Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2021.267
Autopsy and Case Reports
Clinical Case Report

Childhood pemphigus vulgaris is a challenging diagnosis

Gastão Tenório Lins Filho; Nathalia Lages Sarmento Barbosa; Eulina Maria Vieira de Abreu; Klinger Vagner Teixeira da Costa; Kelly Chrystine Barbosa Meneses; Rodrigo Neves Silva; Sonia Maria Soares Ferreira

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Abstract

Pemphigus Vulgaris (PV) is an uncommon autoimmune and blistering mucocutaneous disease. Childhood Pemphigus Vulgaris (CPV) is a pediatric variant of PV, which affects children below 12 years, being very rare among children under 10 years of age. CPV has similar clinical, histological, and immunological features as seen in PV in adults. The mucocutaneous clinical presentation is the most common in both age groups. Vesicles and erosions arising from the disease usually cause pain. A few CPV cases have been reported in the literature. This study reports a case of an 8-year-old male patient with oral lesions since the age of 3 years, and the diagnosis of pemphigus was achieved only 2 years after the appearance of the initial lesions. CPV remains a rare disease, making the diagnosis of this clinical case a challenge due to its age of onset and clinical features presented by the patient. Therefore, dentists and physicians should know how to differentiate CPV from other bullous autoimmune diseases more common in childhood.

Keywords

Pemphigus, Child, Fluorescent Antibody Technique, Direct

References

1 Kincaid L, Weinstein M. Rituximab therapy for childhood pemphigus vulgaris. Pediatr Dermatol. 2016;33(2):e61-4. http://dx.doi.org/10.1111/pde.12744. PMid:26765543.

2 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(6, Suppl 2):S373-6. http://dx.doi.org/10.4103/ccd.ccd_461_18. PMid:30294176.

3 Gürcan HM, Mabrouk D, Razzaque Ahmed A. Management of pemphigus in pediatric patients. Minerva Pediatr. 2011;63(4):279-91. PMid:21909064.

4 Porro AM, Seque CA, Ferreira MCC, Enokihara MMSS. Pemphigus vulgaris. An Bras Dermatol. 2019;94(3):264-78. http://dx.doi.org/10.1590/abd1806-4841.20199011.

5 Gonul M, Keseroglu HO. Pediatric Phemphigus. Clin Pediatr Dermatol. 2015;1(1):1-3.

6 Fuertes I, Guilabert A, Mascaró JM Jr, Iranzo P. Rituximab in childhood pemphigus vulgaris: a long-term follow-up case and review of the literature. Dermatology. 2010;221(1):13-6. http://dx.doi.org/10.1159/000287254. PMid:20389028.

7 Vinay K, Kanwar AJ, Sawatkar GU, Dogra S, Ishii N, Hashimoto T. Successful use of rituximab in the treatment of childhood and juvenile pemphigus. J Am Acad Dermatol. 2014;71(4):669-75. http://dx.doi.org/10.1016/j.jaad.2014.05.071. PMid:25022850.

8 Broshtilova V, Vassileva S. A case of pediatric pemphigus treated with rituximab: our experience. Asian J Res Dermatology Science. 2019;2(1):1-6.

9 Casuriaga Lamboglia AL, Minut Gubitosi A, Guebenlián Bakerdjian C, Giachetto Larraz G. Pemphigus vulgaris in pediatrics: a case report. Rev Chil Pediatr. 2018;89(5):650-4. PMid:30571809.

10 Bajpai M, Pardhe N, Chandolia B. Oral pemphigus vulgaris in a pediatric patient. J Ayub Med Coll Abbottabad. 2016;28(3):636. PMid:28712257.

11 Suliman NM, Åstrøm AN, Ali RW, Salman H, Johannessen AC. Clinical and histological characterization of oral pemphigus lesions in patients with skin diseases: a cross sectional study from Sudan. BMC Oral Health. 2013;13(1):66. http://dx.doi.org/10.1186/1472-6831-13-66. PMid:24261459.

12 Aoki V, Sousa JX Jr, Fukumori LM, Périgo AM, Freitas EL, Oliveira ZN. Direct and indirect immunofluorescence. An Bras Dermatol. 2010;85(4):490-500. http://dx.doi.org/10.1590/S0365-05962010000400010. PMid:20944909.

13 Ariyawardana A, Tilakaratne WM, Dissanayake M, et al. Oral pemphigus vulgaris in children and adolescents: a review of the literature and a case report. Int J Paediatr Dent. 2005;15(4):287-93. http://dx.doi.org/10.1111/j.1365-263X.2005.00640.x. PMid:16011788.

14 Martinez AB, Corcuera MM, Ilundain CB, Gómez GE. Oral manifestations of pemphigus vulgaris: clinical presentation, differential diagnosis and management. J Clin Exp Dermatol Res. 2010;1(3):1-4. http://dx.doi.org/10.4172/2155-9554.1000112.

15 Wozel G, Blasum C. Dapsone in dermatology and beyond. Arch Dermatol Res. 2014;306(2):103-24. http://dx.doi.org/10.1007/s00403-013-1409-7. PMid:24310318.

16 Quaresma MV, Bernardes F Fo, Hezel J, Peretti MC, Kac BK, Azulay-Abulafia L.. Dapsone in the treatment of pemphigus vulgaris: adverse effects and its importance as a corticosteroid sparing agente. An Bras Dermatol. 2015;90(3, Suppl 1):S51-4. http://dx.doi.org/10.1590/abd1806-4841.20153408.

17 Cervera-Castillo H, Torres-Caballero V, Martínez-García E, Pizaña-Serna M, Blanco-Favela FA. Granulomatosis de Wegener: estado de portador de Staphylococcus aureus y terapéutica combinada. Rev Med Inst Mex Seguro Soc. 2006;44(3):249-55. PMid:16870120.

18 Bilgic‐Temel A, Özgen Z, Harman M, Kapıcıoğlu Y, Uzun S. Rituximab therapy in pediatric pemphigus patients: a retrospective analysis of five Turkish patients and review of the literature. Pediatr Dermatol. 2019;36(5):646-50. http://dx.doi.org/10.1111/pde.13926. PMid:31355479.

19 Buch JY, Raval RC. Successful treatment of refractory childhood vesiculobullous disorders with rituximab: a study of five cases. Indian J Paediatr Dermatol. 2016;17(2):104-7. http://dx.doi.org/10.4103/2319-7250.175664.
 


Submitted date:
11/04/2020

Accepted date:
02/01/2021

Publication date:
04/30/2021

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