by French dermatologist Robert Degos in 1957, Morbihan's disease is named after the region in Bretagne where it was first observed.1–6 The disease causes disfigurement of the face and has therefore an enormous impact on patients’ quality of life, self-esteem and psychosocial well-being.3 Currently, there are no data on the incidence and prevalence, but it is considered a rare disease. Most cases have been described in White males and females of middle age, but the disease is also described in people with skin of color, as well as in younger and older individuals.1,5 Diagnosing and treating Morbihan's disease can be challenging, and there are currently no clinical practice guidelines on its management. While several case reports and literature reviews have been published, there is a need for a comprehensive and practical review that can assist clinicians in diagnosing and managing patients presenting with this condition. This narrative review aims to provide an overview of Morbihan's disease, including its clinical features, pathogenesis, differential diagnosis, and management options. It highlights the need for a personalized approach and treatment plans that consider the individual patient's medical history, severity of symptoms, and response to therapy. By improving our understanding of this condition, we can provide better care to patients with Morbihan's disease and alleviate their suffering.

CLINICAL PRESENTATION

The clinical presentation of Morbihan’s disease is characterized by persistent erythema and firm non-pitting swelling involving the central and upper parts of the face. The swelling is typically symmetrical and bilaterally distributed (Figure 1)1,4,5,7 but can also be asymmetrical (Figure 1D and 1E), and may be accompanied by telangiectasias and papules. (Figure 1F).5 Patients may experience symptoms ranging from mild to very debilitating, with persistent erythema and swelling that can result in significant facial disfigurement. In particular festoons under the eyes and ptosis may occur, leading to narrowing of the visual field.1-5,7

FIGURE 1.

FIGURE 1.

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Various presentations of Morbihan’s disease (consent was given to use unedited images). A) 68-year-old woman with severe erythema and solid edema of the periocular region and cheeks, more pronounced on the left side, with lower lid swelling and festoon and an erythematous plaque on the forehead. B) 49-year-old man with solid periocular edema, more pronounced round the right eye, with slight erythema. C) 59-year-old man showing solid facial edema of the cheeks, glabella, around the eyes and slight facial erythema. D) 72-year-old man with slight erythema and severe solid edema of the right periocular region and cheek with lower lid swelling and festoon. E) 37-year-old woman with erythema and severe solid edema of the left periocular region and cheek, with a few papules on forehead, cheeks and chin. F) The same woman as in Figure 1E, but six years later: recurrence demonstrates erythema and swelling of the cheeks and forehead with papules on forehead and cheeks. Repeated treatments with minocycline 100-200mg for more than three months in combination with oral corticosteroids (tapered) usually resulted in remissions. No recurrences for three years.

It is important to note that the swelling itself is usually not painful or itchy. A history of rosacea is often supportive of the diagnosis of Morbihan’s disease as the condition is often regarded as a complication of rosacea, due to chronic inflammation and vasodilatation.1,4,5 However, it can also occur in the context of acne1,5,8–11 or as a distinct entity.2–4

PATHOPHYSIOLOGY

The pathogenesis and pathophysiology of Morbihan’s disease are not yet fully elucidated. The quintessential feature of the condition is persistent edema of the upper part of the face due to impaired lymphatic drainage, which can result from lymphatic obstruction and/or damage of blood and lymphatic vessels as a consequence of chronic inflammation.1,5 In several case studies, an increased number of mast cells has been reported and it is suggested that even if their amount is within normal range, mast cells’ cytokines may be involved in the pathogenesis of Morbihan’s disease.2,4,7

Histopathology of affected facial skin shows inflammatory features consistent with rosacea, including perivascular and periadnexal infiltrates of lymphocytes, histiocytes and sometimes neutrophiles.1,7 Most characteristic are dermal edema, dilated lymphatic vessels and the presence of clusters of mast cells (Figure 2).1,7 Although the histopathology is nonspecific, the diagnosis of Morbihan’s disease should be reconsidered in the absence of edema and dilated lymphatic vessels.7 Immunohistochemical staining with D2-40 and CD31 of histologic material is recommended to confirm the presence of lymphatic involvement. Demodex, sebaceous gland hyperplasia, mild perifollicular fibrosis, elastosis, clusters of mast cells and sometimes perilymphatic or intralymphatic epithelioid cell granulomas can also be found (Figure 2).2,6,7 The presence of epithelioid cell granulomas, which could be a possible cause of lymphatic obstruction and vessel wall damage, and clusters of mast cells, are indicative for the diagnosis.6,7 Absence of granulomas can be explained by either an early stage of the disease or by insufficient depth of the biopsy.6,7 Likewise, presence of epithelioid cell granulomas and increased number of mast cells might correlate with the stage of the condition.6 Biopsies are often taken after (unsuccessful) prior treatment, resulting in a less specific histopathology, further complicating the diagnosis.1

FIGURE 2.

FIGURE 2.

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A) skin biopsy with the characteristic histology of M. Morbihan (Hematoxylin-Eosin (HE) stained) with mild perifollicular fibrosis and perifollicular lymphohistiocytic inflammatory infiltrate (1), dilated lymphatic vessels (2) and intravascular epithelioid cell granulomas (3). 2B and 2C illustrate an intravascular granuloma: in B, the endothelium of an lymph vessel in the deep dermis is stained by immunohistochemistry using the marker D2-40, in C, the histiocytes of the intravascular epithelioid cell granuloma are stained by an anti-CD68 marker.

DIAGNOSIS

Diagnosis is usually based on the characteristic clinical presentation while ruling out other diseases with similar appearances such as seen in thyroid disease, angioedema and Melkersson-Rosenthal syndrome.3,4,12

To effectively exclude other diseases, a thorough clinical history is necessary, which should include inquiries about signs and symptoms of rosacea and any systemic complaints (Table 1). A complete physical examination is also essential, with particular attention given to inspecting the lips and tongue. Furthermore, histopathology of affected skin with specific staining, and laboratory tests are recommended. In some cases allergy tests, an X-thorax, or more advanced investigations such as magnetic resonance imaging (MRI), may be warranted (Table 1).4,12,13

TABLE 1.

Diagnostics

Recommended workup
History Besides a general history, specifically ask about symptoms of rosacea, acne, systemic complaints, allergic diseases/reactions, and facial neuro-sensations/neuropathy. Check medication use.
Physical examination Determine the location and symmetry, check the consistency of the edema (soft or firm non-pitting), inspect the facial skin and contour for other efflorescences, investigate the lips and tongue.
Histology Take a deep 3mm punch biopsy of a representative part of the affected skin, and order additional staining for mast cells, D2-40 and CD31.
To exclude differential diagnoses*
Melkersson-Rosenthal syndrome History, physical examination of skin, lips (edematous changes) and tongue (textural changes, fissures, geographic tongue).
Thyroid diseases Serum thyroid stimulating hormone (TSH).
Angio-edema History, angiotensin-converting enzyme (ACE), C1 esterase inhibitor (C1-INH), complement component 1q (C1q) and C4.
Dermatomyositis History, physical examination of the skin, creatine kinase (CK).
Systemic lupus erythematosus History, physical examination of the skin, histology, and anti nuclear antibody (ANA).
Sarcoidosis History, physical examination of the skin, histology, serum angiotensin-converting enzyme (ACE).
Chronic contact dermatitis History, physical examination of the skin, histology, patch testing including open patch tests with readings after 30 minutes (fragrances and preservatives used in cosmetics).
Chronic actinic dermatitis History, physical examination of the skin, histology.
Neoplastic diseases of the head History, physical examination (asymmetric presentation), histology, CT/MRI.
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*In case of suspicion of one of the differential diagnosis, expand diagnostics or consider referral.

CD31, marker that specifically stains blood and lymph vessels; CT, Computed Tomography; D2-40, marker that specifically stains lymphatic endothelial cells; MRI, Magnetic Resonance Imaging.

DIFFERENTIAL DIAGNOSIS

Differential diagnoses include a range of conditions such as Melkersson-Rosenthal syndrome (MRS), thyroid disease, angioedema, dermatomyositis, systemic lupus erythematosus, sarcoidosis, chronic contact dermatitis, chronic actinic dermatitis, and neoplastic diseases (Table 1).3,4,12,13

Distinguishing between MRS and Morbihan's disease can be challenging due to their shared clinical and histopathological similarities, including orofacial swelling, lymphatic vessel dilation, and non-caseating granulomas.7,13,14 While the complete triad of MRS includes orofacial granulomatosis, facial neuropathy, and a geographic tongue, the presentation may be incomplete. The first symptom of MRS is often orofacial swelling, especially in monosymptomatic cases, but may involve the mid and upper face, similar to Morbihan’s disease.13,15–18 Clinical history and careful physical examination can exclude MRS, as facial neuropathy and textural changes of the tongue (geographic tongue or lingua plicata), both indicative of MRS, are absent in Morbihan’s disease.

Serum thyroid stimulating hormone (TSH) testing is indicated to exclude thyroid disease, which can also cause periorbital edema.1,5 Facial swelling can also be the result of an adverse drug reaction, due to e.g., angiotensin-converting enzyme (ACE) inhibitors, non-steroidal anti-inflammatory drugs (NSAIDs), and some non-benzodiazepine hypnotics.19–21 Unlike the swelling in Morbihan’s disease, this swelling is usually transient and resolves within 48 hours. Hereditary and non-hereditary angioedema can be ruled out by patient’s history and laboratory tests such as C1 esterase inhibitor (C1-INH), component 1q (C1q) and C4 complement.

Histopathology and laboratory tests, including antinuclear antibody (ANA) and creatine kinase (CK), can help exclude systemic lupus erythematosus and dermatomyositis.7

Sarcoidosis should be considered in case of systemic complaints and symptoms. Histology and laboratory testing of ACE and an X-thorax are recommended when sarcoidosis is suspected.

Allergological tests can exclude chronic contact dermatitis. Patch tests including open patch tests (fragrances and preservatives used in cosmetics) with additional readings after 30 minutes are advised, as both hidden immunologic contact urticaria and chronic contact dermatitis may contribute to impaired lymphatic drainage, and thus play a role in the persisting character or recurrence of Morbihan’s disease.12

History and histology might point at chronic actinic dermatitis. Presentation of asymmetric features can be suggestive of underlying neoplastic disease and more advanced investigations, e.g., computed tomography (CT) and magnetic resonance imaging (MRI) are necessary to eliminate these underlying causes.4

TREATMENT

There are no registered and approved treatments for Morbihan’s disease. However, there are various treatment options that have been described in the literature, but the response is not always complete and recurrence can happen.1,5,22,23 Although we did not conduct a systematic literature search, we thoroughly searched PubMed and existing reviews for case reports or case series regarding treatment of Morbihan’s disease. Additionally, we meticulously screened the references of found articles for any additional relevant cases. The comprehensive search yielded a total of 95 studies encompassing 166 patients as outlined in Supporting information Table S1).1–9,11,13,14,18,22–103 Of the 166 patients, 118 were male, 46 were female and the gender of two patients was not reported. The mean age of the patients was 48.3 years with an age range of 14 to 88 years. Of the cases studied, 66 patients had a prior history of rosacea, 32 had a history of acne, one had extrafacial lupus miliaris disseminatus faciei and of the remainder (67/166) no pre-existing skin disease was reported. A variety of treatment options were described with varying rates of success. It should be noted that follow-up details were often lacking, but several studies did report that in the event of a recurrence, repeating the same treatment led to successful outcomes.

We categorized the reports per treatment modality: isotretinoin, isotretinoin combined with antihistamines, isotretinoin plus corticosteroids, antibiotics, antibiotics combined with corticosteroids, procedural treatments, and the remainder group featuring different combinations of treatments or differing treatment strategies among patients in case series (see Supporting information Table S1 on the online version of this article for details of the studies). In numerous reports, particularly those documenting results of surgical procedures, previous therapies often involving (alternative) anti-inflammatory drugs had failed.

PHARMACOLOGICAL TREATMENT.

Isotretinoin. A total of 16 studies involving 23 patients (15 male, 7 female and 1 unreported gender) described the effects of isotretinoin.3,4,7,22,24–35 Of the 23 patients, five had acne and five had rosacea. Good to complete response with isotretinoin therapy was established in 16 out of 23 patients,3,4,24,26–30,32,33,35 while a partial response was observed in two patients.25,30 The prescribed dosages ranged from 10 to 80mg isotretinoin per day with approximately 40mg isotretinoin being the most common. Figure 3 shows an example of a woman successfully treated with isotretinoin. A longer treatment duration (of at least 6–12 months) seems to be associated with more satisfying results. Prolonged treatment with isotretinoin does not always lead to a satisfactory outcome and can be associated with adverse events.5,22,31,34

FIGURE 3.

FIGURE 3.

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Morbihan’s disease over a period of eight years: before and after (consent was given to use unedited images). A) 46-year-old woman with solid edema of periocular regions and cheeks, with slight erythema. B) the same woman seven years later during a recurrence. C) one year later after successful treatment with isotretinoin (20–50mg) for more than six months. No recurrence after six years.

Isotretinoin combined with antihistamines. In eight studies with 11 patients (8 male, 2 female and 1 unreported gender) the combination of isotretinoin with an antihistamine was investigated.11,36–42 All patients achieved a good to complete response. The daily dosage of isotretinoin ranged from 10mg per day to 0.7mg/kg per day. Ketotifen 1–2mg per day was the antihistamine of choice in six studies.36–41 Desloratadine 5mg per day was the preferred antihistamine in one study. Additionally, in one study the choice of antihistamine was not specified.11 Treatment duration varied from 3 to 16 months. Of note are the excellent results with low dose isotretinoin (10–20mg per day) with an antihistamine in 8 of the 11 patients.11,36,40–42 Low-dose and ultra-low-dose isotretinoin (10–20mg per day) are associated with mild side effects, which is a great advantage.27,33,42 Combination with ketotifen, an antihistamine and mast cell stabilizer, or desloratadine is recommended because of a possible synergistic effect based on anti-inflammatory properties of both isotretinoin and antihistamines.37,38,42,104

Isotretinoin combined with corticosteroids. The efficacy of a combination therapy consisting of isotretinoin and corticosteroids was investigated in eight studies involving eight patients, of which seven were male and one was female.1,11,43–47,93 Among them, six patients had acne (aged 18–38 years) and two had rosacea (both 61 years old). Prednisone was used in six cases at a daily dose of 20–40mg, deflazacort was used in one case at a daily dose of 30mg, and intralesional corticosteroids were used in one case (corticosteroid not specified). Treatment duration ranged from 2 to 8 months, with six patients showing good to excellent improvement.11,43–47 However, three studies reported recurrence of symptoms after the discontinuation of treatment.43,44,47 Cabral et al suggest that tapering of corticosteroids should be done gradually and slowly to prevent adverse effects.43 While corticosteroids are associated with well-known side effects, they can be considered as an adjunctive treatment option in some cases.

Antibiotics. Tetracyclines are well-known for their anti-inflammatory properties.105 Treatment response after minocycline and doxycycline may be correlated with the extent of mast-cell infiltration.2,50,52 However, mast cell infiltration is not observed in all cases of Morbihan’s disease.1,2,48 Thirteen studies comprising 20 patients (14 male and 6 female) evaluated the effects of predominantly tetracyclines.2,7,23,48–56,64 In 10 patients a history of rosacea was reported and in one acneiform eruptions in the perioral region. Seven of the eight patients treated with doxycycline 200mg per day for 30 days to 12 months showed marked improvement to complete response.7,48,51,52,64 In one study an initial dosage of 200mg doxycycline for two months, followed by doxycycline 40mg modified release for six months, proved to be successful.48 Similarly, four of the six patients treated with minocycline 100mg per day for four weeks to seven months demonstrated marked improvement.2,7,50,52 One patient switched after seven months from minocycline 100mg per day to doxycycline 200mg per day for another seven months because of side effects.52 It is important to take into account that minocycline can have rare but serious side effects such as drug induced lupus erythematosus, autoimmune hepatitis and hyperpigmentation (with prolonged use).106,107 Two patients only showed partial improvement after minocycline which might be due to a short treatment duration (1 and 2 months).53,56 Varying dosages of tetracycline were used in five patients with only one patient showing improvement,23,49,54 while one patient was treated with fleroxacin 100mg per day for two weeks and showed marked improvement in edema after several days.55

Antibiotics combined with corticosteroids. In seven studies comprising of eight patients (7 male and 1 female; 5 with rosacea and 3 with acne) antibiotics were combined with prednisone or prednisolone.6,57–62 The antibiotic regimens involved mainly doxycycline (200mg/day) or minocycline (100mg/day), whereas the dosages prednisone and prednisolone ranged from 10 to 60mg per day which was tapered over time. Overall, this combined treatment approach resulted in significant improvement in most cases, although mostly with recurrences after treatment cessation. Treatment duration was either not reported or relatively short (1 to 4 months) which may have contributed to the recurrences. Nevertheless, as stated before, recurrences are a common occurrence in Morbihan’s disease.

Procedural treatment. In cases where Morbihan’s disease persists despite pharmacological interventions and negatively impacts a patient’s quality of life or leads to visual impairment, surgical procedures, including blepharoplasty, lymphaticovenous anastomosis and debulking, may be considered.5,64,65,84,108 Authors of 10 studies including 27 patients (20 male and 7 female) have reported favorable outcomes of surgical debulking, however recurrences were frequently observed.5,7,14,63–68,75 Notably, successful outcomes of eyelid reduction surgery seem more likely in cases where histology shows no massive mast cell infiltration.85 Combining eyelid reduction surgery with intra-lesional corticosteroids may improve outcomes and provide more satisfactory results.5,63,64,75,85,94,96,100

Combination of pharmacological, procedural and/or adjunctive treatments.This is the most extensive group comprising of 43 studies including 93 patients (65 male and 28 female) highlighting the challenges faced by physicians in finding a definitive cure or effective treatment for Morbihan’s disease.1,5,7–9,11,13,18,70–103 While some studies have reported on single interventions, the majority have reported combinations of treatments or sequential treatments, each with their own dosage regimens and treatment durations. Certain studies support the results outlined in the preceding sections, while others demonstrate synergistic effects of combining treatments. Due to the limited number of cases and reliance on single case reports, drawing firm conclusions based on these findings is not possible. However, we will summarize two treatments. Manual lymphatic drainage was used in 20 patients (in 5 following debulking surgery) of which 16 showed improvement.73,76,79,80–82,85,87,89,96,100 A single case report described excellent results with the biologic omalizumab, a monoclonal antibody specifically binding to free human immunoglobulin E (IgE), administered for seven months.84 The authors hypothesized that omalizumab could potentially stabilize the mast cells and therefore could be beneficial in Morbihan’s disease. Further information on these combinations of pharmacological, procedural and/or adjunctive treatments can be found in Supporting information Table S1.

Based on the findings of our review we provide a comprehensive overview of diagnostics, potential differential diagnoses, and a list of recommended treatments (Tables 1–2).

TABLE 2.

Recommended treatment options*

MEDICATION DOSE MINIMUM DURATION
Isotretinoin,1 combined with ketotifen or desloratadine 10–20mg respectively 2mg/day or 5mg/day 6–12 months (longer treatment duration more likely to be successful)
  Doxycycline1,2 200mg/day 4–6 months
  Minocycline1 100mg/day 4–6 months
Procedural interventions
  Surgical debulking: Eyelid reduction surgery which may be combined with intra-lesional steroid injections
  Eyelid reduction surgery, followed by manual lymphatic drainage
Adjunctive therapy
  Manual lymphatic drainage
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*Recommended, based on the findings of this review

1Adding systemic corticosteroids may be beneficial but slowly tapering is recommended to reduce the chance of recurrences

2After initially 200mg/day, tapering to 100mg and 40mg/day modified release doxycycline might be an option

DISCUSSION

Having reviewed the literature on the diagnostics and treatments of Morbihan’s disease, combined with decades of clinical expertise and treating these patients, this disease however remains enigmatic. Is it even a distinct disease? Breaking it down to its essentials, Morbihan’s disease is a presentation of an imbalance in upper-face fluid build-up and draining of that fluid. This leads to a distinct pattern of solid and persistent upper-face edema. The reasons for fluid build-up can be multiple. In Morbihan’s disease, this is likely due to an inflammatory process. Likewise, the inadequate draining of fluid can have various underlying mechanisms, e.g. lymphatic dysfunction or other processes like physical tissue blockage due to infiltration or (smooth) muscle dysfunction. The key issue seems that the edema is not transient for misunderstood reasons.

The differential diagnoses of Morbihan’s disease and its treating strategies address the two issues mentioned. One aspect is reduction of fluid build-up: excluding and address other etiologies, reducing inflammation, and address capillary leakage, both pharmacologically. The other one is the inadequate lymph flow: excluding other (mechanical) etiologies, and use procedural interventions removing affected tissue. Since Morbihan’s disease is disfiguring the face, treatment is aimed at improving without causing harm. The latter implies resorting to procedural interventions only after pharmacological interventions have failed.

A striking finding of our review was that 71 percent of the patients were male, higher than anticipated. Surprisingly only about 40 percent had rosacea, which was lower than our initial expectations, while 19 percent suffered from acne, which was higher than we expected. It is uncertain whether selection bias or reporting bias contributed to these findings.

Effective management of Morbihan's disease is essential, as the condition has the potential to considerably affect a patient's quality of life and cause severe symptoms. Given the lack of spontaneous remission, prompt and accurate diagnosis, which involves recognizing the clinical features and ruling out alternative diagnoses, is imperative. An in-depth understanding of the available treatments and their outcomes can aid in making an informed decision for the most appropriate course of treatment.

This review provides an overview of the treatments described in the literature and their respective efficacy, which can facilitate optimal treatment selection.

Based on the literature reviewed, we have attempted to provide recommendations for the management of Morbihan’s disease in clinical practice. While many case reports and case series involved a search for the therapy that ultimately resulted in a satisfactory outcome, several studies have demonstrated that certain treatments yielded satisfactory outcomes in a majority of patients. These treatments include isotretinoin, preferably in combination with antihistamines, as well as doxycycline for at least six months and preferably longer. Systemic or intralesional corticosteroids may be considered in some instances. In cases with severe disfigurement or vision loss, surgical interventions have shown promising results. Additionally, manual lymphatic drainage can be combined with all treatments and has been reported to have a beneficial effect in the majority of cases.

Limitations. The limitations of this review include the lack of a thorough systematic review, as the authors were aware that mainly case reports were available on this rare disease, and well-designed clinical intervention studies were lacking. However, according to our knowledge this is the most complete review to date with the largest number of studies regarding management of Morbihan’s disease. Furthermore, we searched for publications without language restriction and checked references of found articles for additional literature. Another limitation is that this review is based on the included case reports and case series, which come with very low certainty of benefit of the discussed treatments. The summary of the recommended treatments is therefore based on the number of case reports and case series that reported treatment results, and expert opinion.

The strength of this review is that it presents a comprehensive overview of the available literature on Morbihan’s disease, the possible etiology, the most common differential diagnoses (and how to exclude them), a diversity in visual representations of the disease, and a list of treatments that may be most successful. Such an overview and guidance were lacking, and needed addressing. Since Morbihan’s disease is a rare condition, less experienced dermatologists might find this review helpful when dealing with a presentation of upper-facial edema. Furthermore, this review was co-authored with a patient advocate, as to bare and keep in mind the devastating consequences of having upper-facial edema and Morbihan’s disease, and that patients suffering from it need the best care and outcome possible, based on the evidence that is available.

CONCLUSION

Diagnosis of Morbihan’s disease should be based on recognizing the specific clinical features and excluding diseases with similar appearance. Prolonged pharmacological treatment is often necessary to obtain satisfactory results, which in some cases might be limited to a partial and/or temporarily response.

ACKNOWLEDGEMENTS

The authors would like to thank the patients with Morbihan’s disease for giving their informed consent to use their unedited images in this manuscript. Furthermore we are grateful for Marcus V. Starink, dermatologist, and Lies H. Jaspars, pathologist, Amsterdam University Medical Center, Amsterdam, for their valuable histological expertise and assistance with Figure 2.

Supplementary Material

Table S1

Contributor Information

Mireille MD van der Linden, Dr. van der Linden is with the Department of Dermatology at Amsterdam University Medical Center in Amsterdam, Netherlands..

Bernd WM Arents, Mr. Arents is with Skin Netherlands in 's-Hertogenbosch, Netherlands..

Esther J. van Zuuren, Dr. van Zuuren is with the Department of Dermatology at Leiden University Medical Center in Leiden, Netherlands..

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Table S1
van_Zuuren_Supporting_Table_1.pdf (252.3KB, pdf)

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