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interesting presentation of this case may render new insights regard- hypothesise a mechanical factor (Aractinigi et al. 1998; Beckett and
ing the aetiological factors of this disease. Goldberg 1991; Vaughan Jones et al. 1999).
In our case, the hypothesis of the autoimmune mechanism
Case report was plausible, as the patient suffered from type 1 diabetes. Her
A 22-year-old Moroccan woman in her second pregnancy, at 32 pathology worsened during pregnancy (in the first pregnancy she
weeks’ gestation, was admitted to our Department of Obstetrics for had an abortion for hyperglycaemic coma; in the second preg-
elevated glycaemic values (310 mg/dl). nancy, the blood glucose levels remained steadily increased, despite
She had suffered from type 1 diabetes since she was 8 years old. medical treatment). Therefore, we can speculate that the pregnancy
During her first pregnancy, she was hospitalised for hyperglycaemic triggered an uncontrolled immune response, also responsible for
coma (440 mg/dl), causing spontaneous abortion at the 20th week of the skin disorders. The type of treatment confirms this hypothesis,
gestation. in fact immunomodulatory drugs as corticosteroids determined
The course of the first part of the present pregnancy was normal. the resolution of symptoms. Resolution of the skin disorders after
Blood glucose levels were normal with insulin therapy. In the 3rd tri- delivery is another factor in favour of this hypothesis.
mester of pregnancy, blood glucose tended to increase in spite of the Furthermore, the specificity of these skin lesions with pregnancy
fact that insulin therapy had been repeatedly adapted. Therefore, the may also make us reconsider this dermatological syndrome.
patient was hospitalised to perform intensive monitoring of maternal
and fetal wellbeing.
During the admission, the patient developed intense pruritus
and abdominal erythema. The rash was described as erythematous
papules spreading below her umbilicus within abdominal stria
(Figure 1). For 5 days, the rash remained localised, but then it began to
spread centrifugally outwards on the abdomen. The papules coalesced
to form plaques with halos of erythema. The intensity of the pruritus
escalated. The maternal liver function was normal, therefore a tenta-
tive diagnosis of pruritic urticarial papules and plaques of pregnancy
(PUPPP) was made. A dermatologist was consulted on therapeu-
tic options. The treatment included Betamethasone 0.5 mg cpr
twice/day. Considering the improvement of symptoms, biopsy was
not performed on a papula, but the rash did not completely regress.
Given the persistently high blood glucose values in spite of insulin
therapy, caesarean section at 34 weeks’ gestation was performed, and
a boy of 2,780 g was born without complication. The newborn was
admitted to neonatal intensive care for hypoglycaemia, without con-
sequences. After delivery, the patient continued oral Betamethasone
0.5 mg daily for 4 days. The pruritus and rash completely resolved
on this regimen without reoccurrence. At 6 weeks postpartum, the Figure 1. Erythematous papules spreading below umbilicus within an abdominal
symptoms completely resolved. stria.
302 Obstetric case reports
PUPPP may not simply be regarded as a dermatological disease, but Case report
its skin manifestations may represent a sign of a pregnancy at risk. A 26-year-old primigravid woman diagnosed as dystrophic EB, was
Accordingly, the mechanism of occurrence of these skin disorders referred to our hospital in her 17th gestational week. On family history,
must have considerable clinical interest; however, there are still too she had one sister affected with EB who was deceased because of infec-
many doubts about the aetiology of PUPPP. Therefore, further stud- tion. Results of first visit laboratory tests (biochemistry, complete blood
ies are necessary to clarify its pathogenesis. count, TORCH panel) were within normal ranges. Triple test results
demonstrated low risk for chromosomal anomalies and open neural
Declaration of interest: The authors report no conflicts of interest. The tube defects. At the 22nd gestational week, she underwent a 2nd trimes-
authors alone are responsible for the content and writing of the paper. ter ultrasound scan, which revealed normal fetal anatomy with appropri-
ate growth for gestational age. She had regular visits to a dermatologist
in an associated hospital. There was no exacerbation in skin lesions at 34
References weeks. Weekly non-stress tests (NST) were reactive, also amniotic fluid
Ahmadi S, Powell FC. 2005. Pruritic urticarial papules and plaques of pregnancy:
Current status. Australasian Journal of Dermatology 46:53–60. index (AFI) and fetal growth were within normal ranges. The patient was
Aronson IK, Bond S, Fiedler VC et al. 1998. Pruritic urticarial papules and plaques lost to follow-up for 6 weeks. She returned to our hospital at the 40th
of pregnancy: Clinical and immunopathologic observations in 57 patients. gestational week, complaining of painful regular uterine contractions.
Journal of the American Academy of Dermatology 39:933–939. On pelvic examination, she had minimal cervical effacement and 1 cm
Aractinigi S, Berkane N, Bertheau P et al. 1998. Fetal DNA in skin of polymorphic dilatation. On systemic examination, she had widespread skin erosions,
eruption of pregnancy. Lancet 352:1898–1901. including the vulval region and vaginal mucosa, compatible with genital
Beckett MA, Goldberg NS. 1991. Pruritic urticarial papules and plaques of preg- involvement of EB. Ultrasound examination revealed anhydramnios
nancy and skin distension. Archives of Dermatology 127:125–130.
with fetal measurements compatible with 35 gestational weeks, with an
Catanzarite V, Quirk JG Jr. 1990. Papular dermatoses of pregnancy. Clinical
estimated fetal weight of 2,338 g. On Doppler examination, there was loss
J Obstet Gynaecol Downloaded from informahealthcare.com by 94.163.23.201 on 02/28/12
growth restriction in a patient with shown in Figure 1). The woman was discharged from the hospital on the
7th postoperative day. Skin lesions were stable during the puerperium.
dystrophic epidermolysis bullosa The neonate also did not have any important complications and was dis-
charged from the hospital at the same day, together with the mother.
E. Ozkaya, E. Baser, G. Akgul & T. Kucukozkan
Comment
Department of Obstetrics and Gynaecology, Dr Sami Ulus Maternity We present a pregnancy complicated with fetal growth restriction (FGR)
and Children’s Health Research and Training Hospital, Ankara, Turkey in a patient with dystrophic EB. It is unclear if EB has an adverse effect
DOI: 10.3109/01443615.2011.653595
on an otherwise healthy pregnancy. Such pregnancies usually end in
term delivery. There have, however, been instances where the pregnancy
Correspondence: E. Baser, Dr. Sami Ulus Maternity and Children’s Health results in pre-term delivery following pre-term premature rupture of
Research and Training Hospital, Babur sokak No: 44 Altindag, Ankara, Turkey. the membranes (PPROM) (Anum et al. 2009). A case of pregnancy
E-mail: eralpbaser@gmail.com complicated with FGR, similar to our case, was reported previously in
a patient with genital involvement of dystrophic EB (Bianca et al. 2003).
Epidermolysis bullosa (EB) is a group of inherited skin diseases. We The reported case delivered a growth restricted neonate at 36 weeks’
present a case of pregnancy in a patient with dystrophic EB compli- gestation. A question was raised in our minds whether EB is specifically
cated by fetal growth restriction (FGR). A 26-year-old primigravid
patient previously diagnosed as dystrophic EB with genital mucosal
involvement was referred to our hospital. The patient delivered a
growth restricted neonate at 40 weeks’ gestation, by caesarean sec-
tion, under general anaesthesia. There may be an association between
genital involvement of EB and fetal growth restriction, therefore
genital examination should be performed before and during preg-
nancy in these patients.
Introduction
Epidermolysis bullosa (EB) is a group of autosomally inherited skin
diseases, where a small trauma to skin and mucous membranes
causes severe lesions such as blisters and erosions. Three major types
of EB are defined according level of skin separation, which is intra-
epidermal in simplex, intra-lamina lucida in junctional and sub-basal
lamina in dystrophic form. Dystrophic EB is associated with con-
junctival, genital and anal involvement and pregnancies have rarely
been reported in this type of disease (Bianca et al. 2003; Buscher
et al. 1997; Price and Katz 1988). However, the effects of this disease
on an otherwise healthy pregnancy are not well understood. Herein,
we report a case of pregnancy complicated by fetal growth restriction
(FGR), in a patient with dystrophic EB. Figure 1. Caesarean incision on the second postoperative day.