Lupus miliaris disseminatus faciei: when a rare skin disease involves the eyelid / Lupus miliaris disseminatus faciei: quando uma rara dermatose acomete a pálpebra

ABSTRACT The authors present a case of lupus miliaris disseminatus faciei , a rare skin disease of unknown etiology, which may cause unaesthetic scarring due to its difficult treatment. The histopathological examination of epithelioid granulomas with caseating necrosis, together with the clinical features, are important for diagnosis and early treatment with better results. Despite difficult and unsatisfactory treatment, there are ongoing studies on therapy to improve aesthetic and social impairment. This case report describes an initial misdiagnosis delaying appropriate treatment, and highlights the value of physical examination and clinical judgment for another pathological examination, whenever necessary, aiming at better treatment outcomes in daily practice.

RESUMO Os autores apresentam um caso de lupus miliaris disseminatus faciei , uma dermatose rara, de etiologia desconhecida, que pode deixar cicatrizes não estéticas, pela dificuldade de tratamento. O exame histopatológico de granulomas compostos por células epitelioides, com necrose caseosa, e as características clínicas, são importantes para o diagnóstico e tratamento precoce, com melhores resultados. Apesar do tratamento difícil e insatisfatório, há estudos em andamento sobre terapias para melhorar o comprometimento estético e social. Este relato de caso descreve um diagnóstico inicial errôneo, que atrasou o tratamento adequado, e destaca o valor do exame físico e raciocínio clínico para solicitar outro exame anatomopatológico, quando necessário, de forma a obter melhores desfechos com o tratamento, na prática diária.

Demodex folliculorum infestations in common facial dermatoses: acne vulgaris, rosacea, seborrheic dermatitis

Abstract Background: Demodex mites are found on the skin of many healthy individuals. Demodex mites in high densities are considered to play a pathogenic role. Objective: To investigate the association between Demodex infestation and the three most common facial dermatoses: acne vulgaris, rosacea and seborrheic dermatitis. Methods: This prospective, observational case-control study included 127 patients (43 with acne vulgaris, 43 with rosacea and 41 with seborrheic dermatitis) and 77 healthy controls. The presence of demodicosis was evaluated by standardized skin surface biopsy in both the patient and control groups. Results: In terms of gender and age, no significant difference was found between the patients and controls (p > 0.05). Demodex infestation rates were significantly higher in patients than in controls (p = 0.001). Demodex infestation rates were significantly higher in the rosacea group than acne vulgaris and seborrheic dermatitis groups and controls (p = 0.001; p = 0.024; p = 0.001, respectively). Demodex infestation was found to be significantly higher in the acne vulgaris and seborrheic dermatitis groups than in controls (p = 0.001 and p = 0.001, respectively). No difference was observed between the acne vulgaris and seborrheic dermatitis groups in terms of demodicosis (p = 0.294). Study limitations: Small sample size is a limitation of the study. The lack of an objective scoring system in the diagnosis of Demodex infestation is another limitation. Conclusion: The findings of the present study emphasize that acne vulgaris, rosacea and seborrheic dermatitis are significantly associated with Demodex infestation. Standardized skin surface biopsy is a practical tool in the determination of Demodex infestation.

Rosacea fulminans: unusual clinical presentation of rosacea

Abstract Rosacea fulminans or pyoderma faciale is a rare cutaneous disorder that usually affects women usually between the ages of 15-46. The disease is characterized by sudden onset of papules, pustules, cysts, and painful coalescing nodules with red-cyanotic centrofacial erythema. Although its etiology remains unknown, hormonal, immunological, and vascular factors have been reported. Early diagnosis and prompt treatment should minimize unsightly scars. We report a case of a 33-year-old female patient treated with traditional doses of doxycycline, with improvement of the lesions and regression of the condition in two months.

Morbihan syndrome: a case report and literature review

Abstract Morbihan syndrome is a rare entity that more commonly affects women in the third or fourth decade of life. It is considered a special form of rosacea and its pathogenesis is not fully known. It is clinically characterized by the slow appearance of erythema and solid edemas on the upper portion of the face, with accentuation in the periorbital region, forehead, glabella, nose, and cheeks. We report the case of a patient presented with edema on the upper eyelid for a year. These findings suggested the diagnosis of Morbihan syndrome. We aim to report a rare, particularly refractory and chronic form of rosacea, which has received little attention in the literature.

Increased Expression of Cathelicidin by Direct Activation of Protease-Activated Receptor 2: Possible Implications on the Pathogenesis of Rosacea

PURPOSE: Recent findings of increased cathelicidin protein and its proteolytic fragments in rosacea suggest a pathogenic role for cathelicidin in this disease. The relationship between cathelicidin and protease-activated receptor 2 (PAR-2) is therefore of interest, as PAR-2, expressed principally in keratinocytes, regulates pro-inflammatory cytokine expression in the skin. The purpose of this study was to determine the relationship between expression of PAR-2 and cathelicidin in rosacea and to test the effect of direct PAR-2 activation on cathelicidin expression in keratinocytes. MATERIALS AND METHODS: Samples from 40 patients with clinicopathologic diagnosis of rosacea and facial skin tissue samples from 20 patients with no specific findings or milium without inflammation were retrieved. Intensities of immunohistochemical staining for PAR-2 and cathelicidin were compared between normal and rosacea-affected skin tissues. Additionally, correlations between PAR-2 and cathelicidin staining intensities within rosacea patients were analyzed. In cultured keratinocytes, changes in PAR-2, cathelicidin, and vascular endothelial growth factor (VEGF) mRNA and protein were analyzed after treatment with PAR-2 activating peptide (AP). RESULTS: Cathelicidin expression was significantly higher in rosacea skin tissues than in normal tissues (p<0.001), while PAR-2 expression was not significantly higher in rosacea tissues than in normal skin tissues. A positive correlation between PAR-2 and cathelicidin within rosacea samples was observed (R=0.330, p=0.037). After treatment of PAR-2 AP, both mRNA and protein levels for PAR-2, cathelicidin, and VEGF significantly increased in cultured keratinocytes, compared with PAR-2 control peptide treatment. CONCLUSION: PAR-2 may participate in the pathogenesis of rosacea through activation of cathelicidin LL-37, a mediator of innate immune responses in the skin.

Gnatophyma: a rare form of rosacea / Gnatofima: uma forma rara de rosácea

Phyma is the last stage of rosacea and is due to chronic inflammation and edema. It can affect nose (rhinophyma), chin (gnatophyma), forehead (metophyma), ears (otophyma) and eyelids (blepharophyma). Rhinophyma is the most frequent location and there are few reports about gnatophyma. We report the case of a female patient, 41 years old, who had an infiltrated, erythematous, edematous plaque around the chin and lower lip for two years. Histopathology showed perivascular lymphocytic infiltrate, hypertrophied follicles and sebaceous glands, dilated vessels and fibrosis. She was treated with oral tetracycline, oral ivermectin and metronidazole cream with a satisfactory response. The clinical, histopathological and therapeutic response correlation confirmed the diagnosis of gnatophyma, a rare variant of phyma.

Fima é o estágio final da rosácea e ocorre devido ao edema e inflamação crônica. Pode acometer nariz (rinofima), mento (gnatofima), fronte (metofima), orelhas (otofima) e pálpebras (blefarofima). Rinofima é a localização mais encontrada e há raros relatos de gnatofima. Relataremos paciente feminina, 41 anos, que apresentava placa infiltrada, eritêmato-edematosa, em todo o mento e lábio inferior há dois anos. Histopatológico com infiltrado linfocitário perianexial e perivascular, folículos e glândulas sebáceas hipertrofiadas, vasos ectasiados e fibrose perianexial. Foi instituído tratamento com tetraciclina via oral, ivermectina via oral e metronidazol creme com resposta satisfatória. Através da correlação clínica, histopatológica e resposta terapêutica confirmou-se o diagnóstico da variante rara de fima, gnatofima.

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.

Estudio de compromiso ocular en pacientes con rosácea / Ocular involvement in patients with cutaneous rosacea

La rosácea cutánea (RC) es una enfermedad crónica relativamente común que puede presentarse con afectación ocular. La rosácea ocular es considerada una enfermedad bilateral, manifestándose en la mayoría de los casos en forma simultánea al compromiso cutáneo. El objetivo del estudio es determinar el compromiso ocular de pacientes portadores de RC comparados con un grupo control y la asociación de severidad entre el compromiso cutáneo y el ocular. Se analizó un total de 112 pacientes; un primer grupo de 70 pacientes portadores de RC en un período de dos años, y el segundo constituido por 42 controles sanos. Observamos que en nuestros pacientes con RC hay una alteración de la función lagrimal y no existe relación estadísticamente significativa entre la severidad del compromiso cutáneo y el compromiso ocular. Debido a que el compromiso ocular en la RC es subestimado, es importante la evaluación de síntomas oculares en los pacientes con RC para continuar un manejo bidisciplinario entre dermatólogos y oftalmólogos.

Cutaneous rosacea (CR) is a relatively common chronic disease that can occur with ocular involvement. Ocular rosacea is considered a bilateral entity that usually manifests simultaneously with cutaneous disease. The objective of this study is to determine ocular involvement in CR patients, compared with a control group, and to establish the association between severity of cutaneous and ocular involvement. 112 patients were analyzed; a first group of 70 patients with a two year story of CR, and the second group consisting of 42 healthy controls. At the end of the study, we observed that patients with CR had tear dysfunction and there was no statistically significant relationship between the severity of cutaneous and ocular involvement. Since eye involvement in CR is underestimated, we consider it important to investigate ocular symptoms in patients with CR and maintain multi-disciplinary management between dermatologists and ophthalmologists.

Demodicidosis en pacientes con rosácea / Demodicidosis in patients with rosacea

Objetivo: Determinar la frecuencia de demodicidosis y sus características clínicas en pacientes con rosácea. Materiales y métodos: Estudio de casos y controles en 42 pacientes con rosácea y 42 controles para describir la presencia y densidad de D. folliculorum. El estudio se realizó en el Hospital Nacional Cayetano Heredia entre marzo y setiembre del 2004, utilizándose la técnica de Tello. Resultados: Demodex folliculorum fue encontrado en los 42 pacientes con rosácea (100 por ciento) y en 13 (31,0 por ciento) del grupo control, (p= 0,000). La exposición a gatos, la crianza de roedores y cerdos, la seborrea y el uso de corticoides tópicos fueron mas frecuentes en los pacientes con rosácea. Conclusiones: La presencia de Demodex folliculorum fue más frecuente en los pacientes con rosácea. (Rev Med Hered 2007;18:15-21).

Is Demodex really non-pathogenic?

Although usually considered a non-pathogenic parasite in parasitological textbooks, Demodex folliculorum has been implicated as a causative agent for some dermatological conditions, such as rosacea-like eruptions and some types of blepharitis. Several anecdotal reports have demonstrated unequivocal tissue damage directly related to the presence of the parasite. However, this seems to be exceedingly rare, in contrast with the marked prevalence of this infestation. We have had the opportunity to observe one of such cases. A 38-year-old woman presented with rosacea-like papular lesions in her right cheek. Histopathological examination revealed granulomatous dermal inflammation with a well-preserved mite phagocytized by a multinucleated giant cell. This finding may be taken as an evidence for the pathogenicity of the parasite, inasmuch as it does not explain how such a common parasite is able to produce such a rare disease.

Tratamiento de la disebacea y la rosacea con sulfacetamida / Treatment of disebacea y of rosacea with sulfacetamide

La sulfacetamida sódica se nos presenta como una vieja alternativa para un problema actual, de consulta frecuente, como es el eritema centro facial observado en la dermatitis seborreica (disebácea) que muchas veces después de controlar la descamación persiste. Se realizó un estudio prospectivo al azar utilizando sulfacetamida sódica en solución oftálmica al 15 por ciento aplicada en forma tópica, dos veces al día en áreas de eritema. Se controlaron los pacientes por 3 meses. Se obtuvo curación en los casos de dermatitis seborreica en 88 por ciento. En la rosácea se observó curación en un paciente y mejoría en el resto. Sugerimos que la sulfacetamida sódica es una alternativa eficaz y económica para el tratado de la disebácea, no así para la rosácea