Pruritic Urticarial Papules and Plaques of Pregnancy

Author: Joseph C Pierson, MD, Consulting Staff, Department of Dermatology, Guthrie Army Clinic, Ft Drum, NY
Coauthor(s): Christine C Tam, MD, Staff Physician, Dermatology Office of David A Spott, MD

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) occur 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) occur in females only.

Age

Pruritic urticarial papules and plaques of pregnancy (PUPPP) occur during childbearing years because it is a dermatosis related to pregnancy.

Clinical

History

Pruritic urticarial papules and plaques of pregnancy (PUPPP) typically begin 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, as shown in the images below.

Courtesy of Jeffrey P. Callen, MD of Louisville, ...

Courtesy of Jeffrey P. Callen, MD of Louisville, Kentucky.

 

Courtesy of Jeffrey P. Callen, MD of Louisville, ...

Courtesy of Jeffrey P. Callen, MD of Louisville, Kentucky.

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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, though documented in one case, typically do not occur and would suggest the possibility of herpes gestationis.5 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.6

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,7 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.8

A study from Israel also found maternal hypertension and induction of labor to be significantly associated with the condition.9 One large series10 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.11 Finally, a 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.12

Differential Diagnoses

Contact Dermatitis, Allergic
Scabies
Contact Dermatitis, Irritant
Seabather's Eruption
Drug Eruptions
Urticaria, Acute
Erythema Multiforme
Urticaria, Chronic
Insect Bites
 

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

Laboratory Studies

  • No blood or urine laboratory tests are diagnostic of pruritic urticarial papules and plaques of pregnancy (PUPPP).
  • One large series of cases showed a significant reduction of serum cortisol levels in patients with PUPPP compared with normal pregnant controls.10
  • Serum submitted for indirect immunofluorescence or NC16a enzyme-linked immunosorbent assay may help differentiate between PUPPP and herpes gestationis.13 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 pruritic urticarial papules and plaques of pregnancy (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 from pruritic urticarial papules and plaques of pregnancy (PUPPP) 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.14

Treatment

Medical Care

Treatment is directed at relieving the pruritus associated with pruritic urticarial papules and plaques of pregnancy (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 healthcare provider responsible for the patient's obstetrical care should be made aware of the diagnosis, treatment, and prognosis of pruritic urticarial papules and plaques of pregnancy (PUPPP).

Medication

Topical corticosteroids are the mainstay of treatment for pruritic urticarial papules and plaques of pregnancy (PUPPP).15 High-potency topical (class I or II), or even systemic, steroids may be required to alleviate symptoms. Oral antihistamines are only mildly effective.

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.

 

Fluocinonide (Fluonex, Lidex)

Class II topical steroid. High-potency, topical, corticosteroid that inhibits cell proliferation; is immunosuppressive and anti-inflammatory.

Adult

Apply sparingly bid/qid as severity warrants

Pediatric

Apply as in adults

None reported

Documented hypersensitivity; herpes simplex infection; fungal, viral, or tubercular skin lesions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May cause adverse systemic effects if used over large areas, denuded areas, on occlusive dressings, or during prolonged treatment periods; do not apply to areola in breastfeeding; use weaker topical steroid (hydrocortisone) for facial or intertriginous involvement

 

Prednisone (Deltasone)

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Adult

0.5-1 mg/kg/d PO

Pediatric

1-2 mg/kg PO qd or divided bid/qid; taper over 2 wk as symptoms resolve

Coadministration with estrogens may decrease prednisone clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; severe gestational diabetes or hypertension

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use

Antihistamines

Sedative effect may improve sleep.

 

Diphenhydramine (Benadryl, Belix)

For symptomatic relief of pruritus caused by release of histamine in inflammatory reactions.

Adult

25-50 mg PO qhs

Pediatric

12.5-25 mg PO tid/qid, or 5 mg/kg/d, or 150 mg/m2 /d divided tid/qid; not to exceed 300 mg/d

Follow-up

Further Outpatient Care

  • Pruritic urticarial papules and plaques of pregnancy (PUPPP) typically resolve within several weeks of delivery. Continue symptomatic care until resolution.

Complications

  • An older case report describes early cesarean delivery to relieve a severe case of pruritic urticarial papules and plaques of pregnancy (PUPPP).16

Prognosis

  • Pruritic urticarial papules and plaques of pregnancy (PUPPP) do not tend to recur in subsequent pregnancies. Only 7% of multiparous PUPPP patients described a similar rash with prior pregnancies.3 PUPPP is not precipitated by the subsequent use of oral contraceptives. The prognoses for the affected woman and the newborn are excellent.

Patient Education

  • The patient should understand that pruritic urticarial papules and plaques of pregnancy (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 PUPPP does not usually recur with subsequent pregnancies and is not triggered by future use of oral contraceptives.

Miscellaneous

Medicolegal 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.

References

  1. 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] .

  2. Bourne G. Toxaemic rash of pregnancy. Proc R Soc Med . Jun 1962;55:462-4. [Medline] .

  3. Ambros-Rudolph CM, Mullegger 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] .

  4. Kroumpouzos G, Cohen LM. Specific dermatoses of pregnancy: an evidence-based systematic review. Am J Obstet Gynecol . Apr 2003;188(4):1083-92. [Medline] .

  5. Sherley-Dale AC, Carr RA, Charles-Holmes R. Polymorphic eruption of pregnancy with bullous lesions: a previously unreported association. Br J Dermatol . Nov 3 2009;[Medline] .

  6. 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] .

  7. 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] .

  8. 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] .

  9. 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] .

  10. 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] .

  11. 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] .

  12. 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] .

  13. 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] .

  14. 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] .

  15. 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] .

  16. 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] .

  17. 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] .

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