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Pemphigus foliaceus (PF) is an autoimmune blistering disorder which affects the superficial layers of the epidermis with rare mucosal involvement. We present the case of a 12-year-old girl with PF involving the eyes and eyelids. A literature review of pediatric nonendemic PF revealed another two cases with ocular manifestations. Eyelid involvement is an uncommon feature of PF that should be properly identified and treated.

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ABSTRACT Purpose: To evaluate the variables possibly related to actinic keratosis and malignant skin lesions on the eyelid. Methods: A prospective study of patients with suspected eyelid malignancy was conducted. The participants underwent a 2-mm punch biopsy at two opposite sites of the lesion for diagnosis, and the results were compared with those of the histopathological study of the surgical excised specimen. The patients with an actinic keratosis component were divided into two groups (actinic keratosis-associated malignancy and actinic keratosis alone), which were compared for the following variables: age, disease duration, largest diameter, tumor area, Fitzpatrick classification, sex, tumor site and margin involvement. A cluster analysis was also performed. Results: We analyzed 174 lesions, of which 50 had an actinic keratosis component. Actinic keratosis was associated with squamous cell carcinoma in 22% of the cases and to basal cell carcinoma in 38%, which shows that both neoplasms may have contiguous actinic keratosis. Statistical analysis revealed no significant difference among the variables. In a cluster analysis, four groups were identified with malignant lesions in the medial canthus with the largest mean diameter and area. All margin involvements on the lower eyelid were related to malignancy, which means that all cases with margin involvement had an almost 100% risk of malignancy. Conclusions: Larger actinic keratosis lesions in the medial canthus and lesions with margin involvement on the lower eyelid have a greater probability of malignant association.

RESUMO Objetivo: Avaliar as possíveis variáveis relacionadas à ceratose actínica e lesões malignas cutâneas nas pálpebras. Métodos: Estudo prospectivo de pacientes com lesões palpebrais suspeitas de malignidade. Os participantes foram submetidos à biopsia por trépano (punch) de 2-mm em dois pontos opostos da lesão como método diagnóstico e os resultados foram comparados com o estudo histopatológico da peça excisada cirurgicamente. Aqueles que apresentaram ceratose actínica como resultado foram divididos em dois grupos (ceratose actínica associada com malignidade e ceratose actínica isolada) e foram comparados de acordo com as variáveis: idade, tempo de doença, maior diâmetro, área do tumor, classificação de Fitzpatrick, gênero, localização e acometimento da margem palpebral. A análise de cluster também foi realizada. Resultados: Foram analisadas 174 lesões e 50 delas tinham ceratose actínica como componente do tumor. Ceratose actínica esteve associada ao Carcinoma Espinocelular em 22% dos casos e ao Carcinoma Basocelular em 38%, mostrando que ambos podem ter ceratose actínica adjacente. A análise estatística não encontrou diferença entre as variáveis. A análise de cluster identificou quatro grupos e mostrou que lesões malignas no canto medial tinham maiores diâmetro e área. Acometimento da margem na pálpebra inferior relacionou-se em 100% com malignidade, enquanto a ausência de acometimento da margem mostrou menor chance de malignidade. Conclusões: Lesões maiores de ceratose actínica no canto medial e lesões com acometimento da margem palpebral inferior têm maiores chances de associação com malignidade.

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PURPOSE: To evaluate the variables possibly related to actinic keratosis and malignant skin lesions on the eyelid. METHODS: A prospective study of patients with suspected eyelid malignancy was conducted. The participants underwent a 2-mm punch biopsy at two opposite sites of the lesion for diagnosis, and the results were compared with those of the histopathological study of the surgical excised specimen. The patients with an actinic keratosis component were divided into two groups (actinic keratosis-associated malignancy and actinic keratosis alone), which were compared for the following variables: age, disease duration, largest diameter, tumor area, Fitzpatrick classification, sex, tumor site and margin involvement. A cluster analysis was also performed. RESULTS: We analyzed 174 lesions, of which 50 had an actinic keratosis component. Actinic keratosis was associated with squamous cell carcinoma in 22% of the cases and to basal cell carcinoma in 38%, which shows that both neoplasms may have contiguous actinic keratosis. Statistical analysis revealed no significant difference among the variables. In a cluster analysis, four groups were identified with malignant lesions in the medial canthus with the largest mean diameter and area. All margin involvements on the lower eyelid were related to malignancy, which means that all cases with margin involvement had an almost 100% risk of malignancy. CONCLUSIONS: Larger actinic keratosis lesions in the medial canthus and lesions with margin involvement on the lower eyelid have a greater probability of malignant association.

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ABSTRACT Juvenile xanthogranuloma is a rare benign non-Langerhans cell histiocytosis. Clinical manifestation usually occurs up to the age of 2 years, with yellowish papules and variable clinical progression. Approximately 0.75% of patients had systemic involvement and 0.25%, ocular alterations. The purpose of this report is to describe a case of a preschool 2-year-old female patient, with nodules in the upper right eyelid, 0.5-cm wide, with well-defined edges, an uncertain date of onset, a stable growth for 6 months, with no inflammatory signs, pruritus, pain, bleeding, or other similar lesions in the body. No further changes were observed in the physical examination. Histopathological examination of the specimen showed a skin lesion with histiocytoid, spindle-shaped cells and xanthomized cells, inflammatory infiltrate and numerous Touton giant cells. The result was compatible with diagnosis of juvenile xanthogranuloma. Therefore, the importance of including juvenile xanthogranuloma in the differential diagnosis of eyelid lesions is emphasized, especially in children.

RESUMO O xantogranuloma juvenil é uma patologia histiocítica benigna rara. A manifestação clínica ocorre geralmente até os 2 anos de idade com pápulas amareladas e evolução clínica variável. Cerca de 0,75% dos pacientes apresentaram comprometimento sistêmico e 0,25%, comprometimento ocular. O objetivo deste relato é descrever o caso de uma pré-escolar de 2 anos do sexo feminino, com nodulação em pálpebra superior direita, 0,5cm de base e bordos bem definidos, data de início não estimada, mas crescimento estável há 6 meses, sem sinais flogísticos, prurido, dor, sangramentos ou outras lesões similares no corpo. Sem mais alterações ao exame físico. A análise histopatológica da peça evidenciou lesão cutânea com células histiocitoides, fusiformes e outras xantomizadas; infiltrado inflamatório de permeio e numerosas células gigantes do tipo Touton, resultado compatível com o diagnóstico de xantogranuloma juvenil. Assim, ressalta-se a importância da inclusão do xantogranuloma juvenil no diagnóstico diferencial de lesões palpebrais, especialmente em crianças.

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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.

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The purpose of this article is to analyze the outcomes of two surgical techniques to treat major trichiasis. A retrospective chart review of 67 patients (89 eyelids) with major trichiasis was performed who underwent surgical treatment using one of two techniques: intermarginal split lamella with graft (ISLG group) or lid lamella resection (LLR group). There were 30 lids in the ISLG group with mean patient age of 71.8 years and 63.3% were females. There were 59 lids in the LLR group with mean patient age of 72.5 years and 52.5% were female. The minimum postoperative follow up was six months. Statistical analysis included descriptive measures, Goodman association test for contrasts between and within multinomial populations and nonparametric Mann-Whitney test for comparison between groups. P < 0.05 was considered statistically significant. The underlying causes of trichiasis were blepharitis (37.07%), chronic meibomitis (21.3%), multiple causes (20.2%), ectropion (11.2%), actinic keratosis (6.7%), or prior ocular surgery (3.3%). Postoperatively, in the ISLG group, there were 20% lids with complete success, 50% underwent laser or electrolysis, 16.7% required further surgery, and 13.3% were unsuccessful. Postoperatively, in the LLR group, there were 47.5% eyelids with complete success, 46.7% underwent laser or electrolysis, 6.8% required further surgery, and 5.1% were unsuccessful. There was a higher statistical chance of complete success with LLR (P < 0.05). LLR is superior to ISLG surgery for the treatment of major trichiasis. There is a greater chance of success with LLR and it is technically simpler.

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Introdução: O entrópio é definido como uma rotação interna da margem palpebral. O contato dos cílios e da pele palpebral com o globo ocular pode resultar em sintomas irritativos, abrasões e cicatrizes corneanas. Este trabalho tem o objetivo de apresentar a eficácia da técnica descrita por Lessa no tratamento do entrópio involucional. Métodos: Foram submetidos à correção do entrópio involucional 13 pálpebras inferiores de 11 pacientes. Utilizou-se a técnica de Lessa, em que é realizada uma incisão subciliar, descolamento subcutâneo, ressecção de faixa muscular e sutura pele-septo-pele, associada a um procedimento de suporte lateral. Resultados: Foram operados 11 pacientes (5 homens e 6 mulheres), com média de idade de 76,72 anos, (69 até 84 anos), totalizando 13 pálpebras, pois dois pacientes apresentavam a afecção bilateralmente. Em oito pacientes (9 pálpebras) usou-se a suspensão muscular. Em um paciente, a suspensão tarsal, e em dois (três pálpebras) o retalho tarsal. O tempo cirúrgico médio (por pálpebra) dos pacientes submetidos à suspensão muscular foi de 36,55 minutos, enquanto nos submetidos à cantoplastia foi de 56 minutos. Nenhum paciente apresentou recidiva e um paciente apresentou ectrópio. Conclusão: A técnica descrita por Lessa mostrou-se eficaz, pois não houve nenhum caso de recidiva.

Introduction: Entropion is defined as an internal rotation of the eyelid margin. The contact of the eyelid skin and eyelashes with the eye may result in irritating symptoms, corneal abrasions, and scars. The purpose of this study is to present the effectiveness of the technique described by Lessa in the treatment of involutional entropion. Methods: We underwent correction of involutional entropion on 13 lower eyelids. We used the Lessa technique, in which a subciliary incision was made, a skin flap was dissected from the orbicularis oculi, a muscle strip was resected, and a skinseptum- skin suture was made. The procedure was associated with lateral support. Results: There were 11 patients (5 men and 6 women) with a mean age of 76.72 years (69 to 84 years), totaling 13 eyelids, as two patients had bilateral pathology. Eight patients (9 eyelids) used muscular suspension technique. In one patient, the tarsal suspension was performed, and in two patients (three eyelids), we used the tarsal flap. The mean operative time (for eyelid) of patients undergoing muscle suspension was 36.55 min, while that in patient undergoing canthoplasty was 56 min. No patient had recurrence, and one patient developed ectropion. Conclusion: The technique described by Lessa proved to be effective since there was no recurrence.

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Abstract A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area..

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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.

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BACKGROUND: Although many factors are involved in the etiology of xanthelasma palpebrum, lipid disorder is strongly associated with its induction. Xanthelasma palpebrum, the most common type of xanthoma, usually presents in middle-aged females and results in aesthetic problems. OBJECTIVE: To evaluate thelipid profile and important clinical aspects of xanthelasma palpebrum patients. METHODS: In this descriptive study, we enrolled 42xanthelasma palpebrumpatients, and 42 cases of non-inflammatory skin disorders as thecontrol group, matched for age and gender.The clinical characteristics of the patients and fasting serum lipid profile were recorded for both groups. The data obtained were analyzed using SPSS-16. RESULTS: Xanthelasma palpebrum was found more commonly in middle-aged females with disease onset of less than 1 year, and without significant familial history of xanthoma. Furthermore,xanthelasma lesionswere most often seen in the upper lid with mild extension and was rarely associated with systemic disease. There was no statistically significant difference between two groups regarding hypertriglyceridemia (p= 0.231) and hypercholesterolemia (p= 0.302). The mean serum levels of cholesterol (221.51±60.4 mg/dl), triglyceride (185.98±71.1 mg/dl) and VLDL (37.7±17.6 mg/dl) were significantly higher and themedian HDL (36.2 (31, 41) mg/dl) level was lower in thepatient group. CONCLUSION: In our study, hypercholesterolemia and hypertriglyceridemia did not reveal a significant difference between thepatient and control groups; however, mean serum values for cholesterol, triglyceride, VLDL and HDL showed a significant difference between the two groups. Therefore, in addition to lipid abnormality, other factors could be involved in the pathogenesis of xanthelasma palpebrum.

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Abstract: Background: Although many factors are involved in the etiology of xanthelasma palpebrum, lipid disorder is strongly associated with its induction. Xanthelasma palpebrum, the most common type of xanthoma, usually presents in middle-aged females and results in aesthetic problems. Objective: To evaluate thelipid profile and important clinical aspects of xanthelasma palpebrum patients. Methods: In this descriptive study, we enrolled 42xanthelasma palpebrumpatients, and 42 cases of non-inflammatory skin disorders as thecontrol group, matched for age and gender.The clinical characteristics of the patients and fasting serum lipid profile were recorded for both groups. The data obtained were analyzed using SPSS-16. Results: Xanthelasma palpebrum was found more commonly in middle-aged females with disease onset of less than 1 year, and without significant familial history of xanthoma. Furthermore,xanthelasma lesionswere most often seen in the upper lid with mild extension and was rarely associated with systemic disease. There was no statistically significant difference between two groups regarding hypertriglyceridemia (p= 0.231) and hypercholesterolemia (p= 0.302). The mean serum levels of cholesterol (221.51±60.4 mg/dl), triglyceride (185.98±71.1 mg/dl) and VLDL (37.7±17.6 mg/dl) were significantly higher and themedian HDL (36.2 (31, 41) mg/dl) level was lower in thepatient group. Conclusion: In our study, hypercholesterolemia and hypertriglyceridemia did not reveal a significant difference between thepatient and control groups; however, mean serum values for cholesterol, triglyceride, VLDL and HDL showed a significant difference between the two groups. Therefore, in addition to lipid abnormality, other factors could be involved in the pathogenesis of xanthelasma palpebrum.

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Rothmund-Thomson syndrome (RTS) is a rare dermatosis with about 300 cases reported to date. The authors describe two siblings with RTS and inflammatory conjunctival disease featuring fornix shortening and symblepharon as well as palpebral disease with sparse eyelashes. These cases demonstrate RTS ocular surface findings different to those usually described.

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ABSTRACT Rothmund-Thomson syndrome (RTS) is a rare dermatosis with about 300 cases reported to date. The authors describe two siblings with RTS and inflammatory conjunctival disease featuring fornix shortening and symblepharon as well as palpebral disease with sparse eyelashes. These cases demonstrate RTS ocular surface findings different to those usually described.

RESUMO A síndrome de Rothmund-Thomson (SRT) é uma dermatose rara com cerca de 300 casos reportados. Os autores descrevem dois irmãos com síndrome de Rothmund-Thomson e doença inflamatória conjuntival com encurtamento do fundo de saco e simbléfaro, assim como doença palpebral com escassez de cilíos. Ambos os casos demonstram achados da superfície ocular diferentes dos habitualmente descritos.

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A 40-year-old female patient with a 5-year history of systemic lupus erythematosus was referred to our policlinic with complaints of erythema, atrophy, and telangiectasia on the upper eyelids for 8 months. No associated mucocutaneous lesion was present. Biopsy taken by our ophthalmology department revealed discoid lupus erythematosus. Topical tacrolimus was augmented to the systemic therapeutic regimen of the patient, which consisted of continuous antimalarial treatment and intermittent corticosteroid drugs. We observed no remission in spite of the 6-month supervised therapy. Periorbital discoid lupus erythematosus is very unusual and should be considered in the differential diagnosis of erythematous lesions of the periorbital area..

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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.

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OBJECTIVE: To describe the epidemiology, etiology, pathogenesis, clinical characteristics, and management of pemphigus, with an emphasis on ocular involvement. METHODS: Literature review. RESULTS: Pemphigus is an autoimmune epithelial blistering disease of the skin and mucous membranes. The typical pathological finding is acantholysis of the epidermis that leads to blister formation. Immunofluorescence techniques show autoantibody deposition on the epidermal intercellular substance. Although a genetic background is necessary, environmental factors are crucial for the onset and perpetuation of the disease. Exposure to some drugs, toxic agents, and foods and associations with other autoimmune diseases and lymphoproliferative conditions should be assessed. Generally, the skin is the most commonly affected tissue. Ocular involvement might be present and exhibit a clinical course that is independent of skin compromise. Visual function may be affected depending on the severity of the presentation. In untreated cases, mortality is high because of bacterial sepsis and hydroelectrolyte imbalance. A multidisciplinary approach should be used involving a dermatologist, ophthalmologist, and immunologist. Immunosuppressive agents are the mainstay of treatment; corticosteroids typically with azathioprine or mycophenolate mofetil are the drugs of choice. Surgical treatment of trichiasis and malposition of the eyelids and tectonic procedures for corneal perforation are sometimes required in very severe and recalcitrant cases. CONCLUSIONS: Pemphigus is a potential life- and sight-threatening disease. Understanding the disease facilitates the adequate assessment of the modifiable factors and the prompt initiation of immunotherapy. Ocular involvement can develop in patients with pemphigus. Adequate ophthalmological care is needed, in particular, prevention of infections, scarring, and corneal perforation.

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Life expectancy is increasing in most countries. With increasing age, many individuals may develop involutional ophthalmic diseases, such as eyelid aging. Dermatochalasis, ptosis, ectropion, and entropion are common disorders in middle-aged and older adults. This review outlines the pathophysiology and clinical management of these involutional eyelid disorders. Recently, a decrease in elastic fibers with ultrastructural abnormalities and an overexpression of elastin-degrading enzymes have been demonstrated in involutional ectropion and entropion. This may be the consequence of local ischemia, inflammation, and/or chronic mechanical stress. Eyelid aging with progressive loss of tone and laxity may affect the ocular surface and adnexal tissues, resulting in different clinical symptoms and signs. Surgical management depends on the appropriate correction of the underlying anatomical defect.

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