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Year : 2020  |  Volume : 21  |  Issue : 4  |  Page : 340-342

Vaccine–induced childhood pemphigus vulgaris: A case report in a 5-year-old female child

Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India

Date of Submission16-Jun-2019
Date of Decision26-Jun-2019
Date of Acceptance26-Apr-2020
Date of Web Publication30-Sep-2020

Correspondence Address:
Dr. Mudita Gupta
Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_61_19

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Pemphigus vulgaris (PV) is an immunobullous disease affecting the skin and mucosa. It is a disease most commonly manifesting in the third–fifth decades of life. Pediatric PV is rare and usually seen at a mean age of 12 years. Early childhood PV is extremely rare with only few cases reported. There are various inducers of PV in children. Diphtheria toxoid (DT) has rarely been reported as an inducer of PV. We report a 5-year-old female child who developed vesiculobullous mucocutaneous lesions after DT vaccination. The diagnosis was confirmed by histopathology and immunofluorescence. She was treated with pulse methylprednisolone therapy and daily dose azathioprine.

Keywords: Early childhood, pemphigus vulgaris, postvaccination

How to cite this article:
Sharma R, Gupta M, Tegta GR, Rani R. Vaccine–induced childhood pemphigus vulgaris: A case report in a 5-year-old female child. Indian J Paediatr Dermatol 2020;21:340-2

How to cite this URL:
Sharma R, Gupta M, Tegta GR, Rani R. Vaccine–induced childhood pemphigus vulgaris: A case report in a 5-year-old female child. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Oct 28];21:340-2. Available from: https://www.ijpd.in/text.asp?2020/21/4/340/296864

  Introduction Top

Pemphigus is an immunobullous disease most commonly reported in the age group of 35–45 years. Childhood-onset pemphigus vulgaris (PV) is extremely rare with an incidence of 1.4%–3.7% of total cases.[1] The mean age of pediatric pemphigus is 12 years.[2] There are very few cases of PV reported in very young children.[3] PV has rarely been reported to be induced after diphtheria toxoid (DT) vaccination.[4] We report a case of DT-induced PV in a 5-year-old girl.

  Case Report Top

A 5-year-old female child presented with fluid-filled lesions over the right hand for 15 days which were asymptomatic and rupture spontaneously. She developed a similar lesion over the upper back, buttocks, ear, and scalp. Two days prior to presentation, she developed erosions inside the mouth, lips, and genital mucosa also. She had received DT vaccination 2 weeks prior to the development of cutaneous lesions. Earlier she had received immunization as per the universal immunization schedule. There was no history of any other drug intake, fever, joint pains, or any other systemic complaint. On examination, she was a young child of average built. Mucocutaneous examination showed discrete crusted erosive lesions on the scalp, left ear, upper back, dorsa of the right hand, and left foot. There was diffuse hemorrhagic crusting of the lips. Multiple erythematous erosions, with white slough and overhanging margins, were seen on the hard palate [Figure 1]. Multiple erosions were present on labia minora, majora, and over nasal vestibule [Figure 2]. A clinical diagnosis of PV was made with a differential diagnosis of erythema multiforme.
Figure 1: Erosive lesions on the lips and tongue

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Figure 2: Erosive lesions on the labia

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Routine hematological and biochemical investigations were within the normal limits. Serology for herpes virus was negative; Tzanck smear showed plenty of acantholytic cells with few inflammatory cells. Histopathology showed suprabasal blister with acantholytic cell in the blister cavity. Basal keratinocytes and membrane were intact. Direct immunofluorescence was positive for immunoglobulin (Ig) G, C3 around the keratinocytes in a chicken wire pattern [Figure 3]. In our patient, PV developed after DT, so the diagnosis of induced PV was made.
Figure 3: Direct immunofluorescence showing intraepidermal positivity in fishnet pattern

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She was first started on oral steroids in a dose of 2 mg/kg for 1 week, but as she continued getting new lesions, she was shifted to methylprednisolone pulse of 250 mg for 3 days [Figure 4]. Intervening deflazacort (2.4 mg/kg) along with azathioprine (2 mg/kg) was started. Since there was no new lesion after the primary episode, steroids were quickly tapered off and she was continued on azathioprine which was given for 3 months. In our case, as it was an induced pemphigus, we planned a short-term therapy. No new lesions appeared, even after 12 months of stopping maintenance treatment.
Figure 4: Lesions on the lips and hard palate and appearance of new lesions below the lower lip after 1 week of 2 mg/kg of prednisolone

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  Discussion Top

PV is an autoimmune blistering disorder with the destruction of intercellular adhesions in the epidermis. Although all the immunobullous diseases are rare in childhood, linear IgA disease (LAD) and dermatitis herpetiformis occur most frequently. Among intraepidermal autoimmune blistering disorders, endemic pemphigus is the most common followed by PV. The incidence of PV in children is 0.1–0.5 cases/100,000 inhabitants/year.[5] Arbitrarily pediatric pemphigus is divided into juvenile-onset (12–18 years) and childhood-onset pemphigus (age <12 years).[3] There are very few case reports of pemphigus in early childhood. The youngest child was 2 years at the age of onset.[6] Our patient was a 5-year-old girl child.

The childhood pemphigus vulgaris (CPV) resembles adult disease with the presence of flaccid blisters with Nikolsky sign positive; the blisters rupture to form erosions. Oral and genital lesions are more common in children and hence are often misdiagnosed as erythema multiforme.[5] Other mucosae (conjunctiva, laryngeal anal, and urinary tract) involvement may be seen but is less common.

Various trigger/aggravating factors have been associated with CPV which includes ultraviolet radiation, X-rays, emotional stress, nutritional deficiency, drugs (principally those containing thiol and/or phenol groups), and vaccination.[7] Our patient had received DT following which pemphigus was induced. Whether any corelation between immunization and immunobullous lesions actually exists or it is just a coincidence is not certain. There is a nonspecific immune reactivation with vaccination which may trigger autoimmune response in a susceptible host. Furthermore, with vaccination, there is Th 17 activation with an increase in interleukin 17 with an increase in pro-inflammatory cytokines and proteolytic enzymes.[8] Thiols (sodium ethylmercuryl thiosalicylate) which are used as a preservative in DT can induce pemphigus.[3]

PV being a rare disease in childhood, the diagnosis is often delayed. Differential diagnosis of cutaneous lesions in CPV includes various other auto-immune intraepidermal blistering disorders, various infections (bullous impetigo, herpetic infections, staphylococcal scalded skin syndrome), bullous contact dermatitis, bullous drug reactions, inherited forms of porphyria, and congenital blistering disorders, etc.

Diagnosis is usually confirmed by cytological smear (plenty of acantholytic cells with minimal inflammatory cells), histopathology (suprabasal blistering), and direct immunofluorescence (intraepidermal Ig G deposits). In pediatric patients where skin biopsy may not be possible, the detection of anti-epidermal antibodies in the serum of patients with PV by indirect immunofluorescence may be done.

The prognosis of CPV is better than adult variant and should be tailored for each case. Depending on age, drug contraindication, side effects, the area involved, and disease activity,[9] corticosteroids are the treatment of choice in both adults and children. Bjarnason and Flosadotter have suggested an initial dose in a range of 2–3 mg/kg/day with slow tapering to 0.5–0.8 mg/kg/day in approximately 2 weeks following alternate day schedule for further reduction.[3] Dexamethasone pulse therapy as may be given in children also. Although steroids are most preferred, long-term use in children leads to complications. Multiple adjuvants have been used in children: cyclosporine, azathioprine methotrexate, dapsone, cyclophosphamide, gold, intravenous Ig, plasmapheresis, and rituximab. Azathioprine (1–2 mg/kg) as an adjuvant is considered relatively safe. Whether to use these immunosuppressive for long or as a short course is debatable. Long-term use of immunosuppressive leads to increase risk of infections, but short-term use may lead to relapses as studied by Katakam et al. who observed relapse rates of 53.8% with incomplete treatment, 18.2% with irregular intervals, and 8% with regular intervals.[10] As reported by Cozzani et al. in induced pemphigus, short treatment may be sufficient.[4] We suggest for children, the treatment of PV should be tailored according to the severity of the disease, age, and cause of PV to avoid overtreatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Yazganoglu KD, Baykal C, Kucukoglu R. Childhood pemphigus vulgaris: Five cases in 16 years. J Dermatol 2006;33:846-9.  Back to cited text no. 1
Bjarnason B, Flosadotter E. Childhood, neonatal and stillborn pemphigus vulgaris. Int J Dermatol 1999;38:680-8.  Back to cited text no. 2
De D, Kanwar AJ. Childhood pemphigus. Indian J Dermatol 2006;51:89-95.  Back to cited text no. 3
  [Full text]  
Cozzani E, Cacciapuoti M, Parodi A, Rbora A. Pemphigus following diphtheria and tetanus vaccination. Br J Dermatol 2002;147:188-9.  Back to cited text no. 4
Surya V, Kumar P, Gupta S, Urs AB. Childhood pemphigus vulgaris: Report of two cases with emphasis on diagnostic approach. Contemp Clin Dent 2018;9:373-6.  Back to cited text no. 5
[PUBMED]  [Full text]  
Laskaris G, Stouji E. Oral pemphigus vulgaris in a 6-year-old girl. Oral Surc Oral Med Oral Pathol 1990;69:609-13.  Back to cited text no. 6
Tavakolpour S. Pemphigus trigger factors: Special focus on pemphigus vulgaris and pemphigus foliaceus. Acta Dermatol Res 2018;10:422-5.  Back to cited text no. 7
Baroero L, Coppo P, Bertolino L, Maccario S, Savino F. Three case reports of post immunization and post viral bullous pemphigoid: Looking for the right trigger. BMC Pediatr 2017;17:60.  Back to cited text no. 8
Wananukul S, Pongprasit P. Childhood pemphigus. Int J Dermatol 1999;38:29-35.  Back to cited text no. 9
Katakam BK, Kavitha SB, Netha GN, Shahana M, Sri TS, Vani DS. Prospective study of pulse therapy in childhood pemphigus disorders. Indian Dermatol Online J 2018;9:422-5.  Back to cited text no. 10
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