A case of unilateral naevoid telangiectasia in association with the ovarian hyperstimulation syndrome

Abstract

A 30 year old woman was referred by the obstetricians in her 28th week of pregnancy. She developed an asymptomatic rash when 16 weeks pregnant, predominantly affecting her left upper body and face. Two weeks into this pregnancy, which was conceived by IVF (in vitro fertilisation), she was admitted to hospital with gross ascites secondary to the ovarian hyperstimulation syndrome, but since then the pregnancy had progressed uneventfully.She has no personal or family history of epistaxis, abnormal mucosal bleeding or liver disease. She drinks moderate amounts of alcohol only. She took the oral contraceptive pill from the age of 18 to 22 and had developed similar but much more subtle lesions on her face and chest during this time.Multiple stellate telangiectasia were noted on the left arm, left upper anterior and posterior chest and on her face bilaterally, including the lips. Some were surrounded by anaemic haloes. There was no other mucosal involvement.Liver function tests and clotting were normal, and a hepatitis screen including hepatitis B and C serology was negative. A skin biopsy revealed multiple small ectatic vessels in the superficial dermis.The unilateral naevoid telangiectasia syndrome (UNTS) is most commonly associated with conditions in which elevated levels of circulating oestrogens are seen, including pregnancy, female puberty and in patients taking the oral contraceptive pill. Other associations have included hepatitis C, alcoholic cirrhosis, carcinoid with liver metastases and ovarian cancer. Congenital cases have been reported. The upper body is most commonly affected. One author found increased oestrogen receptors within the affected areas, but this has not been replicated subsequently. We feel that the UNTS is the best fit diagnosis for our patient. An atypical feature is the bilateral facial involvement, including the lips, although this has been reported previously. There is no family history, history of mucosal bleeding, epistaxis or typical mucosal lesions to suggest hereditary haemorrhagic telangiectasia. We would expect the telangiectasia to improve post-partum but to recur with subsequent pregnancies, and will consider treatment with the pulsed dye laser if there is no improvement after delivery. We feel that in this case the very high levels of oestrogen seen in the ovarian hyperstimulation syndrome may have been important in the development of the unilateral naevoid telangiectasia syndrome. A 30 year old woman was referred by the obstetricians in her 28th week of pregnancy. She developed an asymptomatic rash when 16 weeks pregnant, predominantly affecting her left upper body and face. Two weeks into this pregnancy, which was conceived by IVF (in vitro fertilisation), she was admitted to hospital with gross ascites secondary to the ovarian hyperstimulation syndrome, but since then the pregnancy had progressed uneventfully. She has no personal or family history of epistaxis, abnormal mucosal bleeding or liver disease. She drinks moderate amounts of alcohol only. She took the oral contraceptive pill from the age of 18 to 22 and had developed similar but much more subtle lesions on her face and chest during this time. Multiple stellate telangiectasia were noted on the left arm, left upper anterior and posterior chest and on her face bilaterally, including the lips. Some were surrounded by anaemic haloes. There was no other mucosal involvement. Liver function tests and clotting were normal, and a hepatitis screen including hepatitis B and C serology was negative. A skin biopsy revealed multiple small ectatic vessels in the superficial dermis. The unilateral naevoid telangiectasia syndrome (UNTS) is most commonly associated with conditions in which elevated levels of circulating oestrogens are seen, including pregnancy, female puberty and in patients taking the oral contraceptive pill. Other associations have included hepatitis C, alcoholic cirrhosis, carcinoid with liver metastases and ovarian cancer. Congenital cases have been reported. The upper body is most commonly affected. One author found increased oestrogen receptors within the affected areas, but this has not been replicated subsequently. We feel that the UNTS is the best fit diagnosis for our patient. An atypical feature is the bilateral facial involvement, including the lips, although this has been reported previously. There is no family history, history of mucosal bleeding, epistaxis or typical mucosal lesions to suggest hereditary haemorrhagic telangiectasia. We would expect the telangiectasia to improve post-partum but to recur with subsequent pregnancies, and will consider treatment with the pulsed dye laser if there is no improvement after delivery. We feel that in this case the very high levels of oestrogen seen in the ovarian hyperstimulation syndrome may have been important in the development of the unilateral naevoid telangiectasia syndrome.