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. Since data about the course and duration of the disease is limited, the aim of the study was to analyze the clinical features and long-term follow-up data on patients with SPD. Methods. A hospital database was searched for medical records of patients with SPD hospitalized at the institution between 1985 and 2014. The focus was on clinical characteristics, associated diseases, 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 (range 18–90 years). Follow-up data were available for 12 patients. The median follow-up time was 9.5 years (1–28 years). In order to establish a histological diagnosis, repeated biopsies were required (on average 1.7 biopsies per patient). In one male patient, IgA pemphigus was diagnosed by direct immunofluorescence. In most patients, skin eruption was widespread, while in a smaller number of patients, the changes were present only on the flexor surfaces. Dysproteinemia was evident in three patients. 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. Most patients with SPD responded positively to dapsone. Conclusion. SPD is a rare disease, and it usually occurs in a person’s fifth decade. While it is self-limiting in some patients, approximately one-third of patients require continuous therapy for continuous flare-ups.

Keywords

• biopsy
• dapsone
• diagnosis
• immunohistochemistry
• skin diseases, vesiculobullous
• treatment outcome