A 1-day-old boy weighing 2900 grams, born after full term pregnancy, was noticed to have multiple flaccid bullae on the face, scalp, trunk and extremities with clear fluid and few areas of erosions. All lesions showed a rim of erythema and abrupt demarcation from the surrounding normal skin (Fig. 1). These lesions were not restricted to trauma-prone areas. A few lesions had profuse serous discharge. Nails and oral mucosa were not involved. Mother of this infant was diagnosed to have oral pemphigus vulgaris 8 months before conception, documented by incisional biopsy from buccal mucosa and direct immunofluorescence test (DIF), and was on daily oral steroids. The child was suspected to have neonatal pemphigus vulgaris based on the morphology and distribution of skin lesions, and maternal history. A differential diagnosis of herpes simplex, candidosis, syphilis, infectious mononucleosis and epidermolysis bullosa were also considered. Tzanck smear was negative for multinucleated giant cells and acantholytic cells. Mother’s VDRL test was negative. Skin biopsy was deferred as the lesions were drying up on second day. Child was managed with warm saline compresses, barrier nursing, topical antibiotics and breast feeds supplemented with formula feeds. Intravenous fluids were not required. Fluid input, output and electrolytes were monitored regularly. All the lesions resolved at the end of one week.

Fig.1 Flaccid bullae and crusted erosions with an erythematous rim distributed over the groin area in the neonate.

Pemphigus is defined as a group of life-threatening blistering disorders characterised by acantholysis (loss of keratinocyte to keratinocyte adhesion) that results in the formation of intraepithelial blisters in mucous membranes and skin. The process of acantholysis is induced by circulating autoantibodies to intracellular adhesion molecules [2]. Patients with pemphigus develop mucosal erosions and/or flaccid bullae, erosions, or pustules on skin. Neonatal pemphigus is a very rare transient form which occurs as a consequence of placental transmission of autoantibodies to the fetus from the mother. Maternal Pemphigus causes premature births and still births, with rare occurrence of neonatal pemphigus. The prognosis is very good with resolution of lesions completely by 3 weeks of life [5].

1. Jacqueline P,　Scott RF, Jason H,　John Z,　Kristin L,　Shery V. Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid. J Am Acad Dermatol. 2009;60:1057-62.

2. Michael H, Cassian S. Pathogenesis, Clinical Manifestations and Diagnosis of Pemphigus. Available from: URL: http// www.uptodate.com/store. Accessed July 7, 2013.

3. Sameera BKI, Yashodhara BM, Shashikiran U. Pemphigus vulgaris in a pregnant woman and her neonate. BMJ. 2012:2013:1-5.

4. Moncada B, Kettelsen S, Hernandez JL, Ramirez F. Neonatal pemphigus vulgaris: role of passively transferred pemphigus antibodies. Br J Dermatol. 1982; 106:465-8.