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Journal of Obstetrics and Gynaecology, April 2012; 32: 301–302
© 2012 Informa UK, Ltd.
ISSN 0144-3615 print/ISSN 1364-6893 online
Obstetric Case Reports
Pruritic urticarial papules and plaques of
pregnancy
E. Giugliano, E. Cagnazzo, T. Servello, E. Mossuto, R. Marci &
A. Patella
Department of Biomedical Sciences and Advanced Therapy, Section
of Obstetrics and Gynaecology, University of Ferrara, Ferrara, Italy
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DOI: 10.3109/01443615.2011.652704
Correspondence: E. Giugliano, Department of Biomedical Sciences and
Advanced Therapy, Section of Obstetrics and Gynaecology, Giovecca Street 203,
University of Ferrara. E-mail: doctorgiugli@hotmail.it
Introduction
The term pruritic urticarial papules and plaques of pregnancy
(PUPPP) was first proposed by Lawley et al. in 1979 to describe
benign dermatoses of pregnancy characterised by erythema, urticarial plaques and papules. It is a skin disorder whose incidence is
estimated to occur in one in 200 pregnancies (0.5%) (Aronson et al.
1998).
In the present study, an unusual case of PUPPP is reported. The
interesting presentation of this case may render new insights regarding the aetiological factors of this disease.
Case report
A 22-year-old Moroccan woman in her second pregnancy, at 32
weeks’ gestation, was admitted to our Department of Obstetrics for
elevated glycaemic values (310 mg/dl).
She had suffered from type 1 diabetes since she was 8 years old.
During her first pregnancy, she was hospitalised for hyperglycaemic
coma (440 mg/dl), causing spontaneous abortion at the 20th week of
gestation.
The course of the first part of the present pregnancy was normal.
Blood glucose levels were normal with insulin therapy. In the 3rd trimester of pregnancy, blood glucose tended to increase in spite of the
fact that insulin therapy had been repeatedly adapted. Therefore, the
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 tentative diagnosis of pruritic urticarial papules and plaques of pregnancy
(PUPPP) was made. A dermatologist was consulted on therapeutic 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 consequences. 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
symptoms completely resolved.
Discussion
PUPPP is a disorder that predominantly occurs in a ﬁrst pregnancy
(Powell 2000). Skin changes typical for PUPPP are erythematous,
urticarial plaques and 1–2 mm papules are usually surrounded by a
narrow and pale halo (Lawley et al. 1979). The distribution of lesions
in PUPPP is a notable clinical feature. The primary location of the
eruption is on the abdomen, frequently occurring in the striae gravidarum, which ﬁrst become itchy, then erythematous and ﬁnally urticarial. The eruption then extends on the buttocks and medial thighs.
In many patients, it remains limited to these areas, but in might
become generalised (Aronson et al. 1998). Although the abdomen
is the most commonly affected site, the periumbilical skin is often
relatively spared. Sparing of the palms, soles and face are typical of
the rash’s presentation. However, there might be a considerable variation in the morphology of the eruption (Ahmadi and Powell 2005).
The rash usually regresses within 1 week postpartum. The maternal
and fetal prognosis is unaffected. Recurrence of the eruption in subsequent pregnancies is rare.
Treatment of PUPPP is focused on the relief of pruritus. The most
common agents used are antipruritic agents, skin emollients and topical corticosteroids. Refractory cases may require oral corticosteroid
therapy (Catanzarite and Quirk 1990).
Although this syndrome has been known for several years, its
pathogenesis is still unclear. Some authors suggest an immunological mechanism; others highlight the hormonal abnormalities; others
hypothesise a mechanical factor (Aractinigi et al. 1998; Beckett and
Goldberg 1991; Vaughan Jones et al. 1999).
In our case, the hypothesis of the autoimmune mechanism
was plausible, as the patient suffered from type 1 diabetes. Her
pathology worsened during pregnancy (in the first pregnancy she
had an abortion for hyperglycaemic coma; in the second pregnancy, the blood glucose levels remained steadily increased, despite
medical treatment). Therefore, we can speculate that the pregnancy
triggered an uncontrolled immune response, also responsible for
the skin disorders. The type of treatment confirms this hypothesis,
in fact immunomodulatory drugs as corticosteroids determined
the resolution of symptoms. Resolution of the skin disorders after
delivery is another factor in favour of this hypothesis.
Furthermore, the specificity of these skin lesions with pregnancy
may also make us reconsider this dermatological syndrome.
Figure 1. Erythematous papules spreading below umbilicus within an abdominal
stria.
302 Obstetric case reports
PUPPP may not simply be regarded as a dermatological disease, but
its skin manifestations may represent a sign of a pregnancy at risk.
Accordingly, the mechanism of occurrence of these skin disorders
must have considerable clinical interest; however, there are still too
many doubts about the aetiology of PUPPP. Therefore, further studies are necessary to clarify its pathogenesis.
Declaration of interest: The authors report no conflicts of interest. The
authors alone are responsible for the content and writing of the paper.
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References
Ahmadi S, Powell FC. 2005. Pruritic urticarial papules and plaques of pregnancy:
Current status. Australasian Journal of Dermatology 46:53–60.
Aronson IK, Bond S, Fiedler VC et al. 1998. Pruritic urticarial papules and plaques
of pregnancy: Clinical and immunopathologic observations in 57 patients.
Journal of the American Academy of Dermatology 39:933–939.
Aractinigi S, Berkane N, Bertheau P et al. 1998. Fetal DNA in skin of polymorphic
eruption of pregnancy. Lancet 352:1898–1901.
Beckett MA, Goldberg NS. 1991. Pruritic urticarial papules and plaques of pregnancy and skin distension. Archives of Dermatology 127:125–130.
Catanzarite V, Quirk JG Jr. 1990. Papular dermatoses of pregnancy. Clinical
Obstetrics and Gynecology 33:754–758.
Lawley TJ, Hertz KC, Wade TR et al. 1979. Pruritic urticarial papules and plaques
of pregnancy. Journal of the American Medical Association 241:1696–1699.
Powell FC. 2000. Pruritic urticarial papules and plaques of pregnancy and multiple
pregnancies. Journal of the American Academy of Dermatology 43:730–731.
Vaughan Jones SA, Hern S, Nelson-Piercy C et al. 1999. A prospective study of 200
women with dermatoses of pregnancy correlating clinical findings with hormonal
and immunopathological profiles. British Journal of Dermatology 141:71–81.
A pregnancy complicated with fetal
growth restriction in a patient with
dystrophic epidermolysis bullosa
E. Ozkaya, E. Baser, G. Akgul & T. Kucukozkan
Department of Obstetrics and Gynaecology, Dr Sami Ulus Maternity
and Children’s Health Research and Training Hospital, Ankara, Turkey
DOI: 10.3109/01443615.2011.653595
Correspondence: E. Baser, Dr. Sami Ulus Maternity and Children’s Health
Research and Training Hospital, Babur sokak No: 44 Altindag, Ankara, Turkey.
E-mail: eralpbaser@gmail.com
Epidermolysis bullosa (EB) is a group of inherited skin diseases. We
present a case of pregnancy in a patient with dystrophic EB complicated 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 section, 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 pregnancy in these patients.
Case report
A 26-year-old primigravid woman diagnosed as dystrophic EB, was
referred to our hospital in her 17th gestational week. On family history,
she had one sister affected with EB who was deceased because of infection. Results of first visit laboratory tests (biochemistry, complete blood
count, TORCH panel) were within normal ranges. Triple test results
demonstrated low risk for chromosomal anomalies and open neural
tube defects. At the 22nd gestational week, she underwent a 2nd trimester ultrasound scan, which revealed normal fetal anatomy with appropriate growth for gestational age. She had regular visits to a dermatologist
in an associated hospital. There was no exacerbation in skin lesions at 34
weeks. Weekly non-stress tests (NST) were reactive, also amniotic fluid
index (AFI) and fetal growth were within normal ranges. The patient was
lost to follow-up for 6 weeks. She returned to our hospital at the 40th
gestational week, complaining of painful regular uterine contractions.
On pelvic examination, she had minimal cervical effacement and 1 cm
dilatation. On systemic examination, she had widespread skin erosions,
including the vulval region and vaginal mucosa, compatible with genital
involvement of EB. Ultrasound examination revealed anhydramnios
with fetal measurements compatible with 35 gestational weeks, with an
estimated fetal weight of 2,338 g. On Doppler examination, there was loss
of end-diastolic flow in umbilical artery and cerebro-placental pulsatility
index ratio was  1. These findings strongly suggested placental insufficiency with brain sparing effect. Non-stress test (NST) revealed regular
uterine contractions with persistent variable decelerations. Caesarean
section was performed due to non-reassuring fetal status. A female
neonate was delivered weighing 2,410 g. First minute Apgar score was 6
and at 5 min, the Apgar score was 7. The neonate was transferred to the
neonatal intensive care unit for close observation. The patient’s postpartum period was uneventful and without any complications. Skin lesions
were treated with topical agents and antibiotics, with the supervision of
a dermatologist. (The caesarean incision on the 2nd postoperative day is
shown in Figure 1). The woman was discharged from the hospital on the
7th postoperative day. Skin lesions were stable during the puerperium.
The neonate also did not have any important complications and was discharged from the hospital at the same day, together with the mother.
Comment
We present a pregnancy complicated with fetal growth restriction (FGR)
in a patient with dystrophic EB. It is unclear if EB has an adverse effect
on an otherwise healthy pregnancy. Such pregnancies usually end in
term delivery. There have, however, been instances where the pregnancy
results in pre-term delivery following pre-term premature rupture of
the membranes (PPROM) (Anum et al. 2009). A case of pregnancy
complicated with FGR, similar to our case, was reported previously in
a patient with genital involvement of dystrophic EB (Bianca et al. 2003).
The reported case delivered a growth restricted neonate at 36 weeks’
gestation. A question was raised in our minds whether EB is specifically
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 intraepidermal in simplex, intra-lamina lucida in junctional and sub-basal
lamina in dystrophic form. Dystrophic EB is associated with conjunctival, 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.