Pemphigus vulgaris and its clinical manifestations

case report

Authors

  • Alayde Ramos de-Sousa Centro Universitário UniFacid https://orcid.org/0000-0001-5755-0714
  • Walleska Gomes Moreno Silva Centro Universitário UniFacid
  • Lara Eunice Cândido-Soares Centro Universitário UniFacid
  • Alexandre Monteiro da-Silva Centro Universitário UniFacid
  • Arthur Gomes Leite Centro Universitário UniFacid

DOI:

https://doi.org/10.5935/2525-5711.20200027

Keywords:

Pemphigus, Signs and symptoms, Autoimmune diseases

Abstract

Autoimmune diseases have been steadily increasing in recent decades. The antibodies produced interact with the tissue itself resulting in the formation of blisters that affect the skin and mucous membranes, called vesicle-bullous autoimmune pathologies, one of the most common being pemphigus vulgaris. Therefore, it is characterized as a chronic and rare disease, with cutaneous and oral manifestations. The lesions are characterized by the formation of blisters that break easily and give place to extensive ulcerations. Thus, it is necessary to know about the main cutaneous and oral clinical manifestations, as well as the clinical evolution of the disease so that an early diagnosis can be made, which can lead to successful treatment. The objective of this study is to present a clinical case of a patient diagnosed with pemphigus vulgaris and its clinical dermatological and oral ma- nifestations at the beginning of the disease, as well as during the treatment confirmed by biopsy, histopathological examination, direct immunofluorescence and clinical findings. For data collection, questionnaires, intraoral and extraoral photographs of the face, armpits, chest, groin and genital mucosa were used. It was concluded that pemphigus vulgaris is a disease characterized by the formation of erythematous blisters and ulcers, which can reach any oral location. On the skin, the lesions progress rapidly in erosion and crusting formation, showing a symmetrical predilection for the chest, groin, armpits, scalp and face.

Author Biographies

Alayde Ramos de-Sousa, Centro Universitário UniFacid

Departamento de Odontologia

Walleska Gomes Moreno Silva, Centro Universitário UniFacid

epartamento de Odontologia

Lara Eunice Cândido-Soares, Centro Universitário UniFacid

Departamento de Odontologia

Alexandre Monteiro da-Silva, Centro Universitário UniFacid

Departamento de Odontologia

Arthur Gomes Leite, Centro Universitário UniFacid

Departamento de Odontologia

References

Cerqueira LS, Moreira CVA, Pinheiro IDG, Falcão MML. Early diagnosis inimmunologically mediated diseases: case report. Rev Cir Traumatol Buco-Maxilo-Fac. 2016;16(4):36-9.

Amagai M. Pemphigus. In: Bolognia JL, Schaffer JV, Cerroni L. Dermatology. 3rd ed. Philadelphia: Elsevier; 2012. p. 469-71.

Schmidt E, Zillikens D. Modern diagnosis of autoimmune blistering skin diseases. Autoimmun Rev. 2010 Dec;10(2):84-9.

Tsunoda K, Ota T, Saito M, Hata T, Shimizu A, Ishiko A, et al. Pathogenic relevance of IgG and IgM antibodies against desmoglein 3 in blister formation in pemphigus vulgaris. Am J Pathol. 2011 Aug;179(2):795-806.

Cruz ABS, Vasconcelos AJA, Pinheiro TN, Cabral LN. Pemphigus vulgaris in a smoking patient: case report. Rev Acad Bras Odontol. 2019;8(2):20-5.

Hertl M, Jedlickova H, Karpati S, Marinovic B, Uzun S, Yayli S, et al. Pemphigus S2 guideline for diagnosis and treatment - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2015 Mar;29(3):415-14.

Santoro FA, Stoopler ET, Werth VP. Pemphigus. Dent Clin North Am. 2013 Oct;57(4):597-610.

Svecova D. Pemphigus vulgaris: a clinical study of 44 cases over a 20-year period. Int J Dermatol. 2015 Oct;54(10):1138-44.

Sticherling M, Erfurt-Berge C. Doenças de bolhas auto-imunes da pele. Rev Autoimun. 2012;11:226-30.

Torres Neto JR, Santiago RR, Prudente ACL, Mariano DR, Torres FAP, Santana RM, et al. Relato de caso: pênfigo vegetante. Rev Bras Coloproctol. 2010;30(2):210-4.

Alpsoy E, Akman-Karakas A, Uzun S. Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid. Arch Dermatol Res. 2015 May;307(4):291-8.

Gonçalves GAP, Brito MMC, Salathiel AM, Ferraz TS, Alves D, Roselino AMF. Incidência do pênfigo vulgar ultrapassa a do pênfigo foliáceo em região endêmica para pênfigo foliáceo: análise de série histórica de 21 anos. An Bras Dermatol. 2011 Nov/Dec;86(6):1109-12.

Risso M, Villalpando KT, Pinho MN, Pallotta Filho R. Pemphigus vulgaris: case report. Rev Gaúcha Odontol. 2011;59(3):515-20.

Shah AA, Seiffert-Sinha K, Sirois D, Werth VP, Rengarajan B, Zrnchik W, et al. Development of a disease registry for autoimmune bullous diseases: initial analysis of the pemphigus vulgaris subset. Acta Derm Venereol. 2015 Jan;95(1):86-90.

Silva DAR, Bernardes AG. Pênfigo: uma cartografia sobre articulações das políticas em saúde. Ciênc Saúde Coletiva. 2018;23(8):2631-40.

Kuten-Shorrer M, Menon RS, Lerman MA. Mucocutaneous diseases. Dent Clin North Am. 2020 Jan;64(1):139-62.

Waschke J, Spindler V. Desmosomes and extradesmosomal adhesive signaling contacts in pemphigus. Med Res Rev. 2014 Nov;34(6):1127-45.

Espana A, Koga H, Suárez-Fernández R, Ohata C, Ishii N, Irarrazaval I, et al. Antibodies to the amino-terminal domain of desmoglein 1 are retained during transition from pemphigus vulgaris to pemphigus foliaceus. Eur J Dermatol. 2014;24(2):174-9.

Ohta M, Osawa S, Endo H, Kuyama K, Yamamoto H, Ito T. Pemphigus vulgaris confined to the gingiva: a case report. Int J Dent. 2011;207153.

Thorat MS, Raju A, Pradeep AR. Pemphigus vulgaris: effects on periodontal health. J Oral Sci. 2010 Sep;52(3):449-54.

Jascholt I, Lai O, Zillikens D, Kasperkiewicz M. Periodontitis in oral pemphigus and pemphigoid: a systematic review of published studies. J Acad Dermatol. 2017;76(5):975-8.e3.

Carli JP, Souza PHC, Westphalen FH, Rozza RF, Silva SO, Linden MSS, et al. Pemphigus and its variations. Odonto (UMESP). 2011;19(38):15-29.

Porro AM, Seque CA, Ferreira MCC, Simões MM, Enokihara MMSS. Pemphigus vulgaris. An Bras Dermatol. 2019 May;94(3):264-78.

Hsu D, Brieva J, Silverberg JI. Costs of care for hospitalization for pemphigus in the United States. JAMA Dermatol. 2016 Jun;152(6):645-54.

Leshem YA, Gdalevich M, Ziv M, David M, Hodak E, Mimouni D. Opportunistic infections in patients with pemphigus. J Am Acad Dermatol. 2014;71(2):284-92.

Mimouni D, Bar H, Gdalevich M, Katzenelson V, David M. Pemphigus, analysis of 155 patients. J Eur Acad Dermatol Venereol. 2010

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Published

2020-01-11

How to Cite

1.
de-Sousa AR, Silva WGM, Cândido-Soares LE, da-Silva AM, Leite AG. Pemphigus vulgaris and its clinical manifestations: case report. J Oral Diagn [Internet]. 2020 Jan. 11 [cited 2024 Sep. 19];5:1-6. Available from: https://jordi.com.br/revista/article/view/89

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Section

Case Report