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Connective tissue diseases share certain common traits such as skin inflammation, and joint and other structures rich in connective tissue, along with immune defects. They represent a heterogeneous group of diseases characterized by a wide variety of signs and symptoms. The autoimmune connective tissue diseases include lupus erythematous, dermatomyositis, and morphea, and are considered classic connective tissue diseases. In this chapter The Epidemiology, etiopathogenesis, clinical presentation, differential diagnosis, complementary examinations, and therapeutic approaches for connective tissue diseases are discussed.

OBJECTIVES: Report the case of pityriasis rubra pilaris (PRP) in a male patient at the age of 2 years and 10 months. METHODS: A review of the medical record as well as the literature regarding the present conditions, via PubMed database. RESULTS: Male patient, 2 years and 10 months, referred for the first year of appearance of pruritic erythematodescamative annular plaques in lower limbs with dissemination to the trunk, upper limbs, and face, associated with scaling in the plantar region and nail dystrophy. The biopsy presented stratum corneum parakeratotic with hypergranulosis and moderate acanthosis. The diagnosis of pityriasis rubra pilaris was considered conclusive. Isotretinoin and oral antihistamine were prescribed, having complete improvement. CONCLUSIONS: PRP remains a challenging disease, be it on its pathogeny, diagnosis or treatment. Randomized studies would aid on establishing a standardized treatment for PRP, improving the quality of life of patients suffering from this disease.

Objetivos: Avaliar as características clínicas e terapêuticas dos pacientes com neurite no Ambulatório de Hanseníase em Hospital Universitário entre janeiro de 2005 e dezembro de 2013. Métodos: Estudo observacional, retrospectivo, baseado na revisão de prontuários. Foram analisados 108 prontuários e a análise estatística foi feita por médias e medianas (Microsoft Excel®). Resultados: A média de idade foi de 49,3 anos. Aproximadamente 67% da população eram homens e a maioria dos pacientes é procedente do Paraná, sendo que boa parte pertencente a grande Curitiba. As formas multibacilares prevaleceram, com a forma virchowiana (40,2%), seguida pela dimorfa (27,1%). Dos pacientes, 34% apresentaram neurite. O espessamento neural foi detectado em mais da metade dos pacientes e quase 50% dos pacientes desenvolveram reações hansênicas, mais comuns nos multibacilares. Cerca de 75% dos doentes foram submetidos a avaliação funcional inicial e 45,6% destes apresentavam algum grau de incapacidade ao diagnóstico. Dos 37 pacientes com neurite em uso de poliquimioterapia específica associada ao tratamento da neurite, apenas 4 melhoram o grau funcional, enquanto 2 pacientes tiveram piora e em 18 indivíduos houve apenas a estabilização do quadro clínico. Conclusão: O presente estudo está de acordo com a literatura em relação aos dados epidemiológicos. Observamos o predomínio das formas multibacilares e a presença significativa da neurite nas reações hansênicas. Sugerimos que o tratamento leva à uma estabilização das incapacidades funcionais com as terapêuticas atuais usadas no Serviço.

Mucormycosis is an uncommon fungal infection caused by Mucorales. It frequently occurs in patients with neutropenia, diabetes, malignancy and on corticoid therapy. However, it is rare in patients with AIDS. Clinical disease can be manifested in several forms. The case reported illustrates the rare occurrence of chromoblastomycosis and mucormycosis in an immunosuppressed patient with multibacillary leprosy, under prolonged corticosteroid and thalidomide therapy to control leprosy type 2 reaction. Neutrophil dysfunction, thalidomide therapy and work activities are some of the risk factors in this case. Chromoblastomycosis was treated by surgical excision and mucormycosis with amphotericin B. Although the prognosis of mucormycosis is generally poor, in the reported case the patient recovered successfully. This case should alert dermatologists to possible opportunistic infections in immunosuppressed patients.

Primary cutaneous nodular amyloidosis is a rare form of amyloidosis. The authors report the case of a 44-year old woman with nodules and plaques on the legs with three years of development and increase in the number and size of lesions. Clinical, histological and laboratory investigation dismissed the possibility of systemic amyloidosis, and the diagnosis of nodular amyloidosis was established. A decision was made for the surgical approach, using curettage and cauterization with good response to the proposed treatment.

Mucormycosis is an uncommon fungal infection caused by Mucorales. It frequently occurs in patients with neutropenia, diabetes, malignancy and on corticoid therapy. However, it is rare in patients with AIDS. Clinical disease can be manifested in several forms. The case reported illustrates the rare occurrence of chromoblastomycosis and mucormycosis in an immunosuppressed patient with multibacillary leprosy, under prolonged corticosteroid and thalidomide therapy to control leprosy type 2 reaction. Neutrophil dysfunction, thalidomide therapy and work activities are some of the risk factors in this case. Chromoblastomycosis was treated by surgical excision and mucormycosis with amphotericin B. Although the prognosis of mucormycosis is generally poor, in the reported case the patient recovered successfully. This case should alert dermatologists to possible opportunistic infections in immunosuppressed patients. Mucormicose é uma infecção fúngica incomum causada por Mucorales. Ocorre frequentemente em pacientes com neutropenia, diabetes, corticoterapia e condições malignas. Porém, é rara em pacientes com AIDS. A doença pode apresentar-se em diferentes formas. Este caso ilustra a rara ocorrência de mucormicose e cromoblastomicose em um paciente com hanseníase multibacilar, que estava sendo tratado com prednisona e talidomida devido a eritema nodoso (reação hansênica tipo II). Disfunção de neutrófilos, uso de talidomida e atividades profissionais são alguns fatores de risco neste caso. A cromoblastomicose foi tratada por excisão cirúrgica e a mucormicose com anfotericina B. Embora o prognóstico da mucormicose seja ruim, neste caso o tratamento foi bem sucedido. Este caso alerta dermatologistas para a possibilidade de infecções oportunistas em pacientes imunossuprimidos.

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: 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.

Porocarcinoma écrino é um tumor de pele raro derivado do acrossiríngeo. Quatro casos de porocarcinoma écrino são estudados. Os pacientes tinham idade entre 49 e 64 anos, sendo dois homens e duas mulheres. O tempo de crescimento dos tumores variou de um a 20 anos. As lesões mediam de 1,2 x 2 a 4 x 5 cm e se localizavam nas extremidades ou na face. Dois pacientes apresentavam carcinoma basocelular em sítios diferentes do porocarcinoma écrino. Duas das biópsias foram previamente descritas como poroma écrino; entretanto, malignidade foi observada em todos os casos após excisão ampla. Portanto, em toda biópsia de pele compatível com poroma écrino, deve-se considerar a possibilidade de malignidade. Eccrine porocarcinoma is an extremely rare skin tumor derived from the acrosyringium. Four cases of eccrine porocarcinoma are described, two males and two females, aged from 49 to 64 years. Tumors grew in an interval of one to 20 years. Lesions ranged from 1.2 x 2 cm to 4 x 5 cm and were on limbs or face. Two patients had basal cell carcinoma in a site different from the eccrine porocarcinoma. Two biopsies were described as eccrine poroma. Malignancy was observed in all cases after wide excision. Therefore, eccrine porocarcinoma should always be considered after a biopsy compatible with eccrine poroma.

Kaposi's sarcoma is a vascular tumor involving the wall of lymphatic vessels. There are four types: classic, endemic, iatrogenic and HIV-associated. It is a systemic, malignant and multifactorial disease and has a variable course. The primary presentation on the penis is uncommon and is mainly observed in HIV-positive patients. In HIV-negative individuals, asymptomatic papules, nodules, plaques and verrucous lesions are found. The treatment for the classic form involves surgery, cryotherapy, electrosurgery, laser and radiation therapy. The authors present a rare case of a patient with the classic form on the penis, who was successfully treated by radiation therapy.

Caso Clínico: Homem de 65 anos, diabético tipo 2, e em tratamento por hanseníase virchowiana (10a cartela de rifampicina, clofazimina e dapsona) foi admitido na enfermaria da Clínica Médica por lesões ulcerativas nas faces extensoras de membros superiores. Duas semanas antes do internamento procurou atendimento médico em outro serviço por quadro de febre, inapetência e convulsões (sic), para o qual foi prescrito ceftriaxona por 5 dias. No 3o dia de tratamento, surgiram lesões inicialmente pustulosas, que evoluíram para úlceras confluentes. Estas lesões localizavam-se principalmente em cotovelos, mas acometiam também antebraços e pernas. Ao exame físico, apresentava linfonodomegalia cervical anterior, madarose e fígado palpável a 1 polpa digita do rebordo costal direito. Qual o diagnóstico?

A infecção pelo vírus do herpes simples leva a um quadro clínico peculiar de fácil reconhecimento pelo médico. No entanto, em indivíduos imunossuprimidos, a apresentação clínica pode ser atípica, dificultando o diagnóstico e o tratamento. Em virtude disso, apresenta-se um caso de herpes simples ocorrendo em paciente submetido à quimioterapia. The herpes simplex infection can produce peculiar clinical features easily recognized by the physician. However, in immunocompromised people, the clinical presentation can be atypical, difficulting the diagnosis and treatment. Therefore, a case of herpes simplex occurring in a pacient taking chemotherapy is presented.

The herpes simplex infection can produce peculiar clinical features easily recognized by the physician. However, in immunocompromised people, the clinical presentation can be atypical, difficulting the diagnosis and treatment. Therefore, a case of herpes simplex occurring in a pacient taking chemotherapy is presented.

Eccrine porocarcinoma is an extremely rare skin tumor derived from the acrosyringium. Four cases of eccrine porocarcinoma are described, two males and two females, aged from 49 to 64 years. Tumors grew in an interval of one to 20 years. Lesions ranged from 1.2 x 2 cm to 4 x 5 cm and were on limbs or face. Two patients had basal cell carcinoma in a site different from the eccrine porocarcinoma. Two biopsies were described as eccrine poroma. Malignancy was observed in all cases after wide excision. Therefore, eccrine porocarcinoma should always be considered after a biopsy compatible with eccrine poroma.