Herpes Gestationis

Algorithm(s)

PDF algorithm(s) available at www.arupconsult.com.

Immunobullous Skin Diseases Testing Algorithm

Clinical Background

Pemphigoid gestationis (herpes gestationis) is a rare disease of pregnancy and the puerperium.

Epidemiology

Incidence - 2/100,000 pregnancies
Age of onset - childbearing years
Sex - only females

Risk Factors

Previous pregnancy with herpes gestationis

Pathology and Immunopathology

Subepidermal blister
Linear C3 at the basement membrane zone on direct immunofluorescence of perilesional tissue in all cases; also linear IgG in 25%
Complement fixing IgG basement membrane zone antibodies (herpes gestationis factor, HGF) in serum in 50% showing epidermal localization on split skin substrate
IgG BP180 (BP Ag2) and, less commonly, IgG BP230 (BP Ag1) antibodies present

Clinical Presentation

Typically occurs in the second to third trimester and resolves within several weeks of delivery
Variable skin lesions ranging from urticarial to vesicles to tense blisters on skin
- Often involves palms and soles
- Usually spares mucosa
Pronounced pruritus
May reoccur during:
- Subsequent pregnancies; may occur earlier and with greater severity
- Menstrual cycles
- Hormonal treatment
- Molar pregnancy
Infants of affected mothers may be small and have lesions from transplacental antibodies

Treatment

Prednisone

See Also

Dermatitis Herpetiformis

Epidermolysis Bullosa Acquisita

Immunobullous Skin Diseases Screening

Linear IgA Disease

Paraneoplastic Pemphigus

Pemphigoid

Pemphigus

Algorithm(s)

PDF algorithm(s) available at www.arupconsult.com.

Immunobullous Skin Diseases Testing Algorithm

Tests

Tests generally appear in the order most useful for common clinical situations

Test name: Cutaneous Direct Immunofluorescence, Biopsy

ARUP #: 0092572

Methodology: Direct Immunofluorescence

Use:

Determine presence and staining pattern of immunoglobulins (IgG, IgM, IgA), third component of complement (C3) and fibrinogen in perilesional skin or mucosal biopsy specimens from patients suspected of having pemphigoid (herpes) gestationis

Limitations:

Test may be inaccurate if tissue is not taken from correct location; type and age of lesion may also be important; specimen must have epidermis/epithelium and basement membrane zone

Tissue must be submitted in Michel’s or Zeus medium; this test cannot be performed on formalin-fixed tissue

Follow-up:

Concurrent serum testing for herpes gestationis factor is most specific for diagnosis

Test name: Herpes Gestationis Factor (IgG Complement-Fixing Basement Membrane Zone Antibodies)

ARUP #: 0092283

Methodology: Indirect Immunofluorescence

Use:

Order for patients suspected of having or known to have any type of pregnancy-related skin eruption (blisters) to:
- Diagnose pemphigoid (herpes) gestationis
- Distinguish pruritic dermatoses of pregnancy
- Monitor disease activity

Test includes complement-fixing basement membrane zone antibodies and IgG BP180 antibodies

Limitations:

About 50% of cases have serum HGF; perilesional skin biopsy for direct immunofluorescence is also helpful for diagnosis

Other immunobullous diseases may occur in pregnancy with overlapping clinical features; therefore, consider concurrent testing for pemphigus (Pemphigus Panel), pemphigoid linear IgA disease (Pemphigoid Panel), and dermatitis herpetiformis (IgA endomysial antibodies)

Follow-up:

Perilesional skin biopsy by direct immunofluorescence is helpful in diagnosis (>90% of herpes gestationis cases are positive)

Additional Information

Because of overlap among immunobullous diseases with similar names, testing for pemphigoid (herpes) gestationis may be confused with testing for other immunobullous diseases and misordered. The pemphigoid group is often confused with the pemphigus group.