Polymorphic eruption of pregnancy: clinicopathology and potential trigger factors in 181 patients.

BACKGROUND: Polymorphic eruption of pregnancy (PEP; synonym: pruritic urticarial papules and plaques of pregnancy) is the most common specific dermatosis of pregnancy. However, its clinical characterization is controversial and its pathogenesis uncertain. OBJECTIVES: To evaluate clinical characteristics of and potential trigger factors for PEP in a large mixed ethnic population. METHODS: A retrospective analysis of epidemiological, clinical, immunopathological and obstetric findings in 181 patients with PEP seen at two university-based dermatological hospitals in Graz, Austria, and London, U.K. RESULTS: PEP mainly affected white (88%) primigravidae (70%) in late pregnancy (83%; mean +/- SD onset 34 +/- 5 weeks) or the immediate postpartum period (15%). The most commonly involved sites were the abdomen and proximal thighs (97%). Involvement of the whole skin, including the face, palms and soles, was only rarely observed. While pruritic urticarial papules and plaques were the main morphological features at disease onset (98%), more than one-half of the patients (51%) later developed polymorphous features including erythema, vesicles, and targetoid and eczematous lesions. Topical treatment with corticosteroids and emollients was sufficient to control symptoms in the majority of patients, and skin lesions resolved after a mean +/- SD of 4 +/- 3 weeks. Multiple gestation pregnancies were observed in 13% of cases, excessive maternal weight gain in 78%. CONCLUSIONS: Our data confirm the benign, self-limiting nature of PEP and its favourable outcome for both the mother and the fetus. For the first time, we have documented a characteristic change in morphology with disease progression. The evidence of polymorphous clinical features in more than one-half of the patients favours the use of the term PEP. Multiple gestation pregnancies and excessive maternal weight gain, but not fetal weight and sex, were found to be significantly associated with PEP.

Neutrophil folliculitis and serum androgen levels.

Pruritic folliculitis (PF) is one of the specific dermatoses of pregnancy the aetiology of which remains unknown. Several theories have been proposed, and the most commonly accepted is that it represents a hormonally induced acne, as seen in patients taking progestogenic steroids. This theory led to the proposition that serum androgen levels may be elevated in this condition, and may be causative. We studied a cohort of 125 pregnant women presenting with a variety of pregnancy dermatoses of whom 12 had PF. These women were compared with 138 pregnant control subjects without a pregnancy dermatosis to determine whether serum androgens were elevated. There was no significant elevation of serum androgens in the 12 cases of PF. There was an overall preponderance of male infants among the PF cases and birthweight of the infants was found to be reduced compared with those of controls. However, the outcome of pregnancy in all cases was favourable implying that PF has no adverse effect on foetal wellbeing.

A prospective study of 200 women with dermatoses of pregnancy correlating clinical findings with hormonal and immunopathological profiles.

In 1994 we set up a specialist clinic for pregnancy dermatoses, both to improve the management of pregnant women with skin problems and to enhance our general understanding of the pregnancy dermatoses. This clinic has provided a large database of 200 women which has formed the basis for a prospective study over a 2-year period. In each case the dermatological diagnosis was clearly defined on clinical criteria, with additional help from histopathology and direct immunofluorescence of the skin where appropriate. We have included a number of patients who presented with relatively trivial diagnoses, as this reflects the referral patterns of our midwives, general practitioners and obstetricians within our hospital and local population. Our results show that all patients with specific dermatoses of pregnancy conformed well to the classification established by Holmes and Black in 1983. The role of the sex hormones [oestradiol, human chorionic gonadotrophin (hCG) and cortisol] in polymorphic eruption (PEP) and prurigo of pregnancy was studied in 125 cases and compared with 138 normal healthy pregnant controls. For pruritic folliculitis (PF), serum androgens were measured to establish if these were elevated. Nearly all patients were followed up postpartum, with respect to both maternal and fetal prognosis (some were unfortunately lost to follow-up). Many patients were primiparous (47%) and presented in their third trimester (49%). This study shows a surprisingly high prevalence of eczema during pregnancy. It is possible that earlier cases in the literature termed prurigo of pregnancy may in fact have been eczema, thus explaining the low incidence of prurigo in this study. Hormonal analysis showed a significant reduction in serum cortisol levels in patients with PEP compared with normal pregnant controls (P = 0.03), although hCG and oestradiol showed no differences. Serum androgens were not significantly elevated in patients with PF compared with controls. Birthweight (analysed by the individualized birthweight ratio) was significantly reduced in both the PF and pemphigoid gestationis groups. In the PEP and PF groups there was a male/female infant ratio of 2 : 1, not noted in previous studies. In all cases studied there were no adverse effects either on maternal or fetal outcome as a result of the pregnancy dermatosis. This study indicates that all patients fulfilled the criteria of the previous classification of the specific dermatoses of pregnancy, although we also now highlight the frequency of eczema in pregnancy and speculate as to possible causes. There were no cases of papular dermatitis of pregnancy. We feel that the specialist clinic is an important service which has improved the management of these women and identified areas for further research.

Pemphigus vulgaris in pregnancy with favourable foetal prognosis.

Pemphigus vulgaris is an immunobullous disease affecting the skin and mucous membranes most commonly during the fifth and sixth decades of life. Its occurrence in pregnancy is rare. We now report two severe cases of the disorder presenting during pregnancy and discuss its potential effects on the foetus and its management in pregnancy.

A typical case of pemphigoid gestationis with a unique pattern of intercellular immunofluorescence.

Pemphigoid gestationis is an autoimmune bullous disorder affecting pregnant women which typically presents in the second and third trimester of pregnancy with an itchy bullous eruption on the abdomen and limbs. The diagnosis is confirmed by the presence of complement-binding IgG antibasement membrane zone (BMZ) antibodies, which bind to the roof of salt-split skin. We describe a case of clinically typical pemphigoid gestationis with the combination of intercellular IgG and BMZ IgG and C3 staining on immunofluorescence, a pattern which is unique for this condition.

Acrokeratosis paraneoplastica: Bazex syndrome.

Bazex syndrome, or acrokeratosis paraneoplastica, is a cutaneous paraneoplastic syndrome characterized by psoriasiform lesions associated with, usually, a squamous cell carcinoma of the upper aerodigestive tract. We present a case of Bazex syndrome associated with metastatic cervical squamous cell carcinoma with an unknown primary. The features of the condition are discussed in the light of current knowledge.

Pruritic urticarial papules and plaques of pregnancy (polymorphic eruption of pregnancy): two unusual cases.

We report two unusually florid cases of polymorphic eruption of pregnancy (PEP) or pruritic urticarial papules and plaques of pregnancy, the first occurring in a triplet pregnancy, and the second with some clinical features more suggestive of pemphigoid gestationis (PG). The first case responded to treatment with moderately potent topical steroids while the second case required a short course of systemic steroids. Our first case is the only reported case of PEP occurring during a triplet pregnancy. We discuss the effect of multiple pregnancy on PEP and some distinguishing factors between PEP and PG.

Cicatricial alopecia occurring in two sisters from Ghana.

We report two sisters from Ghana who presented with cicatricial alopecia simultaneously. In both cases the aetiology is unclear although both gave a history of previous scalp folliculitis. We classify the causes of cicatricial alopecia and the difficulties that can arise in placing such patients in a clinical category. Racial origin may be relevant in our cases; to our knowledge this is the first report of cicatricial alopecia occurring in two sisters.

Chronic verrucous varicella-zoster infection in a patient with AIDS.

The expression of herpes varicella zoster virus in patients already infected with the human immunodeficiency virus (HIV) provides the perfect illustration of an opportunist microbe at work. There is an increased incidence of herpes zoster which may be more severe, recurrent or disseminated. Likewise, varicella may be more severe and recurrent. In both patterns of infection atypical lesions in the form of persistent ulcerative or verrucous lesions have been seen. An HIV-positive patient is described who developed verrucous lesions de novo, 4 months after her child had varicella.

A retrospective analysis of tissue-fixed immunoreactants from skin biopsies maintained in Michel's medium.

656 skin biopsies with positive direct immunofluorescence from the UK and overseas were studied over a 2-year period. The length of time biopsies had remained in Michel's medium at pH 7.0 in various diseases (pemphigoid, pemphigus, linear IgA disease, epidermolysis bullosa acquisita, lupus erythematosus, vasculitis, amyloid, lichen planus and dermatitis herpetiformis) was analysed. We concluded that direct immunofluorescence remained positive at 6 months and that Michel's medium is a reliable long-term maintenance medium for skin biopsies.