Gestational pemphigoid (pemphigoid gestationis)

8 February 2013, by BELTRAMINELLI H. & BORRADORI L.

Gestational pemphigoid or pemphigoid gestationis (previously known as herpes gestationis) is an autoimmune bullous disease of pregnancy that shares many clinical features and pathogenesis with bullous pemphigoid. It is characterised by the development of autoantibodies against bullous pemphigoid antigen 180 (BP180, BPAG2, collagen XVII) which predominantly belong to the IgG1 and IgG3 subclasses. Epidemiological studies have shown that gestational pemhigoid is strongly associated with the presence of certain MHC class II HLA antigens, especially DRβ1*0301 and DRβ1*0401/040X, and with the C4 null allele Q0,. These findings underscore the importance of genetic factors in the development of this disease. BP180 is expressed not only in the dermal-epidermal basement membrane but also in the placenta and the epithelial cells of the amniotic membranes. An association with hydatidiform mole and choriocarcinoma has been described in anectdotal cases. The incidence of gestational pemphigoid has been variably estimated, one case per  2,000 up to 60,000 pregnancies, depending on whether immunopathological analyses are systematically carried out in the presence of a pruriginous dermatosis during pregnancy and according to the prevalence of the MHC HLA class II antigens DR3 and DR4.

1 - IMPACT ON THE PATIENT AND PATIENT INFORMATION

The occurrence of skin symptoms during pregnancy may have a significant emotional impact on the patient. Pruritus can be disabling, leading to disturbed sleep. After the diagnosis is made, the nature and consequences of of the condition has to be explained to the patient . The prognosis of gestational pemphigoid is almost invariably good , while no major complications are expected for the foetus. Nevertheless the risk of recurrence in future pregnancies have to be emphasized, but this does not constitute a contraindication for another pregnancy. Suitable treatments exist which control the disease and reduce its morbidity [25]. The treatments available and their potential side effects should be discussed, (see the information provided in French in this reference tool: http://www.chu-rouen.fr/crnmba/crnmba_informations.html) The various doctors involved in patient’s management should work interdisciplinary and coordinated, in order to ensure best management of the condition.. There are also patients’ associations for gestational pemphigoid (http://www.pemphigus.asso.fr).

When skin biopsies are obtained, the use of chlorhexidine to disinfect the skin and local anaesthetics are free from significant risks.

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