Transmission and scanning electron microscopy of cutis rhomboidalis

Abstract Cutis rhomboidalis nuchae was assessed in a 65-year-old patient. Optical microscopy showed basophilic agglomerations in the reticular dermis with decreased elastic fibers. Trans- mission electron microscopy showed elongated, curved and fragmented structures, and in their interior the presence of electron-dense lumps was reduced and irregular, similar to modified elastic fibers, whereas the collagen fibers had a normal aspect. Scanning electron microscopy showed deposits between the bundles of collagen, resembling pebbles or stones. These findings demonstrate that, at one stage of the disease, the collagen remains normal and the alterations are seen in the elastic tissue.

Scanning electron microscopy of dermatofibroma

Abstract: Dermatofibroma is a proliferation of spindle cells located in the dermis. We used scanning electron microscopy to examine two histologically confirmed lesions and observed preserved collagen bundles in the perilesional area. In the lesional area, the collagen was denser, without formation of bundles. Higher magnification showed collagen with mesh-like appearance similar to stretched tufts of cotton. Very high magnification evidenced the tufts of cotton and spindle cells measuring 2 to 12 microns.

Cutaneous collagenous vasculopathy: light and transmission electron microscopy

Abstract Cutaneous collagenous vasculopathy is a rare acquired idiopathic microangiopathy characterized by progressive development of diffuse asymptomatic telangiectasias and histologically by accumulation of collagen type IV around the affected vessels. It is diagnosed by its clinical history, confirmed by light microscopy with collagen-specific immunostaining. We report a case of a patient with extensive acquired telangiectasias on the left arm, clinically resembling unilateral nevoid telangiectasia. Dilated blood vessels with thickened walls were observed in the dermis. Immunohistochemistry with collagen IV antibodies revealed marked collagen deposition around the vessels, confirming the diagnosis. Transmission electron microscopy observed duplicate and triplicate vascular basal membrane associated with deposition of amorphous material around the membranes.

Scanning electron microscopy of granuloma annulare

Abstract: Light microscopy of granuloma annulare shows mucin deposition with lympho-histiocytic infiltrate. We describe the ultrastructural three-dimensional aspects of a typical case of granuloma with characteristic histopathological findings. At the ultrastructural level, affected collagen bundles and granular mucin deposition were observed. Round cells corresponding to lymphocytes were identified. Bigger oval cells, corresponding to isolated or palisading histiocytes were also found. The ultrastructural aspects overlap with light microscopy and contribute to its iconographic documentation.

Domestic infestation by Sclerodermus sp. with associated skin manifestation

Abstract: Sclerodermus sp. is an aculeate insect (Hymenoptera: Bethylidae), measuring 2-4 mm in length. It is a parasitoid and needs termites as hosts to complete its life cycle. It is found in a wide variety of woods and may accidentally sting humans who come near affected wooden objects. A 50-year-old woman presented two episodes of intense pruritic lesions. Clinical diagnosis of insect bite was doubtful since there were no pets at home, lesions had not started during summer and the patient denied rural activities. During a night episode of itching, the patient examined her bed and found 8 insects likely to be responsible for the bites. Scanning electron microscopy revealed typical features of a female of the genus (wingless and with multiple stingers at the lower end of the abdomen).

Pili canaliculi as manifestation of giant axonal neuropathy

Abstract Giant axonal neuropathy is a rare autosomal recessive neurodegenerative disease. The condition is characterized by neurons with abnormally large axons due to intracellular filament accumulation. The swollen axons affect both the peripheral and central nervous system. A 6-year old female patient had been referred to a geneticist reporting problems with walking and hypotonia. At the age of 10, she became wheelchair dependent. Scanning electron microscopy of a curly hair classified it as pili canaliculi. GAN gene sequencing demonstrated mutation c.1456G>A (p.GLU486LYS). At the age of 12, the patient died due to respiratory complications. Dermatologists should be aware of this entity since hair changes are considered suggestive of GAN.

Acne scars in 18-year-old male adolescents: a population-based study of prevalence and associated factors

Abstract: Background: Acne vulgaris is a pilosebaceous follicle disorder affecting over 85% of adolescents to some degree. It frequently causes psychological distress that may persist into adulthood due to scarring. Little information about post-acne scarring epidemiology is available. Objectives: To describe prevalence, distribution patterns and associated factors of acne scarring in young males, drawing on a representative population sample from a southern Brazilian city. Methods: A cross-sectional study was undertaken during presentation for military service, which is compulsory for all 18-year-old males. A questionnaire was applied, covering topics like diet, smoking habits, ethnicity, family structure, socio-economic level, as well as specific questions about active acne and resulting scars. Dermatologists conducted the clinical examination. Results: A total of 2,201 male adolescents were interviewed and examined. The overall prevalence of acne scarring was 22%. The malar region was the most frequently involved, present in 80% of affected individuals, followed by the frontal region (31.5%), back (17%), anterior chest (8.2%) and mentonian region (6.4%). Correlation between the intensity of clinical acne and the presence of scars was found, but no association was observed with educational level, smoking, ethnicity, obesity or socio-economic status. Conclusions: There is a high prevalence of acne scars among this population. This is the first study to ascertain a correlation between acne scarring and factors such as socio-economic status and educational level. The direct relation between acne severity and scarring indicates that prompt and effective treatment is the best way to reduce scarring.

Transplante autólogo de células-tronco do sangue periférico no Hospital Universitário de Santa Maria / Autologous peripheral blood stem cells transplantation at the University Hospital of Santa Maria

Introdução: O principal objetivo deste estudo foi identificar as características clínicas dos pacientes transplantados na instituição e avaliar os resultados obtidos com a infusão autóloga de células-tronco hematopoiéticas do sangue periférico (CTHSP), a mortalidade relacionada ao transplante (MRT) e a sobrevida global (SG). Métodos: Através da revisão e avaliação retrospectiva dos prontuários dos 120 pacientes submetidos a transplante autólogo no período de dezembro de 1996 a dezembro de 2011. Resultados: Cento e vinte pacientes receberam quimioterapia mieloablativa e resgate com infusões de CTHSP, sendo 78,3% adultos, com mediana de idade de 47 anos e predomínio do sexo masculino. Os diagnósticos foram 32,5% para Mieloma Múltiplo (MM), 35,8% para Linfoma de Hodgkin (LH), 16,7% para Linfoma não Hodgkin (LNH) 4,2% para Leucemia Mieloide Aguda (LMA) e 10,8% para outras neoplasias como Tumor de Wilms, Câncer de Mama Neuroblastoma, Sarcoma de Ewing, Tumor de Testículo, Meduloblastoma, Macroglobulinemia, Amiloidose e Tumor de SNC. A mediana do número de células nucleadas totais infundidas foi de 6,46x108/kg e a de células CD34+ foi de 3,17x106/kg. A mediana de tempo para recuperação de neutrófilos foi de 10 dias e para plaquetas, de 12 dias. Foi encontrada uma correlação entre a quantidade de células CD34+ infundidas e a recuperação de neutrófilos e plaquetas. Para o grupo em geral, a MRT encontrada foi de 5%, e a probabilidade de SG em cinco anos de 55,1%. Conclusão: Os resultados obtidos com os transplantes autólogos em nossa instituição são semelhantes aos descritos na literatura internacional (AU)

Introduction: The aim of this study was to identify the clinical characteristics of patients transplanted in the institution and evaluate the results obtained with the autologous infusion of hematopoietic stem cells from peripheral blood (PBSC), transplant-related mortality (TRM) and overall survival (OS). Methods: A review and retrospective assessment of the charts of 120 patients who underwent autologous transplantation from December 1996 to December 2011. Results: One hundred and twenty patients received myeloablative chemotherapy and rescue with infusions PBSC, of whom 78.3% were adults, with a median age of 47 years and male predominance. The diagnoses were 32.5 % for Multiple Myeloma (MM), 35.8% for Hodgkin lymphoma (HL), 16.7 % for non-Hodgkin lymphoma (NHL), 4.2 % for Acute Myeloid Leukemia (AML ), and 10.8% for other cancers such as Wilms Tumor, breast cancer, neuroblastoma, Ewing's sarcoma, Testicular Tumor, medulloblastoma , macroglobulinemia , amyloidosis and CNS tumor. The median number of total nucleated cells infused was 6.46 x108/kg and of CD34+ cells was 3.17 x106/kg. The median time for neutrophil recovery was 10 days and for platelets 12 days. A correlation was found between number of CD34+ cells infused and recovery of neutrophils and platelets. For the overall group, the MRT was found to be 5% and the probability of OS at five years was 55.1 %. Conclusion: The results obtained with autologous transplantation at our institution are similar to those described in the international literature (AU)

Case for diagnosis / Caso para diagnóstico

Papular-purpuric gloves and socks syndrome is a rare, highly contagious dermatosis caused by parvovirus B19, which may result in the abrupt cessation of red blood cell production in patients with preexisting hematological diseases. It affects predominantly children and young adults and has an unusual presentation.

A síndrome "gloves and socks" pápulo-purpúrica é uma dermatose incomum, altamente contagiosa, causada pelo parvovírus B19, que pode provocar interrupção aguda da produção de glóbulos vermelhos em pacientes com doenças hematológicas preexistentes. Acomete, principalmente, crianças e jovens, e tem uma apresentaçao nao-usual.

Carcinoma de células de Merkel em extremidade inferior / Merkel cell carcinoma in lower end

Merkel Cells Carcinoma is a rare cutaneous neoplasia. Studies revealed an increase in the occurrence from 0.15 to 0.44 cases for every 100.000 inhabitants between 1986 and 2001. Around 50% of the patients, eventually, develop systemic disease, being the most common sites the liver, the bones and the brain. The etiology is still unknown. The present study presents a new case of Merkel cells carcinoma in extremity, treated with local resection and adjuvant radiotherapy.

Impact on the quality of life of dermatological patients in southern Brazil / Impacto na qualidade de vida de pacientes dermatológicos no sul do Brasil

BACKGROUNDS: Skin diseases are extremely frequent and may affect quality of life; therefore, it is important to assess it and identify which factors are associated with it. OBJECTIVES: To assess quality of life and its associated factors in dermatological patients. METHODS: A cross-sectional study involving a sample of 548 patients seen at the Dermatology Outpatient Clinic of Sistema Único de Saúde (the Brazilian National Public Health System), University Hospital, Federal University of Rio Grande do Sul (RS). Information related to socio-demographic data, medical consultation and quality of life was collected by means of the Dermatology Life Quality Index. RESULTS: A median of 7 and a mean of 7.7 (SD = 5.0) were obtained with the Dermatology Life Quality Index. The skin diseases with higher scores on the Dermatology Life Quality Index were psoriasis (median = 15.5), vitiligo (median = 13), atopic dermatitis (median = 12) and acne (median = 10). With respect to factors associated with quality of life, it was found that younger, single patients with a low income, one skin disease and longer disease duration presented poorer quality of life. CONCLUSION: The assessment of the impact of dermatoses on patients' quality of life is important for clinical management. It is essential to detect patients at higher risk of experiencing worse quality of life in order to treat them in a more integrated way.

FUNDAMENTOS: As doenças de pele são extremamente freqüentes e os pacientes podem ter a qualidade de vida afetada. Assim é importante medi-la, bem como verificar quais os fatores que podem estar associados. OBJETIVOS: Verificar a qualidade de vida em pacientes dermatológicos e os fatores associados. MÉTODOS: Estudo tranversal com uma amostra de 548 pacientes atendidos no ambulatório de dermatologia do Sistema Único de Saúde do Hospital Universitário da Universidade Federal do Rio Grande. As informações foram coletadas em um questionário pré-codificado e referiam-se à dados sócio-demográficos, da consulta e qualidade de vida. RESULTADOS: O escore total do Dermatology Life Quality Index foi uma mediana de 7 e uma média de 7,7 (DP=5,0). As dermatoses com escore maior no Dermatology Life Quality Index foram psoríase (mediana = 15,5), vitiligo (mediana =13), dermatite atópica (mediana = 12), acne (mediana=10). Com respeito aos fatores associados à qualidade de vida, identificou-se que pacientes mais jovens, com menor renda, solteiros, com uma dermatose e com um tempo maior de evolução da doença, apresentam pior qualidade de vida. CONCLUSÃO: A avaliação do impacto na qualidade de vida em pacientes com dermatoses é importante para o manejo clínico. É essencial a detecção daqueles pacientes com maior risco de apresentar uma qualidade de vida pior, para poder abordá-los de uma forma mais integrada.

Angiodema due to oral acitretin and isotretinoin / Angioedema por acitretina e isotretinoína oral

Angioedema may be caused by nonsteroidal antiinflammatory drugs, angiotensin- converting enzyme inhibitors, radiocontrast media, antibiotics, sea food etc. It can involve an allergic (IgE-mediated) or non-allergic hypersensitivity reaction, both with a similar clinical presentation. While angioedema due to isotretionin has been described previously, this is the first description of angiodema due to acitretin. We report two uncommon cases of palpebral and labial angiodema due to retinoids, by acitretin and oral isotretinoin respectively: a 48-year-old man with psoriasis and a 24-year-old woman with severe acne resistant to antibiotics and topical drugs. In both cases the reaction persisted through-out treatment with these drugs, but resolved quickly after discontinuation. Reintroduction of the drugs brought on angioedema again.

Angioedema pode ser causado por diversos fármacos como : antiinflamatórios não-esteroidais, inibidores da ECA, contrastes, antibióticos e frutos do mar, entre outras causas. Pode ser uma reação alérgica, mediada por IgE, ou não-alérgica, com apresentações clínicas semelhantes. Angioedema por isotretinoína já foi relatado, mas não por acitretina. Relatamos dois casos, uma com angioedema palpebral e um labial, por acitretina e isotretinoína, respectivamente: um paciente de 48 anos com psoríase e uma paciente de 24 anos com acne resistente à terapia convencional. Em ambos casos a afecção persistiu durante o tratamento, resolveu com a interrupção e recidivou com reexposição.

Facial miliary osteoma in HIV patient / Osteoma miliar da face em paciente portadora do HIV

Facial miliary osteoma is characterized by the presence of multiple normochromic papules, affecting mainly middle-aged women, with a previous history of acne. A 39-year-old HIV positive female patient presented hardened papules which had appeared 3 years before, located on the malar region, glabella and mentum. Optical microscopy showed spherical bone formations in the dermis. Computerized tomography of the face revealed small calcifications on the surface consistent with bone formation. Multiple facial osteomas are rare as well as its association with HIV virus infection.

Os osteomas miliares da face são caracterizados por múltiplas pápulas normocrômicas na face, afetando, geralmente, mulheres de meia idade, com história pregressa de acne. Uma paciente de 39 anos, portadora do HIV, apresentou pápulas endurecidas, com 3 anos de evolução, localizadas na região malar, glabela e mento. A microscopia óptica demonstrou formações ósseas esféricas na derme. A tomografia computadorizada da face observou pequenas calcificações na superfície, compatíveis com formação óssea. Os osteomas múltiplos da face são raros assim como sua associação com a infecção pelo HIV.

Hiperqueratose lenticular persistente / Hyperkeratosis lenticularis perstans

Doença de Flegel ou hiperqueratose lenticular persistente é uma doença rara, caracterizada por pequenas pápulas hiperceratóticas, assintomáticas, localizadas preferencialmente nos membros inferiores. Histologicamente, há hiperortoceratose, atrofia epidérmica e infiltrado inflamatório em banda. Os tratamentos, em geral, são ineficazes. Relatamos um caso em que as lesões biopsiadas não recidivaram após dois anos de seguimento.

Flegel's disease, also known as hyperkeratosis lenticularis perstans, is a rare skin disease characterized by small reddish-brown asymptomatic hyperkeratotic papules usually located on the lower extremities. The histopathological features are hyperorthokeratosis, epidermal atrophy and band-like inflammatory infiltrate in the superficial dermis. Treatment is generally ineffective. We report a case of hyperkeratosis lenticularis perstans that improved following excisional biopsy of the lesions.

Classical Ehlers-Danlos syndrome: clinical, Histological and ultrastructural aspects / Síndrome de Ehlers-Danlos clássica: aspectos clínicos, histológicos e ultraestruturais

A 12-year-old boy with difficulty in wound healing and scars of unusual appearance was examined. Thin, shiny and protruding skin, characterizing pseudotumoral lesions, was observed on his knees and elbows. Loose joints and increased skin elasticity were also observed. Light microscopy showed rare collagen bundles and loosely dispersed collagen fibers. Irregularly distributed elastic tissue was observed, and transmission electron microscopy revealed disorganized collagen fibers. In cross sections under high magnifications, fibers of varying diameters could be seen, as well as an irregular fiber outline. Scanning electron microscopy of the dermis showed disorganized collagen fibers, which were rarely arranged in bundles. Isolated and crossed-over fibers were also found.

Paciente masculino de 12 anos relatou dificuldade em cicatrizar, seguido por cicatrizes de aspecto infrequente . A pele dos joelhos e cotovelos é fina, brilhante e protrusa, caracterizando pseudo- tumores. Além disso apresenta hiperelasticidade cutânea e articular. A microscopia óptica demonstrou raros feixes colágenos, as fibras colágenas encontram-se dispersas. O tecido elástico está presente e secundariamente irregular. A microscopia eletrônica de transmissão também observou fibras colágenas desorganizadas e com cortes transversais em grande aumento evidenciou diâmetros variados e contorno irregular das mesmas. A microscopia eletrônica de varredura da derme demonstrou raros feixes colágenos e fibras colágenas isoladas e entrecruzadas.

Vellus hair cysts presenting as an atypical acneiform eruption / Cisto de pelo velos apresentando-se como erupção acneiforme atípica

A 32-year-old male patient presented for 8 months an asymptomatic therapy-resistant acneiform eruption on his back and buttocks. Skin examination showed several inflammatory papules, which evolved to hyperpigmentation. At the same distribution non inflammatory papules, which resembled rice grains, were also observed. Light microscopy showed small keratin-filled cysts, with an epithelial multilayered wall, without granular layer. Keratin and some vellus hairs were identified inside the cyst, confirming the diagnosis of vellus hair cysts. Diagnosis of vellus hair cysts should be suspected in cases of multiple papules or therapy-resistant cases of acneiform eruptions.

Um paciente de 32 anos apresentou há 8 meses uma erupção acneiforme resistente à terapêutica, localizada no dorso e nádegas. Ao exame apresentava inúmeras pápulas inflamatórias, que evoluiam para hiperpigmentação. Na mesma distribuição havia lesões não inflamatórias, lembrando grão de arroz. A microscopia óptica demonstrou cisto dérmico cuja parede era de epitélio estratificado, sem camada granular, preenchido de queratina e com pelos no interior, confirmando o diagnóstico de cisto de pelo veloso. Essa entidade deve ser suspeitada em casos de múltiplas pápulas ou erupções acneiformes resistentes à terapia.

Macrodactyly with skin hypertrophy: a minimal form of the Proteus syndrome / Macrodactilia com hipertrofia da pele: uma forma mínima da síndrome de Proteu

The Proteus syndrome was described 1983 . It has asymmetric gigantism of the limbs, verrucous epidermal naevi, cerebriform enlargement of the plantar region, vascular malformations and neoplasms, as lipomas. It received this denomination after Proteus from the Greek mythology, who had the ability to change his form . A 15 year-old boy, reported a congenital hypertrophy with syndactily of the second and third right fingers . The second case is a 35 year-old man, who reported that since birth the second right toe was bigger than the other toes, skin hypertrophy was also observed. These cases document a localized form if the Proteus syndrome, which may widen the spectrum of its variability.

A síndorme de Proteus foi descrita por Wiedemann em 1983. Ela é caracterizada por gigantismo assimétrico dos membros, nevos epidérmicos verrucosos, hipertrofia cerebriforme da região plantar, neoformações vasculares e neoplasias, como lipomas. Essa polimórfica enfermidade recebeu essa denominação segundo a figura da mitologia grega, a qual tinha como característica a habilidade de mudar de forma, para evitar sua captura. Examinou-se um menino de 15 anos, o qual referiu hipertrofia e sindactilia do segundo e terceiro quirodáctilos direitos e um homem de 35 anos, que referiu hipertrofia congênita do segundo pododáctilo direito. Havia hipertrofia da pele em ambos os casos. Esses dois pacientes documentam formas localizadas dessa síndrome, ampliando seu espectro clínico.

Escalpe lipedematoso com início precoce / Lipedematous scalp with early onset

Escalpe lipedematoso é uma rara doença de origem desconhecida, caracterizada pelo aumento amolecido do couro cabeludo. Apresentamos um caso em paciente feminina de 13 anos de idade, com espessamento amolecido do vértex, prurido e crescimento lento dos fios na região afetada. O anatomopatológico revelou edema, infiltrado mononuclear esparso e perifolicular discretos, vasos linfáticos ectásicos e aumento do tecido subcutâneo, o qual foi ratificado pela ultrassonografia. Este é o segundo relato antes dos 18 anos de idade.

Lipedematous scalp is a rare disease of unknown cause characterized by increased scalp thickness with a soft consistence. We report the case of a 13-year-old female with pruriginous soft thickening of the scalp vertex and slow growth of hair in the affected area. Anatomopathological examination showed edema, sparse mononuclear inflammatory perifollicular infiltrate, and ectatic lymphatic vessels. Ultrasonography confirmed thickening of the subcutaneous tissue. This is the second report of the disorder in a patient younger than 18 years old.

Spontaneous resolution of zoonotic sporotrichosis during pregnancy / Regressão espontânea de esporotricose zoonótica durante a gestação

Sporotrichosis is the most common deep mycosis in Latin America. The zoonotic transmission from cats has been demonstrated over the last few decades in many countries. A forty year-old veterinarian examined a cat with multiple ulcerations, which mycological examination isolated Sporothrix schenckii. During the examination, she was bitten in the right index finger by the animal. Two weeks later, an inflammatory lesion appeared, which became ulcerated and was followed by ascendant lymphangitis. The diagnoses of lymphocutaneous sporotrichosis and of a six weeks pregnancy were established. The lesions were not treated and by the 28th week the ulceration healed and the abscesses showed involution. This case illustrates that non-life-threatening forms of sporotrichosis during pregnancy could be treated with systemic antifungals after delivery.

Esporotricose é a micose profunda mais comum na América Latina. Sua transmissão zoonótica a partir de gatos foi documentada nos últimos anos em vários países. Médica veterinária foi mordida por um gato, que apresentava múltiplas ulcerações, das quais foi isolado Sporothrix schenckii . Duas semanas depois surgiu lesão inflamatória e após uma ulceração no dedo mordido, seguida de linfangite ascendente. Esporotricose linfocutânea e de gravidez foram então diagnosticados. As lesões não foram tratadas e na 28ª semana de gravidez a ulceração cicatrizou e os abcessos sofreram involução. Esse caso demonstra que formas menos graves de esporotricose poderiam ser tratadas com antifúngicos sistêmicos depois do parto.