AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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. The entity previously had been reported as toxemic rash of pregnancy, 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.¹
 
The Medscape Pregnancy Resource Center may be helpful.

Frequency

International

PUPPP occurs in 1 out of 160-240 initial pregnancies.

Mortality/Morbidity

No mortality is associated with 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

PUPPP may be less common in blacks.

Sex

PUPPP occurs in females only.

Age

PUPPP occurs during childbearing years because it is a dermatosis related to pregnancy.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

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.¹ Additionally, 11.7 % of affected females are multiple-gestation pregnancies.² As many as 15% of PUPPP cases arise in the immediate postpartum period.¹ 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 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.

Causes

The cause and pathogenesis of PUPPP are not known. A meta-analysis reveals 11.7% of patients with PUPPP are multiple gestation pregnancies.² Within that group, a higher PUPPP risk for triplet (14%) over twin (2.9%) pregnancies has been published,³ 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.⁴
 
A study from Israel also found maternal hypertension and induction of labor to be significantly associated with the condition.⁵ One large series⁶ 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 disorder⁷  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.⁸

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Atopic eruption of pregnancy (includes eczema of pregnancy, prurigo of pregnancy, pruritic folliculitis of pregnancy)
Pemphigoid Gestationis (herpes gestationis)
Intrahepatic cholestasis of pregnancy
Impetigo herpetiformis 
Viral exanthem

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• No blood or urine laboratory tests are diagnostic of PUPPP.
• One large series of cases showed a significant reduction of serum cortisol levels in patients with PUPPP compared with normal pregnant controls.⁶
• Serum submitted for indirect immunofluorescence or NC16a enzyme-linked immunosorbent assay may help differentiate between PUPPP and herpes gestationis.⁹ Both studies detect autoantibodies that are present in persons with herpes gestationis but are not present in those with PUPPP.

Procedures

• Check for scabies if the history and physical examination findings are suggestive.
• Direct immunofluorescence (DIF) of skin punch biopsy specimens characteristically is negative in PUPPP. DIF results differentiate PUPPP from herpes gestationis. The latter disorder is a rare, autoimmune blistering disease characterized by linear deposits of C3 along the basement membrane zone.

Histologic Findings

Routine biopsy specimens usually reveal a normal epidermis, but focal spongiosis and parakeratosis may be seen. Within the papillary and mid dermis, a lymphohistiocytic infiltrate with a variable number of eosinophils and dermal edema is present.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

Treatment is directed at relieving the pruritus associated with PUPPP (see Medication). General measures include cool soothing baths, emollients, wet soaks, and light-cotton clothing. PUPPP tends to resolve spontaneously shortly after delivery. Morbidity is not increased for the fetus born to an affected mother.

Consultations

The health care provider responsible for the patient's obstetrical care should be made aware of the diagnosis, treatment, and prognosis of PUPPP.

MEDICATION

Section 7 of 11  

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• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

High potency (class I or II) topical corticosteroids are the mainstay of treatment of PUPPP. Occasionally, systemic corticosteroids are required to alleviate severe symptoms. Oral antihistamines are only mildly effective.

Drug Category: Corticosteroids

Have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli. Use systemic steroids for severe, refractory cases only.

Drug Category: Antihistamines

Sedative effect may improve sleep.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Outpatient Care

• PUPPP typically resolves within several weeks of delivery. Continue symptomatic care until resolution.

Prognosis

• PUPPP does not tend to recur in subsequent pregnancies. Only 7% of multiparous PUPPP patients described a similar rash with prior pregnancies.¹ It is not precipitated by the subsequent use of oral contraceptives. The prognosis for the affected woman and the newborn is excellent.

Patient Education

• The patient should understand that PUPPP is a benign disorder and has not been shown to have adverse consequences for the fetus. Fully explain the side effects of corticosteroids and antihistamines. Reassure the affected patient that the disorder does not usually recur with subsequent pregnancies and is not triggered by future use of oral contraceptives.

MISCELLANEOUS

Section 9 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Ensure diagnosis is correct. Common dermatologic disorders noted in Differentials may occur in pregnant females and should be ruled out. In addition, always consider the possibility of the more rare herpes gestationis.
• Medication usage in pregnancy requires careful assessment of risks versus benefits. Inform the patient of any risks and potential side effects.

Special Concerns

• Medications may need to be used into the postpartum period (see Medication). Pay attention to the potential adverse effects with breastfeeding.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Courtesy of Jeffrey P. Callen, MD of Louisville, Kentucky.
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Media file 2:  Courtesy of Jeffrey P. Callen, MD of Louisville, Kentucky.
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REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

1. 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].
2. Kroumpouzos G, Cohen LM. Specific dermatoses of pregnancy: an evidence-based systematic review. Am J Obstet Gynecol. Apr 2003;188(4):1083-92. [Medline].
3. 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].
4. 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].
5. 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].
6. 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].
7. Aractingi S, Berkane N, Bertheau P, Le Goue C, Dausset J, Uzan S, et al. Fetal DNA in skin of polymorphic eruptions of pregnancy. Lancet. Dec 12 1998;352(9144):1898-901. [Medline].
8. 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].
9. Powell AM, Sakuma-Oyama Y, Oyama N, Albert S, Bhogal B, Kaneko F, 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].
10. 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].
11. 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].
12. Bourne G. Toxaemic rash of pregnancy. Proc R Soc Med. Jun 1962;55:462-4. [Medline].
13. 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].
14. Roger D, Vaillant L, Fignon A, Pierre F, Bacq Y, Brechot JF, et al. Specific pruritic diseases of pregnancy. A prospective study of 3192 pregnant women. Arch Dermatol. Jun 1994;130(6):734-9. [Medline].

Article Last Updated: Mar 25, 2008