Juvenile Pemphigus Vulgaris

Abstract

Pemphigus vulgaris (PV) is a chronic autoimmune blistering disease of the skin and mucous membranes that affects older individuals. The disease rarely affects children and adolescent patients. The first manifestation of PV appears in the oral mucosa in the majority of patients, followed by cutaneous lesions. The diagnosis is based on clinical findings and laboratory analyses, and it is usually treated by the combined administration of corticosteroids and immunosuppressants. Dental specialists must be proficient to recognize the clinical features of PV to ensure early diagnosis and treatment, so that it determines the favorable prognosis and course of the disease. This article reports a case of juvenile PV.

Juvenile Pemphigus Vulgaris as a Differential Diagnosis for Chronic Generalized Oral Ulceration in an Adolescent: A Case Report and a Review of Literature

“…1,2 Usually in pemphigus, oral lesions occur before skin lesions. [7][8][9] In contrast, our patient presented with skin lesions which were preceded by oral lesions.…”

“…When a patient presents with generalized chronic ulceration in the oral cavity, the differentials to be considered are pemphigus vulgaris, erythema multiforme, acute herpetic gingivostomatitis, bullous impetigo, linear IgA disease, epidermolysis bullosa, cicatricial pemphigoid, bullous pemphigoid and paraneoplastic 7,8 However, especially in an adolescent, the possibility of pemphigus vulgaris, erythema multiforme, acute herpetic gingivostomatitis, and aphthous ulcers need to be considered. 5 In this case presented here due to lack of ulcer-free period the possibility of aphthous ulcers becomes less likely and further, as there are neither clinical features suggestive of infection nor immunodeficiency status acute herpatic gingivostomatitis can also be excluded.…”

“…2,5,6 However, in this case, there was a delayed presentation due to misdiagnosis and improper treatment. [5][6][7] This was identified as a problem in the case reports reported by Srivastava et al, Dyer et al, and the majority of case reports summarized by Ariyawardena et al [5][6][7] This results due to lack of awareness of the clinicians' when considering differential diagnosis in patients presented with chronic generalized ulcerations especially in adolescents.…”

“…5 Therefore, misdiagnosis of this condition in a patient presenting with oral ulcerations is extremely high, and this may lead to a delay in diagnosis and proper management. [5][6][7] Early diagnosis is paramount important for proper intervention as that will help to control this condition and thereby improve the quality of life in young patients. 5 Here we the present case of juvenile pemphigusvulgaris to show the importance of considering pemphigus vulgaris as a differential diagnosis for chronic oral ulceration.…”

Rituximab in Childhood and Juvenile Pemphigus Vulgaris: A Systematic Review

“…Before RTX therapy, various drugs were instituted in pediatric PV patients including systemic corticosteroids such as prednisolone and dexamethasone as the primary treatment modality. One study also reported the use of the topical corticosteroid triamcinolone acetonide [ 32 ]. Other immunosuppressants such as azathioprine [ 9 , 28 , 30 , 31 ], cyclosporine [ 24 ], mycophenolate mofetil [ 9 , 30 , 31 ], and intravenous immunoglobulins (IVIG) [ 9 , 11 , 31 ] were also administered.…”