Abstract: BACKGROUND: Permanent alopecia after bone marrow transplantation is rare, but more and more cases have been described, typically involving high doses of chemotherapeutic agents used in the conditioning regimen for the transplant. Busulfan, classically described in cases of irreversible alopecia, remains associated in recent cases. The pathogenesis involved in hair loss is not clear and there are few studies available. In addition to chemotherapeutic agents, another factor that has been implicated as a cause is chronic graft-versus-host disease. However, there are no histopathological criteria for defining this diagnosis yet. OBJECTIVE: the study aims to evaluate clinical and histological aspects in cases of permanent alopecia after bone marrow transplantation, identifying features of permanent alopecia induced by myeloablative chemotherapy and alopecia as a manifestation of chronic graft-versus-host disease. METHODS: data were collected from medical records of 7 patients, with description of the clinical features and review of slides and paraffin blocks of biopsies. RESULTS: Two distinct histological patterns were found: one similar to androgenetic alopecia, non-scarring pattern, and other similar to lichen planopilaris, scarring alopecia. CONCLUSION: The first pattern corroborates the literature cases of permanent alopecia induced by chemotherapeutic agents, and the second is compatible with manifestation of chronic graft-versus-host disease on scalp, that has never been described yet. The results contribute to the elucidation of the factors involved in these cases, including the development of therapeutic methods

BACKGROUND: Masseter hypertrophy has been treated with botulinum toxin injections because of esthetic complaints especially in Asians. OBJECTIVES: The goal of the present study was to evaluate the efficacy of abobotulin toxin use in masseter hipertrophy treatment in Brazilians. METHODS: Ten Brazilian female patients with masseter hypertrophy were subjected to injections of 90U of abobotulinum toxin A applied on each side respecting the safety zone stabilished in literature and were followed up for 24 weeks. RESULTS: When analyzing the coefficients between measures of middle and lower third of the face obtained from standardized photographs, an increase was observed, with statistical significance at 2 weeks (p=0.005) and 12 weeks (p=0.001). The progression of lower third reduction was 3.94%, 5.26%, 11.99%, and 5.47% (2, 4, 12, and 24 weeks respectively). All patients showed improvement in bruxism after treatment. Observed adverse effects were masticatory fatigue, smile limitation, and smile asymmetry. CONCLUSION: The use of abobotulinum toxin A for masseter hypertrophy is effective in Brazilians and reached its maximum effect of facial thinning at 12 weeks. Smile limitation had a higher incidence compared to that reported in the literature and may result from risorius muscle blockage caused by toxin dissemination. Despite its side effects, 80% of the patients would like to repeat the treatment.

BACKGROUND: Alopecia areata is a chronic disorder of the hair follicles and nails, of unknown etiology, with clear autoimmune components and genetic factors. Several therapeutic options have been suggested; however, no treatment is able to modify the disease course. Methotrexate is an immunosuppressant used in various dermatoses and recently introduced as a therapeutic option for alopecia areata. OBJECTIVES: To evaluate the efficacy and safety of methotrexate in alopecia areata. METHODS: In a retrospective, non-controlled study, we evaluated 31 patients with alopecia areata in current or prior treatment with methotrexate to assess the therapeutic response according to sex, age, pattern of alopecia areata, disease duration, cumulative dose of methotrexate, use of systemic corticosteroids or other treatments, and drug safety. RESULTS: Regrowth greater than 50% was observed in 67.7% of patients, with the best responses observed in those with <5 years of disease progression (79%), age over 40 years (73.3%), male patients (72.8%), cumulative dose of methotrexate 1000-1500 mg, and multifocal alopecia areata (93%). Among patients receiving systemic corticosteroids in combination with methotrexate, 77.3% had greater than 50% regrowth, compared with 44.4% in those who used methotrexate alone. The therapeutic dose ranged from 10-25 mg/week. No patient had serious adverse effects. Relapse was observed in 33.3% of patients with more than 50% regrowth. CONCLUSION: Methotrexate appears to be a promising and safe medication for the treatment of severe alopecia areata when used alone or in combination with corticosteroids.

A associação de porfiria cutânea tarda (PCT) e lúpus eritematoso sistêmico (LES) é rara. O LES, de fisiopatologia complexa e manifestações clínicas pleomórficas, assemelha-se à PCT pela fotossensibilidade. Um achado que pode diferenciar as duas doenças são as lesões cutâneas bolhosas, raras no LES, mas características da PCT. Descrevemos um caso de associação de PCT e LES e revisamos a literatura, enfatizando questões fisiopatológicas, clínicas e terapêuticas. Um dado relevante para a prática clínica concerne ao tratamento do lúpus com antimaláricos, o que pode oferecer riscos para a PCT.

The association of porphyria cutanea tarda (PCT) and systemic lupus erythematosus (SLE) is rare. Systemic lupus erythematosus, of complex pathophysiology and pleomorphic clinical manifestations, is similar to PCT regarding photosensitivity. One finding that can differentiate both diseases is the presence of cutaneous blisters, which are rare in SLE, but characteristic of PCT. We report one case of the association of PCT and SLE and revise the literature, emphasizing pathophysiological, clinical and therapeutic aspects. One relevant information for clinical practice relates to the treatment of SLE with antimalarials, which is a risk for PCT.

FUNDAMENTOS: instrumentos de avaliação da psoríase ungueal têm sido publicados na literatura como meio de padronizar as avaliações antigamente subjetivas dos autores. OBJETIVOS: utilizar índice de Gravidade da Psoríase Ungueal ou Nail Psoriasis Severity Index para avaliação em pacientes portadores de psoríase em placas durante o tratamento com acitretina. MÉTODOS: trinta voluntários portadores de psoríase em placas foram selecionados para o estudo. Vinte deles ( 8 mulheres e 12 homens) completaram o estudo. A dose inicial da acitretina foi de 0,3mg/kg/dia por 30 dias sendo elevado posteriormente para 0,5mg/kg/dia. Utilizou-se a avaliação do Nail Psoriasis Severity Index na primeira avaliação, após 2 e 4 meses avaliando-se as unhas de ambas as mãos. RESULTADOS: lesões de unhas estavam presentes em todos os pacientes na primeira avaliação. A mediana inicial do Nail Psoriasis Severity Index foi de 20 e o escore final 20,5 (2,5% de piora ao final do estudo), sem diferença estatística significativa entre as três (X2=08084, GL=2, p=0,6657). Sete pacientes pioraram no escore final. Três pacientes obtiveram melhora de 50% do Nail Psoriasis Severity Index inicial em relação ao final e apenas 1 obteve melhora de 75 . A correlação linear simples mostrou fraca associação entre a porcentagem de melhora do PASI e do Nail Psoriasis Severity Index (r=0,105, F=2,12, p=0,l62). CONCLUSÕES: o método se mostrou de fácil e de rápida execução, podendo trazer informações sobre as alterações de lâmina e matriz de um modo global. O Nail Psoriasis Severity Index não quantifica as lesões existentes isoladamente, podendo não ter a sensibilidade de detectar pequenas alterações.

BACKGROUND: assessment instruments of nail psoriasis have been published in literature as means to standardize the previous subjective assessments of authors. OBJECTIVES: Using Nail Psoriasis Severity Index for evaluation in patients with plaque psoriasis during treatment with acitretin. METHODS: Thirty volunteers with plaque psoriasis were selected for the study. Twenty patients (8 women and 12 men) completed the study. The initial dose of acitretin was 0.3 mg/kg/day for 30 days and was later raised to 0.5 mg/kg/day. Nail Psoriasis Severity Index was collected in the first evaluation, after 2 and 4 months. Nails of both hands were evaluated. RESULTS: nail lesions were present in all patients at first evaluation. The initial Nail Psoriasis Severity Index median was 20 and the final score 20.5 (2.5% of worsening at the end of the study). No statistically significant difference between the three evaluations was found (X2 = 0.8084, GL = 2, p = 0.6657). Seven patients worsened in the final score. Three patients improved 50% of the initial Nail Psoriasis Severity Index and only one had an improvement of 75%. Linear correlation showed a weak association between the improvement percentage in PASI and Nail Psoriasis Severity Index (r = 0.105, F = 2.12, p = 0.162). CONCLUSION: The method was easy and of rapid execution while potentially bringing information about changes in nail plate and matrix during treatment. The Nail Psoriasis Severity Index does not quantify the existing lesions and might not have the sensitivity to detect small changes.

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% 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% 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.

A esclerodermia localizada (EL) ou morfeia é uma doença crônica do tecido conjuntivo, de provável etiologia autoimune, que tem como base alterações na síntese e deposição do colágeno, representadas clinicamente por lesões cutâneas escleróticas. Algumas placas podem apresentar coloração amarelada ou xantocrômica, causando confusão diagnóstica. Este artigo relata o caso de uma adolescente, com concomitância de lesões eritematosas e xantocrômicas, ambas manifestações clínicas da doença.

Localized scleroderma or morphea is a chronic disease of the connective tissue. Its etiology may be autoimmune and the condition results from a disturbance in collagen synthesis and deposition, clinically represented by sclerotic skin lesions. Some plaques may be yellowish, which can be misleading at diagnosis. This article reports the case of an adolescent girl who concomitantly presented erythematous lesions and yellowish lesions, both of which constitute clinical manifestations of the disease.

A sarcoidose é doença granulomatosa multissistêmica que geralmente compromete o trato respiratório e os linfonodos hilares. A pele é comumente afetada, mas raramente o couro cabeludo. Dois casos de sarcoidose com lesões no couro cabeludo são relatados: o primeiro, em paciente negra apresentando áreas de alopecia no couro cabeludo associada a outras lesões cutâneas; e o segundo, em paciente branca, portadora de sarcoidose pulmonar, com alopecia como manifestação cutânea isolada. A sarcoidose de couro cabeludo merece especial atenção, pois nos pacientes com essa forma de lesão cutânea existe alta incidência de acometimento sistêmico.

Sarcoidosis is a multi-system granulomatous disease that generally affects the respiratory tract and hilar lymph nodes. The skin is also commonly involved, although cutaneous sarcoidosis on the scalp is rare. Two cases of scalp sarcoidosis are reported: the first presented with patchy alopecia, cutaneous sarcoidosis and also systemic disease in a black patient; the second case is related to an uncommon presentation with alopecia as the single cutaneous manifestation in a Caucasian patient with pulmonary sarcoidosis. Scalp sarcoidosis deserves special attention because there is a high incidence of other systemic lesions with this cutaneous manifestation, thus a careful investigation should be performed in these patients.

Piloleiomioma é tumor benigno de músculo liso que tem origem no músculo eretor do pêlo. Pode ocorrer isoladamente ou em grande número. Apresenta-se doloroso, sensível ao frio, toque, pressão e emoção. Relata-se caso de um homem com nódulos intradérmicos compostos por fibras de músculo liso. A imuno-histoquímica mostra-se positiva para desmina e actina, confirmando o diagnóstico.

Piloleiomyoma is a benign smooth muscle tumor which arises from the erector pilorum muscles. The lesions can be solitary or multiple. They are painful, tender to cold, touch and pressure and sensitive to emotions. The authors report the case of a man with intradermal nodules composed of smooth muscle fibers. Immunohistochemical staining was positive for desmin and actin, thus confirming the diagnosis.

Descreve-se uma família com síndrome dos cabelos anágenos frouxos (SCAF) associada à distrofia macular. Foram descritos os achados dermatológicos, oftalmológicos e de microscopia óptica e eletrônica de varredura de 11 indivíduos. Dois apresentaram SCAF, sem qualquer alteração ocular. Dos quatro indivíduos com alteração ocular, duas irmãs apresentaram SCAF e dispersões pigmentares em pólo posterior da retina, com coloboma da mácula. Em dois irmãos encontraram-se as mesmas características da SCAF e fundo de olho com dispersões pigmentares em pólo posterior, sem coloboma. Os achados sugerem que essa associação seja uma nova entidade nosológica dentro das displasias ectodérmicas.

The article describes a family with loose anagen hair syndrome (LAHS) associated with macular dystrophy. Complete dermatological and ophthalmological exams, and hair examination under optical and electron microscopy were performed in eleven index cases. Two individuals demonstrated hair findings with LAHS without ocular problems. Within the four cases with ocular problems, two sisters presented with LAHS and pigmentary dispersion on the retina with macular coloboma. Two brothers presented with LAHS and pigmentary dispersion on the retina without macular coloboma. The findings suggest that this new association is a disorder among the ectodermal dysplasia.