31y/o Caucasian female ADAF G1P0 30.5 weeks. Was seen by WHNP for ROB. Patient had rash on abdomen/back diagnosis tinea and pt given fungal topical and dermatology referral. Patient had no improvement with fungal cream and lesions progressed to vesicles when derm provider assessed her. When patient presented to dermatology her rash had also spread to soles of feet, feet, arms, hands and all sections of her chest, abdomen and back. Patient was started on topical steroid by derm provider and lesion biopsies obtained. Differential diagnosis included Pemphigoid gestationis (he was pretty sure this would be confirmed by biopsies as patient was classic presentation per his reference text). I saw patient with OB MD. Preliminary biopsy results were available and they were not consistent with diagnosis of pemphigoid gestationis; “had eosinophilic characteristics”. Patient diagnosis dermatitis; current biopsies to be sent to AFIP for second opinion, Vistaril 100mg po q6h for sleep and continue topical steroid for lesions. Topical steroid seemed to be working—size of lesions was decreasing and fewer vesicular lesions observed. She had previously tried OTC Benadryl but it did not provide pain relief or help her sleep. Patient to start NSTs twice a week. OB provider to consult with dermatology to ensure biopsies were correctly collected (couldn’t be placed in formalin for this diagnosis). Patient was still having a hard time sleeping and even walking because of the lesions on her feet or those rubbing on her uniform. She was the last patient I saw during my rotation and I do not know her definitive diagnosis.
Pemphigoid gestationis (Medscape, 2010)
Pemphigoid gestationis (PG) is a rare autoimmune bullous dermatosis of pregnancy. The disease was originally named herpes gestationis on the basis of the morphological herpetiform feature of the blisters, but this term is a misnomer because pemphigoid gestationis is not related to or associated with any active or prior herpes virus infection.
Pemphigoid gestationisis a pregnancy-associated autoimmune disease. Most patients develop antibodies against 2 hemidesmosomal proteins, BP180 (BPAG2, collagen XVII) and less frequently BP230. Historically known as herpes gestationis factor, these circulating antibodies belong to the heat-stable immunoglobulin G1 subclass. The binding of immunoglobulin G to the basement membrane triggers an immune response, leading to the formation of subepidermal vesicles and blisters. The trigger for the development of autoantibodies in persons with pemphigoid gestationis remains elusive. Cross-reactivity between placental tissue and skin has been proposed to play a role. Pemphigoid gestationis has a strong association with HLA-DR3 (61-80%) and HLA-DR4 (52%), or both (43-50%), and virtually all patients with a history of pemphigoid gestationis have demonstrable anti-HLA antibodies. The placenta is known to be the main source of disparate (paternal) antibodies and can thus present an immunologic target during gestation.
In the United States, pemphigoid gestationis has an estimated prevalence of 1 case in 50,000-60,000 pregnancies.
International
Findings from European studies suggest that pemphigoid gestationis has an overall incidence of 0.5 cases per million people per year. In 1999, Jenkins et al2 described the largest cohort of 87 patients in the United Kingdom with a total of 278 pregnancies, of which 142 were complicated by pemphigoid gestationis.
No increase in fetal or maternal mortality has been demonstrated. A greater prevalence of premature and small-for-gestational-age (SGA) babies is associated with pemphigoid gestationis. Of infants, 5-10% born to affected mothers may present with transient cutaneous involvement that resolves as maternal autoantibodies are cleared. Patients with pemphigoid gestationis have a higher relative prevalence of other autoimmune diseases, including Hashimoto thyroiditis, Graves disease, and pernicious anemia, which are also associated with HLA-DR3 and DR-4 haplotypes
Pemphigoid gestationis is less common among blacks than whites, which might reflect its association with specific HLA haplotypes.
This condition only affects females.
Pemphigoid gestationis occurs in women of childbearing age.
Pemphigoid gestationis typically manifests during late pregnancy, with an abrupt onset of extremely pruritic urticarial papules and blisters on the abdomen and trunk. Unrelenting pruritus often interferes with daily activities. Lesions may appear any time during pregnancy, but they most commonly develop during the second and third trimesters.Symptoms may abate at the end of pregnancy; however, dramatic flares can occur at or immediately after delivery. Pemphigoid gestationis usually resolves spontaneously within weeks to months after delivery and possibly quicker with breastfeeding. The persistence of disease activity for years postpartum has been reported. Pemphigoid gestationis may recur with the resumption of menses, use of oral contraception, and subsequent pregnancies. The 1999 cohort study by Jenkins et al2 showed no association between change in partner and development of pemphigoid gestationis in subsequent pregnancies.
The initial clinical manifestations are erythematous urticarial patches and plaques, which are typically periumbilical. These lesions progress to tense vesicles and blisters. Some patients may present with urticarial plaques and may never develop blisters (see the images below). These hive-like plaques differ from true urticaria because of their relatively fixed nature. The rash spreads peripherally, often sparing the face, palms, and soles. Mucosal lesions occur in less than 20% of cases. Patients may have secondary infections at blister sites.
Differential Diagnoses
Bullous Pemphigoid
Cicatricial Pemphigoid
Linear IgA Dermatosis
Pruritic Urticarial Papules and Plaques of Pregnancy
Urticaria, Acute
Other Problems to Be Considered
Allergic contact dermatitis
Dermatitis herpetiformis
Drug-induced bullous disorders
Erythema multiforme
Papular dermatitis of pregnancy
Prurigo gestationis of Besnier
Pruritic folliculitis of pregnancy
Routine laboratory studies are not helpful in diagnosing pemphigoid gestationis. The results with most hematologic studies are within normal limits, although peripheral eosinophilia is not uncommon and may correlate with disease severity. Laboratory values that may be elevated include immunoglobulin levels, erythrocyte sedimentation rates, acute phase reactant levels, and antithyroid antibodies.
The criteria for the diagnosis of pemphigoid gestationis include an appropriate clinical presentation, histologic findings of a subepidermal blistering process (as described below), and direct immunofluorescence (DIF) results that show a linear band of C3 deposition with or without immunoglobulin G (present in 20-25% of patients) along the basement membrane. The DIF test is the key assay to differentiate pemphigoid gestationis (positive DIF findings) from pruritic urticarial papules and plaques of pregnancy (negative DIF findings). However, a similar pattern of DIF results is observed in patients with pemphigoid gestationis, BP, and epidermolysis bullosa acquisita (EBA). DIF should be performed using samples from noninvolved perilesional skin.
http://emedicine.medscape.com/article/1063499-overview (for pictures and more detail)
Interesting case. Never saw it before and the pics available on google do resemble herpes, as you mentioned that it used to be dubbed "herpes gestationis" but since has been renamed due to misnomer association with HSV. Unlike HSV which follows a dermatome, this presents as an eventual generalized rash with a slightly different presentation. Good in helping differentiate. It seems quite a few autosomal disorders tend to surface during pregnancy, or they subside. I understand this is restricted to women during childbearing years but I wonder if there is a similar form of such autoimmune disorder in men. It's bad enough we get stretch marks and permenant body changes with pregnancy, there should be a sympathy syndrome out there that men have to experience....oh that's right...our mood swings. Thorough Case and explanation of disease process, tx, etc...
ReplyDeleteI've never seen this either - my immediate reaction reading your opening sentence was "PUPP", but then I looked at the pictures, and nope, absolutely not. Thanks!
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