Pedro D. Oliveira

Brazilian Consensus on Photoprotection

Brazil is a country of continental dimensions with a large heterogeneity of climates and massive mixing of the population. Almost the entire national territory is located between the Equator and the Tropic of Capricorn, and the Earth axial tilt to the south certainly makes Brazil one of the countries of the world with greater extent of land in proximity to the sun. The Brazilian coastline, where most of its population lives, is more than 8,500 km long. Due to geographic characteristics and cultural trends, Brazilians are among the peoples with the highest annual exposure to the sun. Epidemiological data show a continuing increase in the incidence of non-melanoma and melanoma skin cancers. Photoprotection can be understood as a set of measures aimed at reducing sun exposure and at preventing the development of acute and chronic actinic damage. Due to the peculiarities of Brazilian territory and culture, it would not be advisable to replicate the concepts of photoprotection from other developed countries, places with completely different climates and populations. Thus the Brazilian Society of Dermatology has developed the Brazilian Consensus on Photoprotection, the first official document on photoprotection developed in Brazil for Brazilians, with recommendations on matters involving photoprotection.

Adult T-cell leukemia/lymphoma triggered by adalimumab

Here, we describe a 48-year-old woman infected by the human T-cell lymphotropic virus type 1 (HTLV-1) with spondyloarthritis, uveitis, bilateral episcleritis and neurogenic bladder. She had a history of a probable infective dermatitis associated with HTLV-1 (IDH) in childhood. After the use of adalimumab, she developed lymphocytosis and a cutaneous lymphoma associated with IDH. She had the diagnoses of IDH and of chronic adult T-cell leukemia/lymphoma, supported by the demonstration of proviral integration in the cutaneous lesion.

Analysis of cutaneous lymphomas in a medical center in Bahia, Brazil.

OBJECTIVES To evaluate the frequency of the different types of cutaneous lymphoma (CL) in 1 university hospital in Brazil and compare this frequency with those observed in other countries. METHODS After review, 72 (84.7%) cases of primary cutaneous T-cell lymphoma (CTCL) and 13 (15.3%) cases of primary cutaneous B-cell lymphoma (CBCL) were included. RESULTS Of the CTCLs, 40.3% were mycosis fungoides (MF); 26.4% were adult T-cell leukemias/lymphomas (ATLs); 23.6% were peripheral T-cell lymphomas, unspecified; and 8.3% were anaplastic large cell lymphomas. Of the MF cases, 17.2% progressed to transformed MF. Five-year survival for primary human T-cell lymphotropic virus type 1-negative CTCL, ATL, and CBCL was 64.0%, 42.1%, and 62.5%, respectively. MF and ATL were the most frequent primary CTCLs. CONCLUSIONS The frequencies observed here are close to those observed in Peru but different from those of European countries. Unfortunately, the World Health Organization/European Organization of Research and Treatment of Cancer classification does not include primary cutaneous ATL.

Adult T-cell leukemia/lymphoma in South and Central America and the Caribbean: systematic search and review:

Adult T-cell leukemia/lymphoma (ATL) is caused by the human T-cell lymphotropic virus type 1 (HTLV-1) which is endemic in countries of Caribbean and Central and South America. We performed a systematic search and review to identify publications on ATL in these countries to verify if this disease was getting recognition in these regions as well as the characteristics of the observed cases. The median age of 49.4 years was lower than that referred to in Japan. According to our findings in most Brazilian states and in some other countries, ATL is not being recognized and should be strongly considered in the differential diagnosis of T-cell leukemias/lymphomas. Failure to identify these cases may be due to the unsystematic realization of serology for HTLV-1 and phenotypic identification of non-Hodgkin lymphomas that may result from lack of resources. Detection of ATL cases has been more feasible with cooperation from foreign research centers. A huge effort should be made to improve the surveillance system for ATL diagnosis in most of the South- and Central-American and Caribbean countries, and this attitude should be embraced by public organs to support health professionals in this important task.

Inherited epidermolysis bullosa: clinical and therapeutic aspects

Inherited epidermolysis bullosa (EB) is a heterogeneous group of genetic disorders that present with skin and, in some cases, mucosal fragility, predisposing patients to the development of blisters and/or erosions after minimal trauma or friction. Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated. Diagnosis must be based on clinical and histopathological findings. To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages.

Acute adult T-cell leukemia/lymphoma (ATL) presenting with cutaneous purpuric lesions: A rare presentation

Adult T-cell leukemia/lymphoma (ATL) is an aggressive mature T-cell malignancy associated with the human T-cell lymphotropic virus type I (HTLV-I). It is generally characterized by a short survival time and poor response to chemotherapy [1]. ATL has been classifi ed clinically into fi ve distinct types: acute, chronic, lymphomatous, smoldering and primary cutaneous tumoral types [2,3]. The disease affects the skin in 43 – 72% of cases and may present as erythroderma, plaques, papules and, less frequently, as nodules and tumors [3]. Specifi c purpuric lesions as a primary manifestation of the disease are rare. To the best of our knowledge, only nine cases of diffuse purpuric lesions resulting from ATL cell infi ltration have been reported up to the present moment and all of these cases occurred in Japan (Table I), four in cases of acute ATL and four in smoldering ATL. In the remaining case, no information was available [4 – 6]. The present report refers to a case of acute ATL in which the patient presented with large, disseminated purpuric skin lesions constituting the only cutaneous manifestation of disease. An 87-year-old male patient, born and living in Salvador, Brazil, presented with disseminated cutaneous purpuric papular lesions on his trunk, limbs and palm over the previous 45 days, that had converged to form infi ltrated plaques (Figure 1). At presentation, the purpuric lesions appeared to be the only manifestation of the disease; however, the patient then went on to develop enlarged inguinal lymph node. He also complained of loss of appetite and

Double integration band of HTLV-1 in a young patient with infective dermatitis who developed an acute form of adult T-cell leukemia/lymphoma.

Few cases of acute adult T-cell leukemia/lymphoma (ATL) have been diagnosed in young patients. This report is the first to describe a young girl with infective dermatitis associated with HTLV-1 that progressed to acute ATL with Southern blot hybridization and gamma-TCR-rearrangement revealing a monoclonal pattern with two copies of the provirus.

Correlations between clinical and pathological features in 17 cases of mycosis fungoides before and after transformation

Mycosis fungoides (MF) may progress to transformed MF (T‐MF), a condition with aggressive behavior.

Adult T-cell leukemia/lymphoma treatment in Bahia, Brazil

Background Adult T-cell leukemia/lymphoma is a peripheral disease associated with human T-cell lymphotropic virus type 1. Treatment is carried out according to clinical type with watchful waiting being recommended for less aggressive types. Aggressive adult T-cell leukemia/lymphoma is generally treated with chemotherapy and/or antivirals. The objective of this study was to correlate the survival of patients diagnosed in Bahia, Brazil, with the therapeutic approaches employed and to evaluate what issues existed in their treatment processes. Methods Eighty-three adult T-cell leukemia/lymphoma patients (26 smoldering, 23 chronic, 16 acute, 13 lymphoma and five primary cutaneous tumoral) with available data were included in this study. Results Complete response was achieved in seven smoldering patients with symptomatic treatment, in two with chronic disease using antivirals/chemotherapy, in one with acute disease using antivirals and in one lymphoma using the LSG15 regimen [vincristine, cyclophosphamide, doxorubicin, and prednisolone (VCAP); doxorubicin, ranimustine, and prednisolone (AMP); and vindesine, etoposide, carboplatin, and prednisolone (VECP)]. Smoldering patients who received symptomatic treatment presented longer survival. Favorable chronic patients treated with antivirals presented longer survival compared to the unfavorable subtype. However, for the acute form, first-line chemotherapy was better, albeit without significance, than antivirals. Only one of the patients with lymphoma and primary cutaneous tumors responded. Conclusions Watchful waiting associated with phototherapy represents the best option for smoldering adult T-cell leukemia/lymphoma with survival in Bahia being superior to that described in Japan. There was a trend of better results with zidovudine/interferon-alpha in favorable chronic disease. Excellent results were achieved in the lymphoma type treated with the LSG15 protocol. Patients are diagnosed late probably due to lack of knowledge of adult T-cell leukemia/lymphoma by primary healthcare doctors and a Brazilian treatment protocol needs to be established.

An Unusual Association of Adult T-Cell Leukemia/Lymphoma With Hyalohyphomycosis.

Infection by human T-cell lymphotropic virus (HTLV-1) causes deregulation of the immune system, which makes the infected individuals more susceptible to infectious diseases. Immune deregulation is even more pronounced in HTLV-1 carriers with adult T-cell leukemia/lymphoma (ATLL), which results in frequent opportunistic infections. Hyalohyphomycosis is a rare subcutaneous mycosis which is more commonly associated with immunocompromised patients. We report a case of a HTLV-1-infected man with skin tumors, inguinal lymphadenomegaly, and lymphocytosis. Histopathological examination of skin biopsies revealed a T-cell lymphoma intermingled with a granulomatous process with abscesses and hyaline-septated hyphae. The lymph node showed only a T-cell lymphoma. The patient was diagnosed with acute ATLL and hyalohyphomycosis. He was treated with itraconazole for the subcutaneous mycosis and with chemotherapy for ATLL. A few months later, despite the treatment, he died because of progression of ATLL.