==== Front Ann Saudi MedAnn Saudi MedAnnals of Saudi Medicine0256-49470975-4466King Faisal Specialist Hospital and Research Centre 1805912710.5144/0256-4947.2007.453asm-6-453Case ReportPemphigus vulgaris in a neonate Amer Yousef Bin *Al Ajroush Waleed † * Section of Dermatology, Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia † Department of Medicine, King Abdulaziz Medical City, Riyadh, Saudi ArabiaCorrespondence and reprint requests: Yousef Bin Amer, MD, Dermatology Section, Department of Medicine, King Faisal Specialist Hospital and Research Centre, MBC 46, P.O. Box 3354, Riyadh 11211, Saudi Arabia, dr.binamer@gmail.comNov-Dec 2007 27 6 453 455 01 4 2007 Copyright © 2007, Annals of Saudi Medicine2007This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. ==== Body Pemphigus vulgaris (PV) is a common blistering disease in Western countries but neonatal pemphigus (NP) is rare.1,3,5,6 It is characterized by multiple skin and mucosal erosions and is caused by tranplacental passage of maternal antibodies against desmoglein.3 It heals spontaneously in two to three weeks. CASE A 3-hour-old baby boy was referred with multiple skin erosions on the left side of the neck, upper left chest and groin without oral involvement (Figure 1A). He was born by normal vaginal delivery without complication with an APGAR score of 8 and 9 at 1 and 5 minutes, respectively, and a weight of 2.57 kg. His mother was known to have PV since 1999, which was proven by biopsy and immunofluorescence. The product of her first pregnancy in 1999 was a baby boy with multiple erosions. The first baby was diagnosed with NP based on the results of a skin biopsy and immunofluorescence. The erosions healed spontaneously in two weeks. The mother was treated with prednisone, and later dapsone was added. She went into complete remission. She went off treatment in July 2001 and developed a new flare of PV in June 2005 during her second pregnancy. She was treated with prednisone 40 mg every other day. Physical examination of the second baby showed multiple erosions over the left side of the neck, shoulder and groin. Skin biopsy was refused by the parents and indirect immunofluorescence showed 1:10. He was treated with topical fucidin and 1% hydrocortisone, and discharged three days after delivery in good condition. At the age of four weeks, he had complete clearance (Figure 1B). DISCUSSION PV is the most common form of pemphigus.5 It is usually seen in the fifth decade of life.2,5 Neonatal PV is a rare disease1,3,6 seen soon after birth and characterized by cutaneous, mucosal or mucocutaneous erosions. PV is diagnosed by histological and immunofluorescence studies. Routine histology will show epidermal basal cell vacuolization and spongiosis with exocytosis of polymorphonuclear leucocytes and eosinophils. Immunofluorescence will show IgG and C3 deposition. Indirect immunofluorescence is positive in patients with active disease. A high titre indicates active disease. 2 It usually resolves spontaneously in 2 to 3 weeks.2–5,7,9,12,13,19–21 Neonatal PV results from transplacental passage of IgG maternal autoantibodies, mainly class 4, against desmoglein 3 (Dsg3), a transmembrane glycoprotein of the cadherin family.1,3,4,5,7,8,11,20,23–27 More than 21 cases of neonatal PV have been reported (Table 1), with the first proven one in 1975 by Rucco et al.28 However, there are only 3 reported cases of pemphigus foliaceus (PF).15–17 This difference in incidence may be related to the distribution of Dsg1 and 3 in neonatal skin. Dsg1, the protein affected in PF, is present throughout the epidermis in both adults and neonates, while type 3 is present in the basal and intermediate suprabasal layer in adults, but throughout the epidermis in neonates. So the frequency of neonatal PF is less than PV in babies born to mothers with pemphigus based on the desmoglein compensation theory.5,18 This is supported by at least 18 cases of pregnant women with PF who delivered babies free of the disease.29,30 There is no correlation between the titer of maternal PV antibodies and the presentation of the disease in neonates.2,5,6 This is supported by cases of neonatal PV from mothers with no active disease5,9,31 and cases of healthy neonates born to mothers with highly active disease.4–6,16,17,32–36 The mother might have only oral disease while the baby has both oral and cutaneous involvement.5,36 Stillbirths have been also reported. 23,24,31 Figure 1a At birth, multiple eroded vesicles at upper chest and left side of the neck. Figure 1b At age four weeks with complete healing. Table 1 Reported cases of neonatal pemphigus vulgaris. Reference number Mother’s Disease Recovery Duration Treatment DIF** IDIF* Site Sex 1 Severe 2 weeks Like above IgG and C3 1/160 Skin and oral cavity F 3 Mild 2 weeks Emollient and mupirocin IgG and C3 1/160 Skin and tongue F 6 Unknown 2 weeks Like above NA NA Skin F 7 Mild 2 weeks Like above IgG and C3 1/160 Skin M 8 Unknown 3 weeks Like above NA 1/20 Skin F 9 Mild 2 weeks No treatment IgG and C3 1/40 Skin and occipit M 10 Mild 2 weeks Emollient and mupirocin IgG and C3 1:80 Skin F 12 Severe 2 weeks No treatment IgG 1/20 Oral M 13 Mild 3 weeks No treatment NA 1/20 Skin, tongue and scalp F 15 Unknown 3 weeks Like above IgG 1/32 Skin and occipit M 22 Mild 2 weeks No treatment IgG NA Skin F 34 Unknown 2 weeks Like above IgG and C3 Neg Skin and scalp F * Indirect immunoflurescence ** direct immunoflurescence Rx: treatment, NA: Not available, Neg: negative result, M: male, F: female ==== Refs REFERENCES 1 Shieh S Frang YV Becker JL Holm A Beutner EH Helm TN Pemphigus, pregnancy and plasma-pheresis Cutis 2004 5 73 5 327 9 15186047 2 Bjarnason B Flosadottir E Childhood, neonatal and stillbirth pemphigus vulgaris Int J Dermatol 1999 38 680 688 10517685 3 Chowdhury MM Natarajan S Neonatal pemphigus vulgaris associated with mild oral pemphigus vulgaris in the mother during pregnancy Br J Dermatol 1998 9 139 3 500 3 9767299 4 Singalavanija S Limpongsanurak W Immunobullous disease in Thai children J Med Assoc Thai 2003 8 86 Suppl 3 S681 8 14700167 5 Campo-Voegeli C Muniz F Mascaro JM Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphigus vulgaris Br J Dermatol 2002 10 147 4 801 5 12366435 6 Kalayciyan A Engin B Serdaroglu S Mat C Aydemir EH Kotogyan A Br J Dermatol 2002 8 147 2 396 7 12174129 7 Parlowsky T Welzel J Amagai M Zillikens D Wygold T Neonatal pemphigus vulgaris: IgG4 autoantibodies to desmoglein 3 induce skin blisters in newborns J Am Acad Dermatol 2003 4 48 4 623 5 12664033 8 Ding X Diaz LA Fairley JA Giudice Gj Liu Z The anti-desmoglein 1 autoantibodies in pemphigus vulgaris sera are pathogenic J Invest Dermatol 1999 5 112 5 739 43 10233765 9 Tope WD Kamino H Briggaman RA Rico MJ Prose NS Neonatal pemphigus vulgaris in a child born to a woman in remission J Am Acad Dermatol 1993 9 29 3 480 5 8349868 10 Grunwald MH Zamora E Avinoach I Pemphigus neonatorum Pediatr Dermatol 1993 6 10 2 169 70 8346114 11 Hup JM Bruinsma RA Boersma ER de Jong MC Neonatal pemphigus vulgaris: transplacental transmission of antibodies Pediatr Dermatol 1986 12 3 6 468 72 3550750 12 Merlob P Metzker A Hazaz B Rogovin H Reisner SH Neonatal pemphigus vulgaris Pediatrics 1986 12 78 6 1102 5 3537950 13 Storer JS Galen WK Nesbitt LT Jr Deleo VA Neonatal pemphigus vulgaris J Am Acad Dermatol 1982 5 6 5 929 32 7047587 14 Fainaru O Mashiach R Kupferminc M Shenhay M Pauzner D Lessing JB Pemphigus vulgaris in pregnancy: a case report and review of literature Hum Reprod 2000 5 15 5 1195 7 10783377 15 Hirsch R Anderson J Weinberg JM Neonatal pemphigus foliaceus. 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