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 Table of Contents  
Year : 2021  |  Volume : 7  |  Issue : 2  |  Page : 88-90

Rituximab in childhood pemphius vulgaris: A case report

1 Department of Dermatology, Deenanath Mangeshkar Hospital, Pune, India
2 Dr. Swaraj Potdar’s Skin & Dermatosurgery Clinic, New Sangvi, India
3 Pune Dental Lounge, Pune, Maharashtra, India

Date of Submission29-Mar-2021
Date of Decision01-Sep-2021
Date of Acceptance08-Sep-2021
Date of Web Publication14-Dec-2021

Correspondence Address:
Vinay Kulkarni
Department of Dermatology, Deenanath Mangeshkar Hospital, Pune, Maharashtra.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijdd.ijdd_11_21

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Pemphigus vulgaris (PV) is an autoimmune bullous disease. It has a very low incidence in childhood with very few cases reported in literatures. A 6-year-old female child had non-healing oral erosions for a month and recent appearance of genital erosions. Differential diagnoses considered were oral candidiasis, herpetic stomatitis, and PV. On investigating further, KOH mount showed Candidial hyphae and spores. Serology for herpes viruses was negative. Tzanck smear showed acantholytic cells. Obtaining biopsy from oral mucosa was difficult; hence, indirect immunofluorescence was done which showed significant titers of anti-epidermal (pemphigus) antibodies. Anti-desmoglein 1 and 3 antibodies measured by ELISA were also positive. Final diagnosis was PV. The patient was started on oral prednisolone. However, when relapsed, considering the long-term adverse effects of systemic corticosteroids on growth of the child, two infusions of rituximab, 15 days apart, were administered after clearance from a pediatrician and appropriate laboratory work up. Outcome: There was complete remission after 1 month of rituximab. Prednisolone was tapered off quickly and withdrawn completely. There was a persistent decline in anti-desmoglein antibodies during follow-up. No severe adverse effects were documented. The child remained in remission up to 12 months of follow-up. Discussion: This is a very rare case of childhood PV. Rituximab appears to be an effective therapeutic option in children with pemphigus. Further studies will help in defining protocols for use of rituximab in children.

Keywords: Childhood pemphigus vulgaris, rituximab, treatment of pemphigus

How to cite this article:
Kulkarni V, Potdar S, Shirol P. Rituximab in childhood pemphius vulgaris: A case report. Indian J Drugs Dermatol 2021;7:88-90

How to cite this URL:
Kulkarni V, Potdar S, Shirol P. Rituximab in childhood pemphius vulgaris: A case report. Indian J Drugs Dermatol [serial online] 2021 [cited 2022 Aug 8];7:88-90. Available from: https://www.ijdd.in/text.asp?2021/7/2/88/332416

  Introduction Top

Pemphigus vulgaris (PV) is an autoimmune bullous disease with autoantibodies targeted against desmoglein (Dsg) 1 and 3. It causes flaccid bullae and poorly healing erosions affecting the skin and mucous membranes. Left untreated it has a deteriorating course. The clinical diagnosis is validated by laboratory findings. Tzanck smear shows acantholytic cells. Hematoxylin and eosin staining of biopsy specimens shows a supra-basal split with tombstone appearance of basal cells and acantholytic cells in the blister cavity. The gold standard for diagnosis is direct immunofluorescence demonstrating a fish-net appearance of IgG antibodies and C3 deposits. Indirect immunofluorescence demonstrates circulating antibodies and their titers are prognostic. Rising titers of anti-Dsg1 and anti-Dsg3 antibodies predict relapses ahead of clinical flare. Systemic immunosuppressants are the mainstay of treatment.

  Case Report Top

A 6-year-old female child, one of the twins born out of non-consanguineous marriage, was brought due to whitish lesion on her tongue for 1 month, initially treated as oral candidiasis. However, the lesions increased and spread to oral cavity and lips. There was associated bleeding and odynophagia. Hematologist and rheumatologist opinions were non-contributory. A dermatologist reference was done when there was no improvement. Provisionally diagnosed as herpetic stomatitis, acyclovir was given without relief. The child developed slight vaginal bleeding. Personal, past, family, and birth histories were non-contributory. The child was well nourished, and vitals were stable. There were vesicles and crusted erosions on the lips and vulva [Figure 1]. Oral cavity showed erosions with irregular margins and slough on the lateral border of the tongue, hard palate, and buccal mucosa. Mucosal Nikolsky’s sign was positive. The differential diagnoses considered were herpes simplex virus infection, candidiasis, and PV. Routine blood biochemistry and hemogram were normal. KOH mount of oral smear showed Candidial hyphae and spores. Serology for viral markers was negative including HSV-1 and -2 antibodies. Tzanck smear from the oral erosions showed acantholytic cells with an enlarged, hyperchromatic eccentrically situated nucleus with smooth outline [Figure 2]. A few of these cells showed vesicular chromatin with 1–4 prominent nucleoli. These findings suggested PV. The child was not cooperative for biopsy. Indirect immunofluorescence was positive for anti-epidermal (pemphigus) antibody (titer 1:1280). Serum levels of anti-Dsg1 (77.56 U/mL) and anti-Dsg3 (>200 U/mL) antibodies were also raised (positive value > 20 U/mL). Thus diagnosis of childhood PV was established. Treatment was initiated with oral prednisolone at 2 mg/kg/day and supportive care. There was incomplete remission. Considering the side effects of long-term systemic corticosteroids in childhood, rituximab as a therapeutic option was discussed. Two infusions of 500 mg each at an interval of 15 days were given in pediatric intensive care unit. Top-up IVIG in a dose of 400 mg/kg was tried but the child developed infusion reaction in the form of fever, chills, headache, and abdominal pain on starting the first infusion. Hence, it was discontinued immediately and the reaction was managed by a pediatrician. Prednisolone was tapered off quickly and completely after second infusion. The patient was followed up closely with appropriate clinical and laboratory monitoring. There was a gradual decline in titers of anti-epidermal (pemphigus) antibodies. Anti-Dsg1 and Dsg3 antibodies measured after 1, 3, and 6 months of rituximab showed sustained low levels. No adverse effects were noted. After a year of follow-up, the patient is in remission without any steroids or immunosuppressants.
Figure 1: Crusted erosions on lips, oral erosions on hard palate, and genital erosions

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Figure 2: Tzanck smear showing presence of predominantly polygonal, round, and ovoid acantholytic cells with an enlarged, hyperchromatic eccentrically situated nucleus with smooth outline. Few of these cells showed vesicular chromatin with 1–4 prominent nucleoli

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  Discussion Top

In India, pemphigus occurs commonly in the third and fourth decades. Children account for just 3.7% of cases.[1] PV in children aged less than 12 years is known as childhood PV and in those aged between 12 and 18 years as juvenile PV.[2] The majority of pemphigus in childhood is the vulgaris type. The mean age of onset is 12 years. PV in children affects both genders equally.[3] The disease course of childhood pemphigus and treatment modalities are essentially the same as those in adults.[4]

In some patients, oral erosions are the only clinical manifestations leading to delay in diagnosis.[5] Experience shows that early intervention translates into better prognosis. PV should be a consideration in children with chronic erosive mucous membrane disease.[3]

Although PV remains a potentially fatal disease, newer treatments have reduced mortality to <5%. There is no consensus yet over optimal systemic management of pemphigus in children.[6] Treatment of juvenile PV with steroids alone can have significant side effects on physical appearance, susceptibility to infections, and nutrition. The reported frequency of side effects of therapy in children with use of steroids and immunosuppressants is higher than that in adults, with growth retardation, sepsis, and death being the most notable.[3]

Treatment is aimed at reducing antibody production and suppressing local inflammation to induce remission.[7] Because of low incidence and paucity of controlled trials in childhood PV, there are currently no Food and Drug Administration-approved therapies, and the available treatment guidelines lack strength of evidence.[3]

Rituximab is a chimeric monoclonal antibody that targets the CD20 molecule on B cells resulting in their lysis. The rationale for the use of rituximab in patients with PV is based on its ability to deplete CD20+ B cells that presumably produce pathogenic antibodies.[8]

A curative protocol has been proposed by Ahmed et al.[9] for adulthood PV, consisting of infusion of rituximab (375 mg/m2 of body surface area) once a week for 3 weeks; in the fourth week, intravenous immune globulin, 2 g/kg of body weight. This is repeated for a second cycle. Following this, in months 3–6, patients receive a single infusion of rituximab plus a single infusion of 2 g of intravenous immune globulin per kilogram at the start of the month. This regimen needs further evaluation, especially in children.

In conclusion, high index of suspicion and thorough investigations are necessary to diagnose pemphigus in children. Conventional treatment using prolonged high dose corticosteroids or other immunosuppressants are not to be preferred in children due to long-term side effects, growth, and developmental adversities. Rituximab is a feasible therapeutic option and needs further studies for validation of its use in children.

Declaration of patient consent

The authors declare that they have obtained necessary consent from patient’s parents. Every attempt is being made not to reveal patient’s identity; however, anonymity cannot be assured.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.



  References Top

Kanwar AJ, Sawatkar GU, Vinay K, Hashimoto T. Childhood pemphigus vulgaris successfully treated with rituximab. Indian J Dermatol Venereol Leprol 2012;78:632-4.  Back to cited text no. 1
  [Full text]  
Gorsky M, Raviv M, Raviv E. Pemphigus vulgaris in adolescence. A case presentation and review of the literature. Oral Surg Oral Med Oral Pathol 1994;77:620-2.  Back to cited text no. 2
Baratta A, Camarillo D, Papa C, Treat JR, Payne AS, Rozenber SS, et al. Pediatric pemphigus vulgaris: Durable treatment responses achieved with prednisone and mycophenolate mofetil (MMF). Pediatr Dermatol 2013;30:240-4.  Back to cited text no. 3
Kanwar AJ, Dhar S, Kaur S. Further experience with pemphigus in children. Pediatr Dermatol 1994;11:107-11.  Back to cited text no. 4
Amagai M. Dermatology. Pemphigus. 3rd ed. Vol. 29. USA: Elsevier Saunders; 2012. p. 446.  Back to cited text no. 5
Asarch A, Razzaque Ahmed A. Treatment of juvenile pemphigus vulgaris with intravenous immunoglobulin therapy. Pediatr Dermatol 2009;26:197-202.  Back to cited text no. 6
Hempstead RW, Marks JG Jr. Pediatric pemphigus vulgaris. Treatment with topical adrenal steroids. Arch Dermatol 1984;120:962-3.  Back to cited text no. 7
Feldman RJ, Ahmed AR. Relevance of rituximab therapy in pemphigus vulgaris: Analysis of current data and the immunologic basis for its observed responses. Expert Rev Clin Immunol 2011;7:529-41.  Back to cited text no. 8
Ahmed AR, Spigelman Z, Cavacini LA, Posner MR. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 2006;355:1772-9.  Back to cited text no. 9


  [Figure 1], [Figure 2]


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