Background: Treatment regimens for pemphigoid gestationis (PG) are non-standardized, with most evidence derived from individual case reports or small series.Objectives: To systematically review current literature on treatments and clinical outcomes of PG and to establish recommendations on its therapeutic management.Methods: An a priori protocol was designed based on PRISMA guidelines. PubMed, Scopus, and Web of Science databases were searched for English-language articles detailing PG treatments and clinical outcomes, published between 1970 and March 2020.Results: In total, 109 articles including 140 PG patients were analyzed. No randomized controlled trials or robust observational studies detailing PG treatment were found. Systemic corticosteroids ± topical corticosteroids and/or antihistamines were the most frequently prescribed treatment modality (n = 74/137; 54%). Complete remission was achieved by 114/136 (83.8%) patients. Sixty-four patients (45.7%) were given more than one treatment modality due to side effects or ineffectiveness. Leaving aside topical corticosteroids as monotherapy ± antihistamines in patients with mild disease, systemic corticosteroids ± topical corticosteroids and/or antihistamines led to complete remission in the highest proportion of patients (83%), while steroid-sparing treatments ± topical corticosteroids and/or antihistamines were associated with the lowest proportion of flares (55.5%).Limitations: The review has been drafted based on a limited number of single case reports and small case series. Underreporting/underdiagnosis of patients with mild-to-moderate PG, partial/absent follow-up, absence of precise description of neonatal outcomes and lack of validated objective scores for measuring disease severity are other limitations of our study. Our systematic review was affected by publication bias.Conclusion: Systemic corticosteroids are the most frequently used treatment for PG. Whilst most patients achieve complete remission, many of them have refractory/persistent disease requiring multiple lines of therapy. Therefore, we provided an algorithm for PG treatment integrating the results of this systematic review with current knowledge available for bullous pemphigoid. High-quality studies will further help assess the effectiveness of different treatment options for PG.

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and limb, although the glands develop at different times even within the same body region. 3 By the eighth fetal month, the glands resemble adult ones. 4 Our patient could sweat on her palms and soles, head and neck and lower extremities; therefore, there appeared to be an abnormality in the gene from the fifth to eighth fetal months from an embryological point of view. In addition to the EDARADD gene mutation, some genetic modifications, such as the presence of a single nucleotide polymorphism, that could modulate the embryological development of sweat glands might account for the phenotype of this patient, given that ferrochelatase enzyme deficiency and an additional single nucleotide polymorphism are necessary for erythropoietic protoporphyria phenotypic expression. 5 We report a Japanese family affected with H/AED, in which a novel mutation in the EDARADD gene was identified. The affected individuals revealed abnormality only in the sweat glands, whereas other ectodermal derivatives, the teeth and hair, were intact. It is important to recognize the broad spectrum of the clinical phenotypes of HED and Q-SART is a useful tool to measure local sweat production and demonstrate a partial anhidrotic site in HED patients.