Abstract Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.

Abstract Primary cutaneous B-cell lymphomas (PCBCLs) constitute 25% of all primary cutaneous lymphomas. They present in the skin with no evidence of systemic or extracutaneous disease at the time of diagnosis, after adequate staging. Primary cutaneous B-cell lymphomas differ significantly from their nodal counterparts in relation to both clinical behavior and prognosis. The distinction between primary and secondary B-cell lymphomas is essential for defining prognosis/course of action. Such distinction is also very difficult to make, since primary and secondary B-cell lymphomas are clinically and histologically indistinguishable. We report the case of a patient with primary cutaneous follicle center lymphoma who underwent surgical excision.

BACKGROUND: notalgia paresthetica is a subdiagnosed sensory neuropathy presenting as a condition of intense itching and hyperchromic macule on the back that interferes with daily habits. OBJECTIVES: To determine the efficacy of treatment of notalgia paresthetica using oral gabapentin, assessing the degree of improvement in itching and influence on quality of life. Moreover, to evaluate the signs and symptoms associated with notalgia paresthetica. METHODS: We conducted an experimental, non-randomized, parallel, non-blinded study including 20 patients with clinical and histopathological diagnosis of notalgia paresthetica. After application of the visual analogue scale of pain adapted for pruritus and of the questionnaire of dermatology life quality index (DLQI), ten patients with visual analogue scale > 5 were given treatment with gabapentin at the dose of 300 mg/day for four weeks. The other ten were treated with topical capsaicin 0.025% daily for four weeks. After the treatment period, patients answered again the scale of itching. RESULTS: The use of gabapentin was responsible for a significant improvement in pruritus (p=0.0020). Besides itching and hyperchromic stain on the back, patients reported paresthesia and back pain. It was observed that the main factor in the worsening of the rash is heat. CONCLUSION: Gabapentin is a good option for the treatment of severe itching caused by nostalgia paresthetica.

O sarcoma de Kaposi é uma desordem angioproliferativa de causa multifatorial. O vírus herpes 8 participa na sua patogenia, porém há dúvidas se a origem das lesões é apenas por hiperplasia reacional ao vírus ou uma neoplasia propriamente dita. Quatro tipos clínicos são descritos: clássico, endêmico, iatrogênico e associado ao HIV. O tipo clássico de sarcoma de Kaposi mantém-se sem padronização de estadiamento ou protocolos de tratamento. Há alguns estudos mostrando o uso de meias compressivas no tratamento do linfedema associado ao Kaposi Comunicamos um caso de uma paciente de 90 anos com sarcoma de Kaposi clássico tratado com meias elásticas compressivas, mostrando uma resposta satisfatória.

Kaposi's sarcoma is a multifactorial angioproliferative disorder. The herpes virus 8 human contributes to its pathogenesis, but it is uncertain whether these lesions are only reactive hyperplasia to the virus or neoplasia. Four clinical types are described: classic, endemic, iatrogenic and HIV-associated. Classic Kaposi's sarcoma has no standard staging or treatment protocols. Some studies have shown the use of compression stockings in the treatment of lymphedema associated with Kaposi's sarcoma. We report the case of a 90 year-old patient with classic Kaposi's sarcoma treated with compression stockings who showed a satisfactory response.

Lúpus neonatal é uma doença rara causada pela transferência de auto-anticorpos maternos para o feto, caracterizada por manifestações clínicas transitórias como cutâneas, hematológicas e hepatobiliares, ou permanentes como o bloqueio cardíaco congênito. As manifestações cutâneas típicas incluem lesões fotossensíveis descamativas, anulares ou arqueadas na face, com discreta atrofia central, clinica e histopatologicamente similares ao lúpus cutâneo subagudo. Entretanto, em alguns casos, as lesões podem lembrar aquelas da cutis marmorata telangiectatica congênita, mas esse fenômeno é raro e somente oito casos foram reportados até hoje. Nós relatamos aqui um caso de lúpus cutâneo neonatal com lesões atípicas nos membros de cor vermelho-purpúricas e aparência marmórea, semelhantes às lesões da cutis marmorata telangiectatica congênita.

Neonatal lupus is a rare disease caused by the transplacental transfer of maternal autoantibodies to the foetus, characterized by transient clinical manifestations such as cutaneous, haematological, and hepatobiliary events or permanent such as congenital heart block. The typical cutaneous manifestations include erythematous, scaly, annular or arched lesions on the face, with slight central atrophy and photosensitivy, clinically and histologically similar to subacute cutaneous lupus. However, in some cases, the lesions may resemble those in cutis marmorata telangiectatica congenita, although this phenomenon is rare and only eight such cases have been reported to date. We report a case of cutaneous neonatal lupus with atypical lesions on the limbs, which had a reddish-purple marbled appearance, resembling the lesions in cutis marmorata telangiectatica congenita.

Prurido é um sintoma que pode estar associado a uma gama ampla de patologias, sejam elas cutâneas, sistêmicas ou mesmo sem causa definida . Conforme sua intensidade e evolução pode comprometer de forma significativa a qualidade de vida dos pacientes. Novas abordagens terapêuticas estão sendo descritas para o controle do prurido, onde a liberação da histamina não é o fator principal. Assim, baseando-se na origem de seus mediadores, faz-se uma revisão desses recursos e ressalta-se que, embora de grande importância no arsenal terapêutico, os anti-histamínicos não são a panacéia para todos os tipos de prurido.

Pruritus is a symptom that may be associated with a wide array of diseases - skin diseases, systemic diseases or even those without a defined cause. According to its severity and evolution, it may compromise the patients' quality of life in a significant way. New therapeutic approaches, in which histamine release is not the main event, are being described for pruritus control. Therefore, taking into account the origin of the mediators of pruritus, we conducted a review of the available resources emphasizing that, although very important, anti-histamines are not the answer for every kind of pruritus.

Acne fulminans é uma manifestação rara, que pode ocorrer durante a evolução da acne vulgar, principalmente, em adolescentes masculinos. Uso de isotretinoína, testosterona, e reações imunológicas exacerbadas no organismo são desencadeantes relacionados. Sinais, sintomas e alterações laboratoriais como: febre, hepatomegalia, poliartralgia, leucocitose, plaquetose, aumento de provas inflamatórias e transaminases, são característicos. A cintilografia óssea pode detectar lesões líticas em vários sítios do esqueleto. O tratamento é realizado com prednisolona, isotretinoína e antibióticos se infecções secundárias. Este caso relata um paciente masculino com diagnóstico de acne grau III, que desenvolveu acne fulminans e sacroileíte bilateral, com incapacidade de deambulação após início de terapia com isotretinoína

Acne fulminans is a rare manifestation that may occur during the evolution of acne vulgaris primarily in male adolescents. Use of isotretinoin, testosterone, and exacerbated immune responses in the body are related triggers. Signs, symptoms and laboratory findings such as fever, hepatomegaly, polyarthralgia, leukocytosis, plaquetose, increased inflammatory markers and transaminases, are characteristic. A bone scan can detect osteolytic lesions in multiple skeletal sites. The treatment is performed with prednisolone, isotretinoin and antibiotics if secondary infection is present. This case describes a male patient with a diagnosis of grade III acne, who developed acne fulminans and bilateral sacroiliitis with inability to ambulate, after initiation of therapy with isotretinoin

A epidermólise bolhosa adquirida é doença bolhosa subepidérmica crônica e rara. Geralmente, inicia-se na fase adulta, sendo a etiologia desconhecida, embora vinculada à presença de anticorpos contra o colágeno tipo VII. Há formação de bolhas, espontaneamente ou após trauma, podendo causar complicações graves. O tratamento é desapontador e difícil. Além da terapia convencional com corticoides sistêmicos, recentemente, novas modalidades terapêuticas promissoras estão sendo utilizadas, dentre elas, a imunoglobulina intravenosa. Destaca-se, neste relato, o difícil manejo clínico desta doença, e a melhora importante com a imunoglobulina intravenosa.

Acquired bullous epidermolysis is a chronic and rare bullous subepidermal disease. It usually begins in adulthood and its etiology is unknown although it is associated with antibodies against type VII collagen. There are spontaneous and trauma induced formation of blisters that may cause serious complications. Treatment is disappointing and difficult. Apart from conventional therapy with systemic corticosteroid, new therapeutic modalities such as intravenous immunoglobulin are currently being used. This report highlights the extremely difficult clinical management of this rare disease and the important improvement provided by intravenous immunoglobulin.

FUNDAMENTOS - A leishmaniose tegumentar americana ocupa o segundo lugar entre as protozoonoses por vetores no Brasil. OBJETIVOS - Descrever a distribuição da doença na macrorregião de Campinas-SP e identificar as principais dificuldades em sua prevenção. MÉTODOS - A área estudada abrange 42 municípios. Os dados foram coletados no Sistema Nacional de Agravos e de Notificação do Grupo de Vigilância Epidemiológica, de 1998 a 2004, da Superintendência de Controle de Endemias, do Centro de Zoonoses e foi feita revisão da literatura. RESULTADOS - Foram registrados 458 casos, de caráter endêmico e com maior ocorrência em Campinas e Jundiaí. A doença predominava na área urbana (57%), no sexo masculino (62%) e acometia todas as faixas etárias. As espécies vetoras encontradas foram Lutzomyia intermedia sl, L. neivai, L. migonei, L. whitmani, L. fisheri, e L. pessoai. CONCLUSÕES - A leishmaniose tegumentar está distribuída amplamente na região (81% dos municípios estudados) e predominava na área urbana (57%). As dificuldades encontradas em seu controle foram a crescente adaptação do vetor ao peridomicílio, a multiplicidade dos fatores envolvidos na transmissão e a resposta insuficiente às medidas de controle atuais. O acompanhamento do ambiente e da doença, o diagnóstico e o tratamento precoces, a notificação compulsória e o seguimento dos casos, além de investimento em pesquisas, campanhas e ações diretas junto aos pacientes são importantes para o controle da doença.

BACKGROUND - American cutaneous leishmaniasis (ACL) is ranked second among vector-transmitted zoonoses in Brazil. OBJECTIVES - This paper aims at verifying the distribution of this disease in Campinas-SP and surrounding region and to identify the main difficulties for preventive actions to this illness. METHODS - The Campinas area encloses 42 counties. Data from 1998 to 2004 were collected from the National System of Injuries and Notification in Campinas, from the Superintendence for the Control of Endemic Diseases and from the Center for Zoonoses. A literature review on the subject was performed. RESULTS - There were 458 reported endemic cases and most of them were found in the Campinas and Jundiaí metropolitan areas. ACL was predominant in urban areas (57%) and among males (62%). It was also observed in all age ranges, and among women and children. Among the known vector species, Lutzomyia intermedia sl, L. neivai, L. migonei, L. whitmani, L. fisheri and L. pessoai were reported. CONCLUSIONS - Four hundred and fifty eight cases were reported, in 81% of the studied counties. The main difficulties in ACL control have been increasing vector adaptation to the peri-urban environment; the multiplicity of factors involved in its transmission and poor results of current control measures over disease progression. More research efforts are needed to understand the environmental and epidemiological features involved in ACL endemic progression, early diagnosis and treatment. Also important is an efficient notification system, as well as improved patient follow-up and educational programs.