Therapeutic outcomes of pemphigus in a referral service in northern Brazil: a retrospective study of 32 patients.

07:00 EST 14th January 2020 | BioPortfolio

Summary of "Therapeutic outcomes of pemphigus in a referral service in northern Brazil: a retrospective study of 32 patients."

: Pemphigus are rare chronic autoimmune blistering disorders with challenging therapeutic management.: To investigate the therapeutic outcomes of pemphigus patients admitted to a dermatology referral service in northern Brazil.: We conducted a retrospective analysis of 32 patients with histopathological diagnosis of pemphigus between 2010 and 2016. Clinical and epidemiological data were collected. Therapeutic outcome was evaluated according to the criteria proposed by the International Pemphigus Committee. Pemphigus Disease Area Index (PDAI) was used to quantify disease severity.: Of the 32 patients, 68.75% had pemphigus vulgaris (PV) and 31.25% had pemphigus foliaceus (PF). Female-to-male ratio was 1:1. The average age was 49.5 years. All patients received oral prednisone as the first-line therapy. Adjuvant immunosuppressive drugs were gradually added in refractory and/or severe cases. After 24 months of treatment, disease control and complete remission rates were 37.5% and 25%, respectively. The mortality rate was 9.37%. PDAI score was significantly lower at 24 months of therapeutic follow-up (p < 0.0001).: Therapeutic management with corticosteroids and/or immunosuppressive drugs was able to induce disease control/complete remission in most of patients. PDAI was a useful tool for objective assessment of disease severity during therapeutic follow-up.


Journal Details

This article was published in the following journal.

Name: The Journal of dermatological treatment
ISSN: 1471-1753
Pages: 1-16


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Referral by physicians to testing or treatment facilities in which they have financial interest. The practice is regulated by the Ethics in Patient Referrals Act of 1989.

Separation of the prickle cells of the stratum spinosum of the epidermis, resulting in atrophy of the prickle cell layer. It is seen in diseases such as pemphigus vulgaris (see PEMPHIGUS) and DARIER DISEASE.

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