[POPP syndrome: Psoriatic onychopachydermoperiostitis]. / Síndrome POPP: onicopaquidermoperiostite psoriásica.

Psoriatic onychopachydermoperiostitis (POPP) syndrome characterizes a clinical variant of psoriatic arthritis originally described by Fournie et al in 1989. Both great toes are generally affected presenting with nail changes, painful swelling of the soft tissue close to the distal phalanx as well as specific radiologic changes such as periosteal reaction and bone erosions of the distal phalanges. Joint involvement is characteristically absent and classic psoriatic lesions may be associated. Painful symptoms may lead to severe functional and quality of life impairment. Traditional systemic treatment is generally frustrating. Here we report a female patient presenting POPP syndrome refractory to traditional systemic treatments and adalimumab, further presenting a favorable response to treatment with etanercept.

Oral lesions in 166 patients with cutaneous psoriasis: a controlled study.

OBJECTIVES: This study was aimed to test if the frequency of oral lesions bears statistical correlation or not with the condition of cutaneous psoriasis. STUDY DESIGN: Two groups were examined, one made up of 166 patients with skin psoriasis and the other with the same number of individuals with a negative history of skin diseases (control group), matched by age, race, and sex. Patients with psoriasis were grouped according to their having localized or generalized forms of the disease. The oral mucosa was thoroughly examined in both groups. Data were analyzed using chi-square test, Fisher's test, the odds ratio (OR) with a 95% confidence interval (CI), and the Ryan-Holm step-down Bonferroni procedure. The overall significance was set at P < or = 0.05. RESULTS: The oral lesions significantly associated with psoriasis were fissured tongue (FT, OR = 2.7; 95% CI: 1.3-5.6), and geographic tongue (GT, OR = 5.0; 95% CI: 1.5-16.8). Other factors analyzed, such as topical and/or systemic medication for treatment of psoriasis versus nontreated patients, and localized versus generalized forms of psoriasis presented no statistical association with the frequency of FT or GT lesions (p>0.05). CONCLUSIONS: Patients with psoriasis presented no specific oral lesion different from those seen in the control group. Although further investigation is warranted to establish whether or not either FT or GT can be characterized as an oral expression of psoriasis, the present investigation did find for both these types of lesions that the frequency of each bore a statistically significant relation with the presence of cutaneous psoriasis.

Alpha-1-antitrypsin deficiency associated with panniculitis treated with plasma exchange therapy.

BACKGROUND: Alpha-1-antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, alpha-1-antitrypsin deficiency may be associated with panniculitis. The treatment of this type of panniculitis presents a significant challenge. Previous attempts using immunosuppressive, anti-inflammatory, and cytotoxic drugs have shown variable results. AIM: To report a case of alpha-1-antitrypsin deficiency-associated panniculitis treated with plasma exchange therapy. METHODS: A 23-year-old patient developed painful red nodules on her thighs and buttocks with spontaneous ulceration and discharge of oily fluid. A skin biopsy specimen showed septal and lobular panniculitis. The serum alpha-1-antitrypsin level was 22 mg/dL. She was treated with plasma exchange therapy. RESULTS: Treatment of this patient with plasma exchange therapy led to the control of the cutaneous lesions. CONCLUSIONS: Plasma exchange therapy represents an alternative treatment which restores serum and tissue alpha-1-antitrypsin levels. This method is proposed because of its clinical benefits and greater availability.

Psoríase / Psoriasis

A psoríase é uma doença inflamatória comum, carcterizada por hiperproliferação da epiderme. Na patogênese interagem condições genéticas e respostas imunológicas aos fatores externos, os quais são necessários tanto ao aparecimento quanto à evolução. Trata-se de doença incurável, caracterizada por períodos de remissões e exacerbações, cujo diagnóstico é fundamentalmente clínico. A escolha do tratamento deve ser feita considerando-se a gravidade e a extensão do quadro clínico e o comprometimento psicoemocional. Devem ser priorizados, inicialmente, as medidas gerais e terapia tópica, a seguir, fototerapia e, por último, tratametno sistêmico. Este artigo revisa conceitos sobre epidemiologia, etiopatogenia, características clínicas e tratamento da psoríase

Estudo clínico com ditranol (antralina) em altas concentraçöes na terapia de contato curto na psoríase / Multicenter study evaluating short contact therapy for psoriasis with dithranol (anthralin) in high concentrations

Foi realizado um estudo multicêntrico aberto, näo comparativo em quatro centros dermatológicos universitários, com a participaçäo de 95 pacientes, utilizando-se pomadas contendo 1, 2 e 3% de ditranol (antralina ou cignolina) para avaliaçäo da terapia de contato curto na psoríase. A pomada foi aplicada uma vez ao dia, por 10 a 30 minutos no máximo, sendo as avaliaçöes clínicas realizadas no pré-tratamento, primeira, terceira, oitava e décima segunda semanas de tratamento (se necessário). Dos 95 pacientes tratados, 60 (63,2%) eram do sexo masculino e 35 (36,8%) do sexo feminino, com idades variando entre 18 e 79 anos (média = 40), com quadro clínico característico de psoríase vulgar, apresentando lesöes localizadas ou disseminadas. Ao final do estudo foram avaliados 69 pacientes dos quais 57(82,6%) apresentaram resultados excelentes ou bons e em quatro (5,8%) os resultados foram regulares. Em seis pacientes näo houve resposta terapêutica satisfatória e em dois ocorreram reaçöes irritantes näo toleradas. Dos 26 pacientes restantes, dois abandonaram o tratamento logo no início por motivos particulares e 24 näo compareceram à última ou duas últimas avaliaçöes. Destes, 14 pacientes apresentavam resultados bons e oito regulares quando examinados pela última vez. A tolerância ao ditranol em altas concentraçöes foi satisfatória já que somente dois pacientes foram retirados do estudo por intolerância à açäo irritativa tópica