Retrospective analysis of pemphigoid gestationis in 32 Saudi patients – Clinicopathological features and a literature review

Abstract

Background and objectivesPemphigoid gestationis (PG) is a rare autoimmune-mediated blistering disease that mainly affects pregnant women in their 2nd or 3rd trimester and immediate postpartum period. In addition to the clinical assessment, the diagnosis of PG is usually confirmed by histological and immunological studies. PG usually flares up at the time of delivery and spontaneously improves postpartum. Prompt recognition and appropriate management may reduce morbidity associated with this disorder. This study aimed to determine the clinical, histopathological features and treatment of PG of Saudi patients. Materials and methodsA retrospective study of 32 patients with pemphigoid gestationis (PG) was conducted from 1990 to 2014 at King Khalid University Hospital and Derma Medical Center, Riyadh, Saudi Arabia. Data regarding epidemiology, medical histories, clinical course, diagnostic test results and management were collected and analyzed. ResultsA total of 32 patients with PG were analyzed. The mean age was 31.9 years. Seventy-four percent of the patients were multigravidas, and 2 patients were primigravidas. One hundred percent of the cases were singleton pregnancies. Eighty-four percent of the cases had the onset of PG during the 2nd and 3rd trimesters. One hundred percent of patients complained of pruritus, and 94% reported this as the first symptom. Erythematous plaques and vesiculobullous eruption were the most common skin presentation. The primary sites of involvement were the abdomen, trunk, lower (mainly thighs) and upper limbs. The face and mucus membranes were rarely involved. Fifty percent of patients had recurrent symptoms with their next pregnancy. Direct immunofluorescence revealed a linear deposition of the third component of the complement along the basement membrane zone in all cases (C3),while most of the cases showed positive linear deposition of IgG. Seventy five percent of our patients had a good response to oral corticosteroids, and only one patient needed IVIG. The vast majority of the patients (61%) became free of symptoms within 1–2 months of treatment. In 53% of the patients, maternal and fetal outcomes were good with no complications. Six pregnancies were complicated by preterm labor, 2 experienced IUGR (intrauterine growth restriction), and 2 had an abortion or stillbirth. ConclusionOur study does not differ dramatically when comparing the onset of PG, the high frequency of multigravida women, the clinical course and good patient outcomes but we observed that the first attack extended from primigravida to 11th pregnancy and slight increase in recurrence rate. Finally the timely diagnosis and appropriate management of PG may improve both maternal and neonatal outcome.