Remarkable follow-up experiences of a severe persistent case of pemphigoid gestationis

In 1998, we reported a severe case of pemphigoid gestationis that eventually went into remission with repeated courses of intravenous immunoglobulins (IVIg) combined with oral ciclosporin.1 In brief, the patient was a 17‐year‐old Kuwaiti woman who developed pemphigoid gestationis at 20 weeks' gestation in her first pregnancy. There was a striking family history of autoimmune disease, with her father having vitiligo and her elder sister having aggressive systemic lupus erythematosus complicated by lupus nephritis. The patient's tissue type was HLA A23, 33 B50, 51 BW 4, 6 CW 6, DR 4, 7 53, DQ 2, 8, and that of her husband (a first cousin) was HLA DR 4, 17, 52, 53, DQ 2, 8. By the time she presented in London, she had unfortunately sustained a fetal death in utero at 30 weeks' gestation and was markedly cushingoid from oral corticosteroid therapy. The pemphigoid gestationis was fully confirmed immunopathologically, and after two courses of IVIg, combined with oral ciclosporin, her disease gradually went into partial remission and the dosages of corticosteroids could be reduced. She then returned to Kuwait. The follow‐up information obtained on this patient is of interest. Against medical advice, she became pregnant again in August 1997, while she was still on 10 mg of prednisolone, but was soon able to discontinue this after delivery. She had a full term pregnancy, and delivered a baby girl, weighing 2.62 kg, in April, 1998. At no stage during the pregnancy or the postpartum period did the patient show any flare of her pemphigoid gestationis. Another pregnancy followed, and she delivered a healthy full term baby girl, birth weight 3.155 kg, in April 2001. Her fourth child, a full term healthy boy, birth weight 2.70 kg, was delivered in June 2003. At no time during any of these three subsequent pregnancies did she illustrate any flare‐up of her disorder. The only medical complication she sustained was during the second pregnancy, in October 1997, when she developed papilloedema of the left eye with rapid visual deterioration at 16 weeks' pregnancy. She then received pulse steroid therapy, 1 g methylprednisolone for five consecutive days, advised by an ophthalmologist, and showed good response, with rapid improvement of her vision. The condition was diagnosed as autoimmune optic neuritis. In November 1998, the patient developed some weakness, fatigue and a goitre, and thyroid function tests revealed high thyroid‐stimulating hormone levels: 40.8 U/mL (normal range 0.27–4.6 U/ml). Diagnosis of autoimmune thyroid disease was made. During a recent visit to Kuwait (March 2004), we reviewed the patient, who appeared normal in every way, apart from residual striae distensae, relating to pregnancies and high‐dose previous corticosteroid therapy. In our experience, it is usual for successive pregnancies to be affected by pemphigoid gestationis once the disease has been initiated. Occasional pregnancies that skip this complication have been reported, with an instance of approximately, 8%.2 For our patient, there was no evidence of male consort change and as far as we were aware, her husband (also a first cousin), fathered all four of these pregnancies. During all the years of our interest in this remarkable and perhaps unique auto immune dermatosis of pregnancy, we have never encountered three successive pregnancies being spared from the condition when the original condition was so severe in the first pregnancy. For this reason, we think the follow‐up on this case is worth reporting. Conflict of interest: none declared.

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