Subcorneal pustular dermatosis: Clinical characteristics and long-term follow-up of seventeen patients

Abstract

Background/Aim. Subcorneal pustular dermatosis (SPD) is a rare, relapsing vesiculopustular disease, predominantly presenting on the flexor surfaces. In this study, clinical features and long-term follow-up data on patients with SPD were analyzed, since data about the course and duration of the disease is limited. Methods. A hospital database was searched for medical records of patients with SPD evaluated at the institution between 1985 and 2014. The focus was on clinical characteristics, associated diseases, the disease courses and outcomes. Results. Seventeen patients with clinical features of SPD were analyzed: nine females and eight males with a median age at presentation of 45 years (18-90). Follow-up data were available for 12 patients; the median time of follow-up was 9.5 years (1-28 years). Repeated biopsies (mean 1.7 biopsies per patient) were needed to establish histological diagnosis. In one male patient, IgA pemphigus was diagnosed by direct immunofluorescence. In most patients, skin eruption was widespread; in the minority, it was present only on flexor surfaces. Dysproteinemia was evident in three patients with SPD. The disease was self-limiting in three patients; five patients had mild flare-ups occurring 1-3 times a year without the need for treatment; four patients had continuous flare-ups requiring treatment. SPD responded favorably to dapsone in the majority of our patients. Conclusion. SPD is rare disease that usually occur in a person?s fifth decade. While it is self-limiting in some patients, approximately one-third of patients require continuous therapy for continuous flareups.