Case for diagnosis. Eyelid edema and erythematous papules disseminated on the face

Abstract Lupus miliaris disseminatus faciei or acne agminata is a chronic inflammatory disorder of the skin, considered an intriguing entity due to its pathogenesis, which is still largely speculative. It has been linked to tuberculosis, sarcoidosis, rosacea, and other granulomatous diseases, but it is considered an independent entity.

Consensus on the therapeutic management of rosacea - Brazilian Society of Dermatology

Abstract Background: Rosacea is a chronic inflammatory disease of the skin, relatively more frequent in women over 30 with a low phototype and proven genetic predisposition. Although its etiology is unknown and possibly multifactorial, the immunological abnormality, associated with neurovascular dysregulation and triggering factors, are important elements in its pathophysiology, which lead to the main changes of inflammation, vasodilation, and angiogenesis that are responsible for the clinical manifestations. Despite the lack of cure, numerous therapeutic options are available for the different clinical presentations of the disease, with satisfactory responses. Objective: To reach a consensus, with recommendations from experts, on the therapeutic management of rosacea suitable to the Brazilian setting. Methods: The study was conducted by five specialized dermatologists from university centers, representatives of the different Brazilian regions, with experience in rosacea, who were appointed by the Brazilian Society of Dermatology. Based on the adapted DELPHI methodology, the experts contributed through an updated bibliographic review of the scientific evidence, combined with personal experiences. Results: The group of experts reached a consensus on the relevant aspects in the therapeutic management of rosacea, providing information on epidemiology, pathophysiology, triggering factors, clinical condition, classification, quality of life, and comorbidities. Consensus was defined as approval by at least 90% of the panel. Conclusion: Despite the impossibility of cure, there are several therapeutic alternatives specific to each patient that provide excellent results, with chances of total improvement and long periods of remission, promoting a positive impact on quality of life. This consensus provides detailed guidance for clinical practice and therapeutic decisions in rosacea.

Modern aspects of rosacea therapy

This work is aimed at studying the problems of timely diagnostics and therapy of various forms of rosacea, identifying the factors that influence the compliance, prognosis, and quality of life of the patients, as well as the stages of combination therapy. The efficiency of rosacea therapy is determined by the timely identification of patients, as well as the clinical variety of the disease. Complex therapy of rosacea includes identification of the precipitating factors, basic skincare, and the use of systemic and local pathogenetic preparations. The "Gold Standard" of topical rosacea therapy is the antimicrobial and antiprotozoal drug called metronidazole. An important role in disease therapy is played by active cooperation between the doctor and the patient. Comprehensiveness, timeliness, and rationality of rosacea therapy are defined not only by the mechanisms of the disease development but also by aggravating factors, the need for basic care and photosensitivity of the patients

Síndrome de Morbihan ­ relato atípico de uma doença pouco conhecida / Morbihan syndrome ­ an atypical report of a rare condition

A síndrome de Morbihan é uma doença rara, de fisiopatologia pouco conhecida, tipicamente caracterizada por edema e eritema facial, de aparecimento lento, simétrico, afetando terço superior da face. Este relato de caso se trata de um paciente com caso atípico de Síndrome de Morbihan, com acometimento assimétrico de face. Foram ressaltados aspectos clínicos e histopatológicos para diagnóstico dessa rara patologia, possíveis diagnósticos diferenciais e opções de tratamento. Também foi buscado difundir formas menos típicas desta doença.

Morbihan syndrome is a rare condition and its pathogenesis is not fully known. This entityh is characterized by facial edema and facial erythema, with a slow, symmetrical appearance, affecting the upper portion of the face. This case report is about a patient with an atypical case of Morbihan syndrome, with asymmetric facial involvement. Clinical and histopathological aspects were highlighted aiming the diagnosis of this rare pathology, as well as possible differential diagnoses and treatment options. It has also been aimed to call attention to less typical forms of this disease.

Tratamiento del acné en la adolescencia / Treatment of acne in adolescents

El acné es la enfermedad dermatológica más común de la adolescencia. Aunque casi todos los casos remiten alrededor de la tercera década de la vida, cuando este problema conlleva una carga emocional para el paciente se requiere indicar tratamiento. En esta actualización, los autores revisan los distintos grados de compromiso de esta patología, el tratami-ento y los niveles de evidencia que tiene cada uno de ellos, con el objetivo de facilitar a los médicos de atención primaria el manejo de los pacientes que presentan esta enfermedad. (AU)

Acne is the most common dermatological condition in adolescents. Even though almost all cases will resolve around the third decade of life, treatment is indicated when this health problem carries an emotional burden for the patient. In this update, the authors review the grades of involvement of the disease and the available treatments according to levels of evidence, with the aim of helping primary care physicians to manage the patients presenting this illness. (AU)

An unusual presentation of ocular rosacea / Uma apresentação incomum da rosácea ocular

ABSTRACT Rosacea is a chronic, progressive disease of unknown cause affecting the eye and the facial skin. Ocular rosacea is often underdiagnosed if the ophthalmologist does not inspect the patient's face adequately during the ocular examination. Severe ocular complications and blindness can occur if the treatment is delayed because of non-diagnosis of the rosacea. Here, we present a case of ocular rosacea in a 78-year-old Caucasian woman. Based on the ocular lesions, which preceded cutaneous involvement, she was misdiagnosed as having ocular cicatricial pemphigoid initially. This case emphasizes the difficulty in diagnosis when ocular findings precede those of skin manifestations, and rosacea should be kept in mind in the differential diagnosis of chronic cicatrizing conjunctivitis.

RESUMO A rosácea é uma doença crônica e progressiva de causa desconhecida que afeta o olho e a pele do rosto. A rosácea ocular é muitas vezes pouco diagnosticada se o oftalmologista não inspecionar adequadamente o rosto do paciente durante o exame ocular. Podem ocorrer complicações oculares graves e cegueira se o tratamento for adiado devido ao não diagnóstico da rosácea. Um caso de rosácea ocular em uma mulher caucasiana de 78 anos de idade é apresentado. Com base nas lesões oculares, que precederam o envolvimento cutâneo, ela foi mal diagnosticada como penfigóide cicatricial ocular inicialmente. Este caso enfatiza a dificuldade de diagnóstico quando os achados oculares precedem aqueles das manifestações da pele e um diagnóstico de rosácea deve ser mantido em mente no diagnóstico diferencial da conjuntivite cicatrizante crônica.

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)

Rosácea: puesta al día / Rosacea: update

Rosacea is a chronic inflammatory disease. Its frequency is higher in pale-skinned people and women over 30 years. Disease mechanisms include: abnormalities in innate immunity, inflammatory reactions to microorganisms, ultraviolet radiation damage, and vascular dysfunction. There are four clinical subtypes: erythematotelangiectatic, pustular papules, phymatous and ocular rosacea. Patients may present one or more characteristics of each subtypes. Injuries are classically located in midface area. Extreme temperatures, UV radiation, hot beberages, spicy foods, alcohol, exercise, topical irritants, psychological symptoms and drugs are associated to exacerbations. Clinical evaluation of the patient is usually enough for diagnosis. Nonpharmacologic interventions are essential for treatment. These include avoiding use of cosmetic, and triggers, skin care, and broad-spectrum sun protection. Patients with no response to general measures can respond to pharmacological agents. Topical metronidazole and azelaic acid are considered first-line treatments in mild to moderate disease. Oral tetracycline, have been used for many years for the treatment of papulopustular rosacea. Isotretinoin is useful in refractory disease. Treatment must be continous to maintain the response. (AU)

Ocular rosacea: a review / Rosácea ocular: revisão

Rosacea is a prevalent chronic cutaneous disorder with variable presentation and severity. Although considered a skin disease, rosacea may evolve the eyes in 58-72% of the patients, causing eyelid and ocular surface inflammation. About one third of the patients develop potentially sight-threatening corneal involvement. Untreated rosacea may cause varying degrees of ocular morbidity. The importance of early diagnosis and adequate treatment cannot be overemphasized. There is not yet a diagnostic test for rosacea. The diagnosis of ocular rosacea relies on observation of clinical features, which can be challenging in up to 90% of patients in whom accompanying roseatic skin changes may be subtle or inexistent. In this review, we describe the pathophysiologic mechanisms proposed in the literature, clinical features, diagnosis and management of ocular rosacea, as well as discuss the need for a diagnostic test for the disease.

A rosácea é uma condição cutânea crônica, que possui apresentações clínicas variáveis. Apesar de considerada uma doença dermatológica, os olhos podem ser acometidos em 58-72% dos casos, causando inflamação palpebral e da superfície ocular. Aproximadamente um terço dos pacientes desenvolve acometimento corneano, podendo causar baixa visual significativa. Diagnóstico precoce e tratamento adequado são de extrema importância, devido à significativa morbidade ocular que a doença pode causar. Não há, até o momento, um teste diagnóstico para rosácea. O diagnóstico da rosácea ocular depende da observação das manifestações clínicas, o que pode ser bastante desafiador em até 90% dos pacientes, em que os achados cutâneos são discretos ou inexistentes. Nesta revisão, descrevemos os mecanismos fisiopatológicos propostos na literatura, manifestações clínicas, diagnóstico e tratamento da rosácea ocular, assim como abordamos a necessidade de um teste diagnóstico.

Uso da capilaroscopia ungueal como método diagnóstico e prognóstico de rosácea / Nailfold capillaroscopy as a diagnostic and prognostic method in rosacea

FUNDAMENTOS: Não há um método adequado e fidedigno de avaliação e seguimento da severidade na rosácea. OBJETIVO: Determinar a importância da capilaroscopia periungueal como método diagnóstico e prognóstico em pacientes portadores de rosácea. MÉTODOS: Estudo transversal onde foram submetidos ao exame da capilaroscopia periungueal 8 pacientes com rosácea e 8 controles no período entre maio e julho de 2009. Foram coletados dados clínicos relacionados ao sexo, idade, fototipo, classificação da rosácea de acordo com a classificação de Plewig e Kligman e a classificação da National Rosacea Society. Adicionalmente, avaliamos o tempo de evolução da doença e tratamentos previamente utilizados. RESULTADOS: A grande maioria das pacientes avaliadas (6 das 8 pacientes) apresentavam rosácea grau I (vascular) ou eritêmato-teleangiectásica. A idade média de duração da rosácea foi de 5,96 anos, sendo que 87,5 por cento faziam tratamento com metronidazol tópico. Nenhum paciente tanto do grupo rosácea como controle demonstrou evidência de desvascularização ao exame capilaroscópico. CONCLUSÃO: A capilaroscopia periungueal apresenta um padrão inespecífico e não parece auxiliar no diagnóstico ou prognóstico da rosácea.

BACKGROUND: There is no appropriate and reliable method of evaluating and monitoring severity in rosacea. OBJECTIVE: To determine the importance of nailfold capillaroscopy as a diagnostic and prognostic method for patients with rosacea. METHODS: A cross-sectional study where eight patients with rosacea and 8 control subjects were submitted to nailfold capillaroscopy from May to July 2009. We collected clinical data related to gender, age, skin phototype, and rosacea stage according to Plewig and Kligman classification and the classification of the National Rosacea Society. Additionally, we evaluated the progression of the disease and treatment therapies previously used. RESULTS: The majority of the patients evaluated (6 out of 8 patients) had rosacea subtype I (vascular) or erythematotelangiectatic rosacea. The mean duration of the disorder was 5.96 years, and 87.5 percent of the patients were under treatment with topical metronidazole. Nailfold cappilaroscopy showed that evidence of devascularization was absent in both groups. CONCLUSION: Nailfold capillaroscopy presents a nonspecific pattern and does not seem to help in the diagnosis or prognosis of rosacea.

Oral Azithromycin for Treatment of Intractable Rosacea

Rosacea is a common chronic cutaneous disorder that primarily occurs on the convex surfaces of the central face and is often characterized by exacerbations and remissions. A case of a 52-yr-old woman visited our clinic in February 2008 complaining typical features of rosacea including multiple pinhead to rice-sized erythematous papules. We applied various conventional treatments including topical benzoyl peroxide and metronidazole as well as oral metronidazole, isotretinoin, and doxycycline. The lesions were not controlled but were rather aggravated by complications from these treatments. Therefore, we prescribed oral azithromycin, which has anti-inflammatory effects and reduces reactive oxygen species. Ten weeks after the administration of oral azithromycin, 500 mg per day for 2 weeks, the lesions had mostly disappeared and no specific side effects related to the azithromycin were noted. Oral azithromycin dosing 500 mg/day for 2 weeks is effective for treatment of intractable rosacea.

Rosácea / Rosacea

A rosácea é uma doença comum em adultos e tem uma apresentação muito variável. A doença com frequência produz eritema, pápulas, pústulas e edema na pele médio-facial.

Spontaneous corneal perforation in ocular rosacea

Rosacea is a dermatologic condition that affects the midfacial region. Ocular rosacea is most frequently diagnosed when cutaneous signs and symptoms are also present. Ocular manifestations are essentially confined to the eyelids and ocular surface. Ocular involvement ranges from minor irritation, dryness, and blurry vision to potentially severe ocular surface disruption including corneal ulcers, vascularization and rarely perforation. We present a 49'year-old Saudi Arabian female with the diagnosis of rosacea who presented with a peripheral corneal performation. The perforation was successfully managed by surgical repair, oral doxycycline and topical steroid. The final best corrected visual acuity was 20/30 after treatment. Early referral to an ophthalmologist and careful long-term followup are recommended

Enfermedad de morbihan / Morbihan´s disease

La enfermedad de Morbihan (morbus Morbihan) es una entidad poco frecuente, que se caracteriza por la aparición recurrente, a nivel del tercio medio y superior del rostro, de eritema y edema, que conducen a una induración desfigurante. Actualmente se plantea que la urticaria de contacto inmunológica desempeña un rol crucial en la patogenia. Presentamos un paciente de 65 años con enfermedad de Morbihan de 2 años de evolución.

Morbihan’ s disease (morbus Morbihan) is an infrecuent entity, characterized by the appearance of relapsing erithema and edema on the center and upper aspect of the face, leading to disfi guring induration.Nowadays, immunologic contact urticaria plays a crucial role in the pa-togenesis. We present a 65 year-old patient with Morbihan’ s disease, with its onset 2 years ago.

Actualización en rosácea / Update in rosacea

La rosácea es una condición crónica muy común en la práctica clínica diaria, principalmente en poblaciones de origen celta. Se caracteriza más frecuentemente por eritema centrofacial transitorio o persistente, vasos sanguíneos visibles, y a menudo, pápulas y pústulas. Fimas y compromiso ocular pueden presentarse. Basados en los hallazgos clínicos, se divide en cuatro subtipos: eritemato-telangiectásica, papulopustular, fimatosa y ocular. La causa es a la fecha desconocida y varias hipótesis plantean que incluyen el papel potencial de anomalías vasculares, degeneración de la matriz dérmica, factores ambientales y microorganismos tales como Demodex folliculorum y Helicobacter pylori. El tratamiento dependerá del subtipo clínico. Se discuten las indicaciones y las alternativas terapéuticas, ya sean tópicas, orales, láser y luz.

Rosacea is a chronic condition very common in clinical practice mainly among celtic population. It is characterized by transient or persistent central facial erythema, visible blood vessels, and often papules and pustules. Phymas and ocular involvement can be found. Based on the physical findings, rosacea can be classified into 4 broad subtypes: erythematotelangiectatic, papulopustular, phymatous, and ocular. The cause is unknown and several hypotheses have been postulated and include potential roles for vascular abnormalities, dermal matrix degeneration, environmental factors, and microorganisms such as Demodex folliculorum and Helicobacter pylori. Treatment depends on the clinical subtype. Indications and different therapeutics oral, topical, laser and lights are discussed.