Blastic plasmacytoid dendritic cell neoplasm: an early presentation.

A blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a cutaneous lymphoma derived from a plasmacytoid dendritic precursor cell that exhibits aggressive clinical behavior. Herein, we report a 46-year-old woman with a complaint of a painless nodule on the back, associated with pruritus. The nodule grew and new growths appeared over six months of evolution. The histopathological examination of one of the left upper limb lesions showed a dense lymphoid cell infiltrate with atypia in the superficial and deep dermis. Immunohistochemistry showed positivity for CD45, S-100 protein, CD123, and TCL 1. About two months after the initial evaluation, the patient was admitted to the Emergency Hospital of Marituba-PA with dyspnea. She progressed to cardiorespiratory arrest and death within 12 hours of admission. There is still no consensus for the treatment of BPDCN. Intensive therapy for acute leukemia can be useful, but allogeneic bone marrow transplantation has a greater chance of long-term survival.

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

BACKGROUND: Pemphigus are rare chronic autoimmune blistering disorders with challenging therapeutic management. OBJECTIVE: To investigate the therapeutic outcomes of pemphigus patients admitted to a dermatology referral service in northern Brazil. MATERIALS AND METHODS: 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. RESULTS: 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 < .0001). CONCLUSION: 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.