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Topical use of immune response modifiers, such as imiquimod, has increased in dermatology. Although its topical use is well tolerated, it may be associated with exacerbations of generalized cutaneous inflammatory diseases, possibly through the systemic circulation of pro-inflammatory cytokines. This report describes a case of development of pityriasis rubra pilaris, a rare erythematous-papulosquamous dermatosis, in a woman aged 60 years during treatment with imiquimod 5% cream for actinic keratosis. It evolved with erythrodermic conditions and palmoplantar keratoderma, presenting progressive clinical resolution after the introduction of methotrexate. The authors emphasize the importance of recognizing possible systemic reactions associated with the topical use of imiquimod.

Background The main risk factor associated with basal cell carcinomas (BCCs) is believed to be exposure to ultraviolet radiation (UVR). In the case of lower limb BCC, the frequency is higher in women, possibly because of greater exposure of the leg to UVR. Chronic venous insufficiency (CVI), also more common in women, may have some association with leg BCCs. Methods We retrospectively evaluated the histopathological features of leg BCCs removed between 1993 and 2017 in a tertiary referral center. The patients’ clinical data were obtained from medical records, considering, in particular, CVI. Results We selected 149 patients with leg BCCs, predominately occurring in elderly Caucasian women. Of those, 71 had a clinical diagnosis of CVI in whom the clinical tumor size and frequency of recurrences were significantly higher than patients without CVI. There was an association between clinical diagnosis of CVI and histological findings of (1) follicular induction in epidermis and (2) distal sweat duct hyperplasia. Conclusions CVI, besides the already known UVR exposure, is probably associated with leg BCCs and may determine a worse BCC course.

BACKGROUND More than 200 000 new cases of leprosy are detected worldwide annually. Physicians commonly have difficulty in differentiating tuberculoid form of leprosy (TL) from sarcoidosis' cutaneous manifestation. METHODS Skin biopsies of 33 patients with TL and 24 with sarcoidosis were reviewed on hematoxylin and eosin- and Gomori-stained sections, in order to find reliable criteria for distinguishing one disease from another. RESULTS Nine of the 24 features analyzed presented significant predictive value for diagnosis (P

Agradecimentos: This study was supported by the São Paulo Research Foundation—FAPESP (Grant Nos. 2016/22927-7, 2017/17149-8, and 11/51959-0) and by Coordination of Superior Level Staff Improvement (CAPES) (Grant No. 01-P-3368/2017). We would like to express our thanks for the access to equipment and assistance provided by the National Institute of Science and Technology on Photonics Applied to Cell Biology (INFABIC), which is cofounded by FAPESP (Grant No. 2014/50938-8) and by National Council for Scientific and Technological Development (CNPq) (Grant Nos. 465699/2014-6 and 312049/2014-5). We are also grateful to Adilson A. Piaza and Mercedes F. Santos for assistance in photographic documentation, Ana C. Piaza and Luzia A. Magalhães for technical assistance, and Cleide A.M. Silva and Juliana L.P. Argenton for statistical assistance Abstract: Sarcoidosis and tuberculoid leprosy (TL) are prototypes of granulomatous inflammation in dermatology, which embody one of the histopathology limitations in distinguishing some diseases. Recent advances in the use of nonlinear optical microscopy in skin have enabled techniques, such as second-harmonic generation (SHG), to become powerful tools to study the physical and biochemical properties of skin. We use SHG images to analyze the collagen network, to distinguish differences between sarcoidosis and TL granulomas. SHG images obtained from skin biopsies of 33 patients with TL and 24 with sarcoidosis retrospectively were analyzed using first-order statistics (FOS) and second-order statistics, such as gray-level co-occurrence matrix (GLCM). Among the four parameters evaluated (optical density, entropy, contrast, and second angular moment), only contrast demonstrated statistical significance, being higher in sarcoidosis (p = 0.02||4908.31 versus 2822.17). The results may indicate insufficient differentiating power for most tested FOS and GLCM parameters in classifying sarcoidosis and TL granulomas, when used individually. But in combination with histopathology (H&E and complementary stains, such as silver and fast acid stains), SHG analysis, like contrast, can contribute to distinguishing between these diseases. This study can provide a way to evaluate collagen distribution in granulomatous diseases FUNDAÇÃO DE AMPARO À PESQUISA DO ESTADO DE SÃO PAULO - FAPESP COORDENAÇÃO DE APERFEIÇOAMENTO DE PESSOAL DE NÍVEL SUPERIOR - CAPES CONSELHO NACIONAL DE DESENVOLVIMENTO CIENTÍFICO E TECNOLÓGICO - CNPQ Aberto

RESUMO Objetivo: Descrever a evolução clínica do nevo de Spitz, desde sua característica inicial plana até o aparecimento de uma superfície irregular, nodular e avermelhada e a conduta perante essas alterações. Descrição do caso: Criança do sexo feminino, fototipo II, com um pequeno nevo congênito na perna esquerda e outros pequenos nevos adquiridos. Paciente passou por avaliações anuais clínicas e dermatoscópicas para controle entre 3 e 7 anos de idade, quando um desses nevos, localizado na coxa esquerda, apresentou crescimento rápido. A hipótese clínica foi nevo de Spitz, com indicação de remoção cirúrgica com margem de segurança e posterior análise anatomopatológica. Considerando a idade da paciente e os aspectos clínicos e histológicos, a lesão foi diagnosticada como nevo de Spitz. Comentários: Uma lesão de padrão dermatoscópico globular e menor que 5 mm permitia acompanhamento clínico, porém a hipercromia, a estética local, o crescimento rápido, a possibilidade de trauma na região e os riscos de transformação maligna na puberdade nortearam a decisão de remoção total e posterior acompanhamento para monitorar qualquer recidiva. ABSTRACT Objective: To report the clinical evolution and handling of a Spitz nevus, from its initial flat feature to becoming an irregular, nodular, reddish lesion. Case description: Female child, phototype II, with a small congenital nevus on the left lower limb and other sustained small nevi. The patient went through annual clinical and dermoscopic evaluations between the ages of three and seven, period during which the nevi located on the left thigh grew rapidly. The clinical hypothesis was Spitz nevus, with indication of surgical removal with a safety margin and anatomopathological study. Considering patient’s age and clinical/histological aspects, the diagnosis of Spitz nevus was confirmed. Comments: Initial globular pattern and size under 5 mm upon dermoscopy allowed clinical follow-up. However, onset of hyperchromia and rapid growing of the lesion, along with aesthetic concerns, possibility of trauma in the region, and risk of malignancy at puberty guided the decision of total resection and follow-up for recurrence.

INTRODUCTION: Little is known about ethnic differences in the frequency of skin diseases, and even less in terms of Brazilian population, which is characterized by miscegenation. OBJECTIVE: To evaluate the distribution of skin disorders in black and Caucasian patients through pathological specimens. METHODS: 826 biopsies from black-skinned individuals and 1,652 from white-skinned patients were retrieved and studied from the files of the Pathology Department, UNICAMP Hospital within the period of 1993-2009. The clinical data were obtained from medical records and the results were tested by statistical methods. RESULTS: Non-melanoma cancer was the most frequent diagnosis in Caucasians (45%), differing from the frequency among black patients (8%), both arising in sun-exposed skin. Regarding topography and age, in white-skinned patients aged over 50 years, biopsies of "head and neck" prevailed. As to black patients, the disease predominated among female individuals aged from 15 to 50 years and in the genital area. In the comparative analysis of vulvar diseases, we observed differences in diagnoses of sexually transmitted diseases more common among black women. Excluding cancers and genital lesions, black patients had a higher percentage of infectious diseases. Among the non-infectious diseases, cutaneous lupus was the most frequent diagnosis in both groups. Lichen planus and drug reactions were more frequent in black patients. CONCLUSION: Apart from intrinsic differences among skin types, social factors may interfere in the distribution of diseases. Not only may these results be useful to public health programs, but they may also aid the approach to dermatological diseases in black skin patients. INTRODUÇÃO: Pouco se conhece sobre as diferenças étnicas na frequência das doenças da pele e, menos ainda, na população brasileira, caracterizada pela miscigenação. OBJETIVO: Avaliar a distribuição das afecções da pele de indivíduos negros, comparativamente com a dos brancos, em material anatomopatológico. MÉTODOS: Foram estudadas 826 biópsias de indivíduos de pele negra e 1.652 dos de pele branca, obtidas do Departamento de Anatomia Patológica do Hospital das Clínicas da Universidade Estadual de Campinas (HC-UNICAMP), entre 1993 e 2009. Os achados clínicos foram obtidos dos prontuários e os resultados testados por métodos estatísticos. RESULTADOS: O câncer não melanoma foi o diagnóstico mais frequente nos brancos (45%), diferindo, significantemente, da frequência nos negros (8%), assestando-se, em ambos, na pele exposta ao sol. Quanto à topografia e à idade, nos brancos predominavam biópsias da "cabeça e pescoço", na faixa acima dos 50 anos. Nos negros, as doenças predominavam entre 15 e 50 anos, no sexo feminino, na topografia dos genitais. À análise comparativa das doenças vulvares, observou-se diferença nos diagnósticos de doenças sexualmente transmissíveis mais frequentes nas mulheres negras. Excluindo-se os cânceres e a topografia genital, os negros apresentaram porcentagem maior de doenças infecciosas. Entre as doenças não infecciosas, o lúpus cutâneo foi a mais frequente nos dois grupos; o líquen plano e a farmacodermia foram mais frequentes nos negros. CONCLUSÃO: Além das diferenças intrínsecas de tipos de pele, fatores sociais podem atuar na distribuição das doenças. Esses resultados podem ser úteis, tanto para os programas de saúde pública quanto para a abordagem das doenças dermatológicas nos pacientes de pele negra.

Eosinophilic angiocentric fibrosis (EAF) and granuloma faciale (GF) share several histopathologic features, including eosinophil-rich inflammation, microangiitis, and progressive fibrosis. Concurrent presentation of EAF and GF suggests a pathogenetic link between them.To identify histologic findings that tell them apart and construe the pathogenetic mechanisms behind each morphologic variable, 14 immunohistochemical markers were used to study the cells subpopulations in 14 cases of GF and 3 cases of EAF.The lesions were classified according to their stage of development. The antibodies studied were: CD4, Foxp3, CD8, granzymes A and B, perforin, granulysin, CD20, CD56, CD68, ICAM-1, CD34, CD105, and 1A4.The intensity of the sclerotic response and the density of 1A4-immunostained cells were significantly higher in EAF. In both diseases, CD68 cells were the most numerous, followed by CD20, CD8, and CD4 cells. About 30% of cells expressed ICAM-1. Among cells with cytotoxic granules, granulysin-positive cells were the most frequent.Differences between GF and EAF were found to be mostly like due to anatomic site (usually skin of the face vs. sinonasal cavity) and stage of the disease development (usually earlier in cutaneous lesions because of their visibility). Innate and adaptive immunity, including B cells, T cells, and cytotoxic granules have a role in their pathogenesis.

Introduction Lichenoid drug eruption (LDE) shares similar features with lichen planus (LP), that could reflect the same pathogenesis. In LP, an autoimmune attack is accepted and cytotoxic T-lymphocytes (CD8+) predominate, especially in late lesions. Apoptosis of keratinocytes may be mediated by CD8+ T and NK cells in two distinct ways: by the release of cytotoxic molecules such as perforin and granzyme B or by the Fas/FasL system. The immunological mechanisms involved in LDE are not yet fully established. Objectives Investigate immunohistological features in LP and LDE to add clues to better understand their pathogenesis. Material and methods Twenty-two patients fulfilled all clinical, laboratory, histopathological, and follow-up features of lichen planus (n = 16) and lichenoid drug eruption (n = 6). Classic histological features favoring LP or LDE were evaluated by two observers. HAM56, MAC387, UCHL-1, OPD4, CD8, Granzyme B, Perforin, and ICAM-1 antibodies were used to decorate the immune infiltrate. Results were analyzed through Pearson correlation, Student’s t-test, and linear discriminant analysis. Results A higher number of necrotic keratinocytes as well as plasma cells and eosinophils within inflammatory cells were associated with LDE diagnosis. Only in LDE, a correlation was found between the number of T and CD8+ cells and between the number of granzyme B+ cells and apoptotic keratinocytes. Conclusion Our findings suggest a more important role of CD8+ granzyme B-containing cells in LDE group, being its synthesis associated with more intense apoptosis. So, LP and LDE may have a somewhat distinct pathogenesis.

Background: Depigmented lesions (DL) have been described in areas previously damaged by inflammation in lupus erythematosus (LE). In the absence of typical lesions, distinction with other achromic diseases might be challenging. We studied the histological features and the behavior of melanocytes in these lesions. Methods: Tissue sections of 12 patients with lupus and DL were stained with hematoxylin-eosin, periodic acid-Schiff and Fontana-Masson. Melanocytes were counted by immunohistochemistry methods using Melan A and HMB-45. Ten biopsies of normal skin were used as controls. Results: The most common histological findings were: cellular infiltration (75%); hyperkeratosis (66.7%); thickening of basement membrane (66.7%); thinning and flattening of the epidermis (58.3%) and degenerative changes in collagen fibers (50%). Epidermal melanin and melanocytes were found in 41.7%. The melanocyte counts by HMB-45 and Melan A were significantly lower than in normal skin. Conclusions: The DL still fulfill histological criteria for lupus. In the absence of a precise histological diagnosis, thickening of basement membrane, hyperkeratosis, cellular infiltration, epidermal atrophy and elastosis are the most common features. Loss of melanocytes and the dermal fibrosis suggests that DL in cutaneous LE behave as post-inflammatory scars. Franca AFEC, de Souza EM. Histopathology and immunohistochemistry of depigmented lesions in lupus erythematosus.

The purpose of this study was to investigate clinical and histomorphometric features of cat skin under long-term solar exposure. Ear skin of 34 Domestic Shorthair cats that were chronically exposed to sun was classified as follows: group 0, normal ( n = 13); group 1, initial stage of photodamage (PD) ( n = 10); group 2, advanced stage of PD ( n = 11). Histologic sections were examined independently by 2 pathologists, and epidermal thickness, adnexal unit area, and dermal cellularity were assessed by morphometry. A positive correlation was obtained between age, degree of edema and sclerosis in the upper dermis, telangiectases, squamatization of basal keratinocytes, and epidermis thickness and the degree of PD. The area occupied by adnexal structures in the dermis diminished with increased PD. Dermal sclerosis and edema best separated the 3 groups. The results indicated a high level of skin hypersensitivity to sun rays in cats. The findings may be useful for clinical testing and in general veterinary pathology and dermatology.

Summary Background: Lentigo maligna (LM) is a common melanocytic malignancy which requires therapy because of the risk of progression to invasive lentigo maligna melanoma which a much worse prognosis. Patients and Methods: 18 patients with clinical and histopathological diagnosis of LM were treated with cryosurgery.The patients were older Caucasians (mean age 59.5 years) and 11 were male. They were chosen for cryosurgery because the lesion posed a surgical challenge or the patient was not a good surgical candidate. They were treated with two freeze-thaw cycles of liquid nitrogen under local anesthesia in a single sitting. Lesions larger than 2 cm2 were divided into smaller segments for freezing. Results: The lesions resolved clinically in all cases, with no recurrence or metas-tasis detected during a mean follow-up of 75.5 months. Some patients developed hypopigmented scars. Conclusions: Cryosurgery with liquid nitrogen is an efficient, safe and in most cases aesthetically acceptable alternative method to treat LM.

Several dermatoses are considered idiopathic diseases. Many times lesions such as erythema nodosum or erythema multiformis, for example, cannot have their etiology defined. Human infection caused by Bartonella spp. may determine several clinical syndromic expressions. Starting with a clinically, histologically and ultrastructurally documented bacillary angiomatosis case, a review of medical literature was undertaken to evaluate the available diagnostic criteria regarding infection caused by these agents. Histological evaluations were concluded to be, practically speaking, an important and useful diagnostic method, especially when serology is not available.

Background: Hypertrophic lupus erythematosus (HLE) is a distinct and rare subset of lupus erythematosus (LE). It is characterized by verrucous lesions, chronic in its course, and resistant to treatment. The purpose of this study was to identify clinical and histological characteristics of HLE. Methods: We review our experience with 14 cases of HLE identified in a group of 220 patients with different forms of LE, at the UNICAMP Hospital, between 1976 and 2002. Results: All patients presented verrucous plaques concomitantly with discoid lesions. The most common sites of involvement were the face and the arms. Histology of HLE lesions revealed pseudoepitheliomatous hyperplasia engulfing elastotic material. Elastic fibers were seen in migration throughout the epidermis. Classic features of LE were noted in all cases. Three of the patients developed hyperkeratotic papules with central keratinous plug on their arms at the previous LE sites. These lesions resemble clinically and histopathologically keratoacanthomas. In one patient, HLE lesion progressed to squamous cell carcinoma (SCC), 26 years after the onset of the disease. Conclusions: Transepithelial elimination of the elastotic material may be a feature of HLE. Some HLE lesions may present as keratoacanthoma, but classical features of LE aid the correct diagnosis. SCC may arise on a long-standing HLE lesion; therefore HLE requires clinical and histopathological follow up.

Apresenta-se caso clínico de dermatite de contato e reação a distância por contato com mercúrio elementar.Paciente apresentou eritema, edema e vesículas após contato dérmico com mercúrio. Lesões evoluíram para placas eritematosas com pequenas áreas enegrecidas sugerindo necrose e vesículas secas. Ocorreram lesões eritematosas a distância no tórax, abdômen e face flexora do cotovelo. Dosagem de Hg na urina 36 horas após início do contato foi de 5,9µg/L, e no sétimo dia 19,6µg/L, indicando absorção através da pele inflamada. Lesões dermatológicas por contato direto por mercúrio metálico elementar devem ser esperadas em casos acidentais. A clinical case of contact dermatitis following direct skin contact with elemental mercury is presented. Patient had metal mercury in contact with extensive part of his leg and foot skin resulting in erythematous, edematous, and vesicular dermatitis. Lesions evolved to erythematous plaques with small scattered areas of necrosis and drying vesicle lesions. Distant lesions appeared in the right forearm, chest and abdomen. Urinary mercury 36h after contact was 5.9 µg/L, and one week later 19.6 µg/L, indicating dermal absorption. dermal absorption. Dermal effects due to elemental mercury must be expected after direct skin contact.

Objectives. To describe imaging findings of central nervous system involvement in patients with lipoid proteinosis. Methods. Patients with lipoid proteinosis followed in the dermatology and neurology departments of the State University of Campinas between 2002 and 2004 were evaluated using high-resolution MRI and computerized tomography (CT). Results. We reviewed the charts of three patients (two women) with lipoid proteinosis with, ages ranging from 5 to 44 years. Symptomatic disease duration ranged from 2 to 39 years. MRI scans showed hypointense signal in FLAIR and T2 images in the amygdalae in two of three patients. Brain CT scans from two patients showed that MRI findings were calcifications. The calcification was more evident in patients with longer disease duration. Epilepsy was identified in one patient with longer disease duration. No other epileptogenic lesion was identified on MRI in this patient. Conclusion. Lipoid proteinosis is associated with bilateral amygdalae calcification. These findings were more evident in patients with longer disease duration. Epilepsy, when present, may be related to these calcifications. Patients with lipoid proteinosis should be followed with MRI/CT in order to identify these abnormalities.

Background: Although cutaneous and oral lichen planus (LP) share similar histopathological features, oral LP often follows a recalcitrant course while LP skin lesions tend to be self-limiting. Apoptosis, mediated by cytotoxic T-cells in LP, may be triggered by the release of molecules such as perforin and granzyme B. As variation in clinical behavior can reflect differences in LP immune expression, we studied the role of those cytotoxic molecules in oral and cutaneous LP. Methods: We analyzed 16 cases of cutaneous LP and 29 of oral LP. The sections were studied on hematoxylin and eosin, CD4, CD8, perforin and granzyme B staining. Results: The mean number of immunostained cells expressing each cytotoxic molecule was significantly higher in oral LP than in cutaneous LP. A higher number of single necrotic keratinocytes (apoptotic bodies) was found in oral LP lesions when compared to cutaneous LP. Only in oral LP lesions, a higher number of CD4-positive cells was found in active lesions when compared to regressive lesions. Conclusions: Our results confirm increased expression of granzyme B and perforin in oral LP lesions as compared to cutaneous LP. The increased expression suggests a relationship with the clinical behavior of the disease. Lage D, Pimentel VN, Soares TCB, Souza EM, Metze K, Cintra ML. Perforin and granzyme B expression in oral and cutaneous lichen planus – a comparative study.

The connexins are a family of proteins whose major function is as part of the gap junctions of cell-to-cell channels. They are expressed in several tissues including brain, skin, and cochlea. Mutations in connexin genes play a major role in non-syndromic sensorineural deafness, but have been also described in individuals with variable dermatological features. In recent years, many genes responsible for hereditary skin diseases have been discovered. These genes may encode different proteins that participate in the terminal differentiation of the epidermis. Therefore alteration or absence of these proteins causes a keratinization disorder. It has been demonstrated that distinct germline mutations within six connexin (Cx) genes GJB2 (Cx26), GJB6 (Cx30), GJB3 (Cx31), GJA1 (Cx43), GJB4 (Cx30.3), and GJB5 (Cx31.1), may cause sensorineural hearing loss and various skin disease phenotypes. The crucial functional importance of each of these connexins in the mentioned ectodermic tissues is reflected by the finding that genetic defects in their genes produce a wide spectrum of genetic disorders comprising sensorineural hearing loss, disorders of cornification of the skin, hair, and nails, and keratitis. Here, we report on different mutations in the connexin genes in individuals with or without hearing loss and different skin disorders illustrating the clinical and genetic heterogeneity of the condition.

Background The dermal dendritic cell (DC) is considered to be an important component of the host defense against basal cell carcinomas (BCCs). Imiquimod, an immunologic response modifier, has recently been introduced in the topical therapy of BCCs. There is some evidence that the DC pretreatment density may affect the efficacy of imiquimod. The aim of our study was to find out which clinical or histological variables are related to the DC density at the margins of BCCs. Methods Thirty cases of BCCs of aggressive and 30 cases of nonaggressive subtypes were selected from our files. In histological sections, the density of FXIIIa-positive DCs and 1A4-positive myofibroblasts in the tumor surrounding stroma was quantified, as well as the stroma type, the tumor size and the DC density in the normal dermis. Results In nonaggressive BBCs, a multiple linear regression showed that a higher DC density was associated with an increased number of myofibroblasts, smaller tumors and those located on the face. For the aggressive subtypes, a higher DC density was related not only to an increased myofibroblast density, smaller BCCs or location on the face, but also to the presence of less mucinous and more granulation type stroma and an increased DC density in the normal dermis. The stability of the models was confirmed by bootstrap resampling. Conclusion Our study demonstrates that the density of DCs around BCCs is related to tumor size, localization and characteristics of the surrounding tumor stroma.

Multibacillary, lepromatous or borderline leprosy patients may present two types of vasculonecrotic reactions: Lucio phenomenon and that associated with erythema nodosum leprosum. Despite they can be distinguished through clinical and histological characteristics; both are often used as synonyms. It is said that leprosy reaction should be properly classified for therapeutic reasons, since it is well known that in Lucio phenomenon there is not a good response to thalidomide. The authors reported two cases of vasculonecrotic phenomena in lepromatous leprosy sharing clinical and histopathological characteristics of both reaction subtypes. The findings may indicate the spectral nature of the reaction phenomena in leprosy and emphasize the importance of the clinic-pathological correlation for proper classification. Our findings may contribute to the understanding of leprosy reactions pathogenesis, broaden the knowledge about their outcome with standard treatment, and provide the scientific background to design better therapeutic strategies for these complications.

We report the case of a 34-year-old white man with a 7-year history of nodules on the face, neck, upper torso, and arms which evolved to anetodermic lesions. After onset of these lesions, deep vein thrombosis developed in the patient's right arm and leg in association with circulating antiphospholipid antibodies.

Mycosis fungoides (MF) is the most common form of primary cutaneous T-cell lymphoma. In its early stage it may mimic benign dermatoses both on a clinical and histologic basis. MF usually expresses CD3 and CD4 (T-cell) markers. CD7 is expressed on about 90% of CD4 T cells and is often deficient on malignant T cells. Thus, CD7 may be useful in evaluating the nature of dermal lymphoid infiltrates. The aim of this study was to evaluate the usefulness of immunohistochemical detection of T-cell markers on paraffin-embedded sections, CD3 and CD7 (clone CBC.37), in the differential diagnosis of MF and benign dermatoses. Forty-two patients with diffuse dermal T-lymphocytic infiltrates were selected. Previous clinicopathologic correlation and follow-up had established the diagnosis of MF in 31 patients and benign dermatoses in 11. The mean value of stained cells in MF was 86.45% for CD3 and 53.09% for CD7 (P

A 47-year-old black woman presented with progressive asymptomatic skin lesions of more than 5 years’ duration. Physical examination revealed lax skin masses on her neck (Figure 1) and trunk, the upper part of both arms (Figure2), and the inner area of both thighs. There was a large indurated plaque on her abdomen and ulcerated tumoral lesions on her arms and legs. The lymph nodes were not enlarged. The results of routine laboratory tests (ie, complete blood cell count, erythrocyte sedimentation rate, liver function, and serum amylase, lactate dehydrogenase, calcium, electrolyte, total serum protein, urea, and creatinine levels) and radiography of the chest were all within the normal range. A biopsy specimen was obtained from the right upper arm area (Figure 3 and Figure 4). What is your diagnosis?

CONTEXT: Non-specific lymphocytic infiltrates of the skin pose difficulties in daily practice in pathology. There is still a lack of pathognomonic signs for the differential diagnosis between benign and malignant lymphocytic infiltrates. OBJECTIVE: To evaluate the morphological and immunohistochemical profile of lymphocytic infiltrations of the skin according to clinical outcome. TYPE OF STUDY: Retrospective; histopathological and immunohistochemical analysis. SETTING: Referral center, university hospital. SAMPLE: 28 cases of lymphocytic infiltrates of difficult differential diagnosis selected from the records. MAIN MEASUREMENTS: Eighteen histological variables and the immunophenotypic profile were assessed using the CD4, CD8, CD3, CD20 and CD30 lymphoid markers and compared to subsequent follow-up. RESULTS: The most common diagnoses were: initial mycosis fungoides (eight cases) and drug reactions (five cases). Single morphological variables did not discriminate between benign and malignant infiltrates except for the presence of Pautrier-Darier's microabscesses, which were found only in mycosis fungoides (p = 0.015). Patterns of superficial and deep infiltration (p = 0.037) and also the presence of eosinophils (p = 0.0207) were more frequently found in benign lymphocytic infiltrates. Immunohistochemical profile of T-cell subsets showed overlap between benign and malignant infiltrates with a predominance of CD4-positive (helper) lymphocytes in the majority of cases. CONCLUSIONS: A combination of clinical and histological features remains the most reliable approach for establishing a definite diagnosis in cases of lymphoid skin infiltrates. CONTEXTO: Infiltrações linfocitárias não-específicas da pele representam dificuldades diagnósticas na prática diária da patologia. Não há sinais patognomônicos para o diagnóstico diferencial entre infiltrações linfocitárias benignas e malignas. OBJETIVOS: Avaliar o perfil morfológico e imunofenotípico das infiltrações linfocitárias de acordo com a evolução clínica. TIPO DE ESTUDO: Retrospectivo: análise histopatológica e imunoistoquímica. LOCAL: Centro de referência, hospital universitário. AMOSTRA: 28 casos de infiltrações linfocitárias de diagnóstico diferencial difícil selecionados dos arquivos. PRINCIPAIS MEDIDAS: Análise de 18 variáveis histológicas e perfil imunofenotípico utilizando os marcadores linfóides CD4, CD8, CD3, CD20 e CD30, comparados à evolução clínica. RESULTADOS: Os diagnósticos mais comuns foram: micose fungóide - inicial (oito casos) e farmacodermias (cinco casos). Variáveis morfológicas isoladas não discriminaram infiltrados benignos e malignos, exceto pela presença dos microabscessos de Pautrier-Darier, que foram encontrados apenas na micose fungóide (p = 0,015). O padrão de infiltração superficial e profunda (p = 0,037) e a presença de eosinófilos (p = 0,0207) foram mais freqüentes nas infiltrações linfocitárias benignas. O perfil imunoistoquímico dos linfócitos T mostrou sobreposição entre infiltrações benignas e malignas, com predomínio de linfócitos T auxiliares CD4 positivos na maioria dos casos. CONCLUSÃO: A combinação das informações clínicas e histológicas representa a abordagem mais consistente para o diagnóstico diferencial dos infiltrados linfóides cutâneos.

A angiomatose bacilar é uma das bartoneloses humanas. É doença sistêmica de manifestação cutânea freqüente. Caracteriza-se por lesões angioproliferativas causadas pela Bartonella henselae e pela B. quintana, que comprometem especialmente indivíduos imunodeficientes. Deve ser bem conhecida pelos dermatologistas, pois é potencialmente fatal, mas responsiva à antibioterapia. Para estabelecer o diagnóstico nosológico eles deverão sugeri-lo ao anatomopatologista, facilitando a diferenciação histológica, sobretudo, com o granuloma piogênico e o sarcoma de Kaposi. O presente artigo apresenta revisão da literatura e fotografias clínicas, de microscopia de luz e eletrônica de transmissão. Bacillary angiomatosis is one of the human bartoneloses. It is a systemic disease usually affecting the skin. Its angioproliferative lesions are caused by B. henselae and by B. quintana. Such lesions are more frequent in immunodeficient hosts. Dermatologists must be well aware of this disease because it is potentially fatal, though it is treatable with antibiotic therapy. To establish a nosologic diagnosis, dermatologists should refer patients to a pathologist. In so doing, they will facilitate histologic differentiation especially from pyogenic granuloma and Kaposi's sarcoma. This paper presents a literature review of the disease, and includes clinical, light and transmission electron microscope photographs.

Abstract 5064 Introduction Livedoid vasculopathy is a rare skin disease caused by the extensive formation of microthrombi in dermis vessels, which leads to infarction and ulceration of the epidermis. The pathogenesis of livedoid vasculopathy is unknown, and hypercoagulation states have been implicated. Moreover, success using anticoagulation was reported in some patients. We hypothesized that decreased levels of ADAMTS13 activity and Von Willebrand factor (vWF) levels could contribute to the pathogenesis of local thrombus formation in livedoid vasculopathy. Objective Here we evaluated the levels of ADAMTS13 activity and vWF in patients with livedoid vasculopathy. Patients and methods Fourteen patients (93% female) with livedoid vasculopathy, median age of 41.5 years (22 - 48) were included in the study. All patients were evaluated for the presence of classical acquired and hereditary thrombophilia markers. In addition, vWF antigen levels (Elisa) and activity (collagen binding assays), as well as ADAMTS13 activity (evaluated by residual collagen binding) were evaluated. Results nine patients (64%) were carriers of the MTHFR C677T mutation (6 heterozygous and 3 homozygous); 1 patient was a carrier of factor V Leiden mutation, and 1 patient was a carrier of the FII G20210A mutation. None of the patients presented deficiency of antithrombin, protein C or S, and antiphospholipid antibodies were not identified in our study population. ADAMTS13 and VWF activities were normal in all patients. Median vWF antigen was 120.5U/dl (reference value of 40.0 - 232.0%), and vWF activity was 102.3% (reference value of 45.5 - 203.7%). ADAMTS13 activity was 138.9% (reference value of 56.0 - 227.2 %). Two patients had a history of thromboembolic diseases: one patient with 2 strokes and one patient with central retinal artery occlusion. Conclusion We show here that ADAMTS13 activity and vWF levels are not altered in patients with livedoid vasculopathy. The MTHFR C677T mutation is frequent in patients with livedoid vasculopathy but quantification of homocysteine levels is necessary to consider any relationship with this disease. Disclosures No relevant conflicts of interest to declare.