Neonatal pemphigus vulgaris in a child born to a woman in remission

doi:10.1016/0190-9622(93)70216-G

Journal of the American Academy of Dermatology

Volume 29, Issue 3, September 1993, Pages 480-485

https://doi.org/10.1016/0190-9622(93)70216-G Get rights and content

We describe the tenth reported case of neonatal pemphigus that mimicked Bart's syndrome and review previously published cases. Unlike previous cases, the child was born with significant blistering to a mother who was in complete remission throughout the pregnancy. High antepartum maternal titers of antiintercellular space antibodies, increased maternal disease activity, and maternal disease that requires high doses of corticosteroids or use of combined therapy correlate with poor fetal outcome, including intrauterine death.

References (31)

DE Green et al.
Maternal pemphigus vulgaris with in vivo bound antibodies in the stillborn fetus
J Am Acad Dermatol
(1982)
JS Storer et al.
Neonatal pemphigus vulgaris
J Am Acad Dermatol
(1982)
Y Takahashi et al.
Experimentally induced pemphigus vulgaris in neonatal BALB/c mice: a time-course study of clinical, immunologic, ultrastructural, and cytochemical changes
J Invest Dermatol
(1985)
LM Solomon et al.
Neonatal dermatology I: the newborn skin
J Pediatr
(1970)
AT Lane et al.
Identification of bullous pemphigoid, pemphigus, laminin, and anchoring fibril antigens in human fetal skin
J Invest Dernatol
(1985)
LA Lee et al.
The demonstration of SSA/Ro antigen in human fetal tissues and in neonatal and adult skin
J Invest Dermatol
(1985)
K Hashimoto et al.
Transient bullous dermolysis of the newborn: two additional cases
J Am Acad Dermatol
(1989)
EM Dugan et al.
Pemphigus
JHS Smitt
Pemphigus vulgaris in childhood: clinical features, treatment, and prognosis
Pediatr Dermatol
(1985)
V Ruocco et al.
Patomorfosi del pem-figo in primigravida
G Ital di Dermatol-Min Dermatol
(1977)

V Ruocco et al.

A congenital acantholytic bullous eruption in the newborn infant of a pemphigus mother

Ital Gen Rev Dermatol

(1975)

H Terpstra et al.

In vivo bound pemphigus antibodies in a stillborn infant: passive intrauterine transfer of pemphigus vulgaris?

Arch Dermatol

(1979)

B Moncada et al.

Neonatal pemphigus vulgaris: role of passively transferred pemphigus antibodies

Br J Dermatol

(1982)

N Wasserstrum et al.

Transplacental transmission of pemphigus

JAMA

(1983)

J Middelkamp Hup et al.

Neonatal pemphigus vulgaris: transplacental transmission of antibodies

Pediatr Dermatol

(1986)

Cited by (44)

Bullous diseases: Kids are not just little people
2015, Clinics in Dermatology
Citation Excerpt :
In adults, desmoglein-3 is localized to the mucous membranes and the deep epidermis, whereas desmoglein-1 is localized to the more superficial epidermis, specifically the granular layer; hence, adults with antibodies only against desmoglein-3 will have disease localized to the mucosa because the unaffected desmoglein-1 can maintain the integrity of the skin.98,99 The majority of newborns with neonatal pemphigus are born to mothers with clinical symptoms; however, it has been reported in mothers in clinical remission.100,101 Antibody titers in the mother have ranged from 1:20 to 1:640, and several authors suggest that neither antibody titers nor the clinical severity of the mother’s findings predict the severity of disease in the child.99,102,103
Bullous diseases may be rare; however, this does not preclude the clinician from being familiar with their manifestations and treatment. After ruling out infection, genetically inherited blistering diseases are more likely to be the cause of blistering or erosions in the neonatal period, whereas immunobullous diseases are more common in adults. Published literature on immunobullous disorders reflects information gleaned from case reports and open-label case series; prospective studies and evidence-based treatments are limited. Although there may be overlapping clinical features, significant clinical differences exist between adults and children. Evidence-based treatment guidelines are limited, and information from the adult literature cannot be readily generalized to the pediatric population. This paper reviews the approach to blistering conditions and the differences among bullous pemphigoid, linear immunoglobulin A disease, dermatitis herpetiformis, pemphigus foliaceus, pemphigus vulgaris, and paraneoplastic pemphigus in adult versus pediatric patients.
The vesiculobullous reaction pattern
2009, Weedon's Skin Pathology: Third Edition
Effect of Pregnancy on Other Skin Disorders
2008, Obstetric and Gynecologic Dermatology
Vesicles, Pustules, Bullae, Erosions, and Ulcerations
2008, Neonatal Dermatology
Oral and vulvar changes in pregnancy
2006, Clinics in Dermatology
The endocrinologic, immunological, and vascular changes that occur during pregnancy are far-reaching. These systemic factors produce profound local impact on the physiology and pathology of the oral cavity, vagina, and vulva. Physiological changes can be expected and tolerable or of such severity that they are viewed as pathological. Existing disease can be exacerbated and dermatoses specific to pregnancy can erupt. Each of these conditions can pose potentially challenging treatment considerations.
Oral mucosal lesions during pregnancy and in the postpartum period
2022, ENT Diseases: Diagnosis and Treatment during Pregnancy and Lactation

View all citing articles on Scopus

^a: From the Division of Dermatology, Duke University Medical Center

^b: From the Department of Pathology, Duke University Medical Center

^c: From the Department of Dermatology, University of North Carolina at Chapel Hill.

View full text

Neonatal pemphigus vulgaris in a child born to a woman in remission

J Am Acad Dermatol

J Am Acad Dermatol

J Invest Dermatol

J Pediatr

J Invest Dernatol

J Invest Dermatol

J Am Acad Dermatol

Pemphigus

Pemphigus vulgaris in childhood: clinical features, treatment, and prognosis

Pediatr Dermatol

Patomorfosi del pem-figo in primigravida

G Ital di Dermatol-Min Dermatol

A congenital acantholytic bullous eruption in the newborn infant of a pemphigus mother

Ital Gen Rev Dermatol

In vivo bound pemphigus antibodies in a stillborn infant: passive intrauterine transfer of pemphigus vulgaris?

Arch Dermatol

Neonatal pemphigus vulgaris: role of passively transferred pemphigus antibodies

Br J Dermatol

Transplacental transmission of pemphigus

JAMA

Neonatal pemphigus vulgaris: transplacental transmission of antibodies

Pediatr Dermatol