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Pruritic Urticarial Papules and Plaques of Pregnancy
Updated: Sep 16, 2009
Introduction
Background
Pruritic urticarial papules and plaques of pregnancy (PUPPP) is a benign dermatosis that usually arises late in the third trimester of a first pregnancy.1 The entity previously had been reported as toxemic rash of pregnancy,2 toxemic erythema of pregnancy, and late-onset prurigo of pregnancy. The term polymorphic eruption of pregnancy (PEP) is used extensively in Great Britain, while PUPPP typically is used in the United States. Following atopic eruption of pregnancy, which occurs earlier in gestation, PUPPP is the second most common dermatoses of pregnancy.3
Frequency
International
Pruritic urticarial papules and plaques of pregnancy (PUPPP) occurs in 1 out of 160-240 initial pregnancies.
Mortality/Morbidity
No mortality is associated with pruritic urticarial papules and plaques of pregnancy (PUPPP). The mere appearance of an unusual skin eruption in pregnancy can provoke anxiety, but the pruritus is the most distressing feature. The latter weeks of pregnancy can be associated with many physical symptoms, and the severe itching of PUPPP may further debilitate and aggravate sleep loss in the weeks prior to delivery. No known systemic complications exist for affected females, and fetal mortality or morbidity do not increase.
Race
Pruritic urticarial papules and plaques of pregnancy (PUPPP) may be less common in blacks.
Sex
Pruritic urticarial papules and plaques of pregnancy (PUPPP) occurs in females only.
Age
Pruritic urticarial papules and plaques of pregnancy (PUPPP) occurs during childbearing years because it is a dermatosis related to pregnancy.
Clinical
History
Pruritic urticarial papules and plaques of pregnancy (PUPPP) typically begins with intensely pruritic papules arising within striae distensae late in the third trimester of a first pregnancy. Of all cases, 73% are seen in primigravidae pregnancies.3 Additionally, 11.7 % of affected females are multiple-gestation pregnancies.4 As many as 15% of PUPPP cases arise in the immediate postpartum period.3 In a few days, the eruption spreads to the trunk and extremities. Patients present for a diagnosis of their unusual skin eruption and seek relief from the intense itching.
Physical
Classic pruritic urticarial papules and plaques of pregnancy (PUPPP) reveals papules within prominent striae distensae (see Media Files 1-2). Erythematous urticarial papules and plaques of the trunk and extremities also are observed, although the periumbilical area is spared. Small vesicles often are noted, but larger bullae do not occur and would suggest the more rare herpes gestationis. Less commonly, target lesions and annular and polycyclic wheals may be present. PUPPP usually does not affect the face, palms, or soles. Although the eruption is intensely pruritic, excoriations rarely are found. One report describes a case of PUPPP that progressed to involve the neck, arms, and legs in a photosensitive distribution as the initial abdominal involvement settled.5
Causes
The cause and pathogenesis of PUPPP are not known. A meta-analysis reveals 11.7% of patients with pruritic urticarial papules and plaques of pregnancy (PUPPP) are multiple gestation pregnancies.4 Within that group, a higher PUPPP risk for triplet (14%) over twin (2.9%) pregnancies has been published,6 suggesting a relationship between skin distension and the development of PUPPP. Most studies reveal increased maternal weight gain in patients with PUPPP when compared with normal pregnancies, further supporting the role of increased skin distension.7
A study from Israel also found maternal hypertension and induction of labor to be significantly associated with the condition.8 One large series9 of cases revealed a male-to-female infant ratio of 2:1. Investigators have identified fetal DNA in the skin of mothers with PUPPP, suggesting that chimerism might be relevant in the pathogenesis of this disorder.10 Finally, a 2008 case-control study from France confirmed previously documented associations with multiple gestations, cesarean deliveries, and male fetuses, although no relationship to maternal or fetal weight gain was noted.11
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References
Lawley TJ, Hertz KC, Wade TR, Ackerman AB, Katz SI. Pruritic urticarial papules and plaques of pregnancy. JAMA. Apr 20 1979;241(16):1696-9. [Medline].
Bourne G. Toxaemic rash of pregnancy. Proc R Soc Med. Jun 1962;55:462-4. [Medline].
Ambros-Rudolph CM, Müllegger RR, Vaughan-Jones SA, Kerl H, Black MM. The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients. J Am Acad Dermatol. Mar 2006;54(3):395-404. [Medline].
Kroumpouzos G, Cohen LM. Specific dermatoses of pregnancy: an evidence-based systematic review. Am J Obstet Gynecol. Apr 2003;188(4):1083-92. [Medline].
Goolamali SI, Salisbury JR, Higgins EM. Polymorphic eruption of pregnancy in a photodistribution: a potentially new association?. Clin Exp Dermatol. Oct 2009;34(7):e381-2. [Medline].
Elling SV, McKenna P, Powell FC. Pruritic urticarial papules and plaques of pregnancy in twin and triplet pregnancies. J Eur Acad Dermatol Venereol. Sep 2000;14(5):378-81. [Medline].
Rudolph CM, Al-Fares S, Vaughan-Jones SA, Mullegger RR, Kerl H, Black MM. Polymorphic eruption of pregnancy: clinicopathology and potential trigger factors in 181 patients. Br J Dermatol. Jan 2006;154(1):54-60. [Medline].
Ohel I, Levy A, Silberstein T, Holcberg G, Sheiner E. Pregnancy outcome of patients with pruritic urticarial papules and plaques of pregnancy. J Matern Fetal Neonatal Med. May 2006;19(5):305-8. [Medline].
Vaughan Jones SA, Hern S, Nelson-Piercy C, Seed PT, Black MM. A prospective study of 200 women with dermatoses of pregnancy correlating clinical findings with hormonal and immunopathological profiles. Br J Dermatol. Jul 1999;141(1):71-81. [Medline].
Aractingi S, Berkane N, Bertheau P, et al. Fetal DNA in skin of polymorphic eruptions of pregnancy. Lancet. Dec 12 1998;352(9144):1898-901. [Medline].
Regnier S, Fermand V, Levy P, Uzan S, Aractingi S. A case-control study of polymorphic eruption of pregnancy. J Am Acad Dermatol. Jan 2008;58(1):63-7. [Medline].
Powell AM, Sakuma-Oyama Y, Oyama N, et al. Usefulness of BP180 NC16a enzyme-linked immunosorbent assay in the serodiagnosis of pemphigoid gestationis and in differentiating between pemphigoid gestationis and pruritic urticarial papules and plaques of pregnancy. Arch Dermatol. Jun 2005;141(6):705-10. [Medline].
Ahmadi S, Powell FC. Pruritic urticarial papules and plaques of pregnancy: current status. Australas J Dermatol. May 2005;46(2):53-8; quiz 59. [Medline].
Scheinfeld N. Pruritic urticarial papules and plaques of pregnancy wholly abated with one week twice daily application of fluticasone propionate lotion: a case report and review of the literature. Dermatol Online J. Nov 15 2008;14(11):4. [Medline].
Beltrani VP, Beltrani VS. Pruritic urticarial papules and plaques of pregnancy: a severe case requiring early delivery for relief of symptoms. J Am Acad Dermatol. Feb 1992;26(2 Pt 1):266-7. [Medline].
Roger D, Vaillant L, Fignon A, et al. Specific pruritic diseases of pregnancy. A prospective study of 3192 pregnant women. Arch Dermatol. Jun 1994;130(6):734-9. [Medline].
Further Reading
Keywords
pruritic urticarial papules and plaques of pregnancy, PUPPP, polymorphic eruption of pregnancy, PEP, toxemic erythema of pregnancy, toxemic rash of pregnancy, late-onset prurigo of pregnancy, benign dermatosis, pregnancy-related dermatoses, erythematous urticarial papules, intensely pruritic papules




Overview: Pruritic Urticarial Papules and Plaques of Pregnancy