Juvenile pemphigus vulgaris

Laskaris, George; Sklavounou, Alexandra; Bovopoulou, Olga D.

doi:10.1016/0030-4220(81)90152-3

Cited by 23 publications

(8 citation statements)

References 16 publications

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“…More cases of childhood and teenage disease need to be studied to characterize better the features of oral pemphigus vulgaris in adolescents. Pemphigus vulgaris tends to be a disorder of middle to late life and is rare in juvenile patients, in whom the disease can be more severe 18 ,. 19 The majority of affected patients in most previous studies have thus been aged between 40 and 60 years at the time of onset, and the condition has rarely been recorded in persons younger than 30 years or after the eighth decade.…”

Section: Discussionmentioning

confidence: 99%

Pemphigus vulgaris: the manifestations and long-term management of 55 patients with oral lesions

Scully

Almeida

Porter

et al. 1999

British Journal of Dermatology

156

143

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Perhaps surprisingly, the manifestations and management of patients with pemphigus vulgaris and oral lesions have been detailed only infrequently. The present study has examined the clinical features, diagnosis and management of a cohort of 55 patients, including three adolescents, with pemphigus vulgaris predominantly affecting the oral mucosa. There was about a 6-month delay from the onset of symptoms until presentation for diagnosis, longer in men than in women. Patients typically had multiple lesions affecting mainly the buccal and/or palatal mucosae, and over half the patients had lesions affecting non-oral mucosal sites. Nearly one-quarter (24%) had cutaneous involvement. Most patients were otherwise healthy with no other autoimmune disorders. Classical histopathological features of pemphigus vulgaris were present in all patients, as well as IgG intraepithelial deposits in all patients tested and circulating epithelial antibodies in most. Thirty-two patients were treated in the clinic, four responding to topical immunosuppressive therapy, the remainder needing and responding, at least in part, to systemic immunosuppression. Systemic corticosteroids often with adjunctive immunosuppressives, particularly azathioprine, were required in 87% of patients. In 18% of the patients, the disease resolved in 3 months, but 76% had recalcitrant disease. Adverse effects were seen in 78%, and two patients died, at least one as a consequence of immunosuppressive therapy. It is concluded that pemphigus vulgaris affecting the oral mucosa is still diagnosed only after considerable delay because patients, especially men, present late; it has a chronic course; it is often associated with lesions in other mucosae and/or skin; it can be resistant to currently available therapies; and immunosuppressive therapy frequently produces adverse effects, occasionally lethal.

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Section: Discussionmentioning

confidence: 99%

Pemphigus vulgaris: the manifestations and long-term management of 55 patients with oral lesions

Scully

Almeida

Porter

et al. 1999

British Journal of Dermatology

156

143

View full text Add to dashboard Cite

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“…Most cases appear to be related to mucous membrane pemphigoid, but lichen planus is also a common cause. 4,6,7 Pemphigus is the most serious cause, as it is potentially lethal, but there have also been reports of a number of other disorders manifesting as DG, including psoriasis, dermatitis herpetiformis, linear IgA disease, chronic ulcerative stomatitis, erythema multiforme, pyostomatitis vegetans, epidermolysis bullosa acquisita (EBA), epidermolysis bullosa, and Kindler syndrome. [8][9][10][11] Similar appearances may be seen in reactions to various mouthwashes, chewing gum, medications and dental materials, candidosis, lupus erythematosus, plasma cell gingivitis, Crohn's disease, sarcoidosis, some leukemias, and even some factitious lesions.…”

Section: Discussionmentioning

confidence: 99%

“…1,12 Pemphigus vulgaris is mainly a disease of adults and is uncommon in children and young adults. 6,13,14 There is a fairly strong genetic background to pemphigus; certain ethnic groups, such as Ashkenazian Jews and those of Mediterranean origin are especially liable to pemphigus. 15 Pemphigus is an autoimmune disorder in which there is damage to desmosomes, leading to intraepithelial vesiculation.…”

Section: Discussionmentioning

confidence: 99%

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Gingival Lesions Diagnosed as Pemphigus Vulgaris in an Adolescent. Case Report

Navarro

Sposto

Onofre

et al. 1999

Journal of Periodontology

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Desquamative gingivitis (DG) is a fairly common disorder in which the gingivae show chronic desquamation. Originally considered to be related to hormonal changes at menopause, since many of the patients are middle-aged women, DG is now recognized to be mainly a manifestation of a number of disorders ranging from vesiculobullous diseases to adverse reactions to a variety of chemicals or allergens. Desquamative gingivitis can be an important early clinical manifestation of serious systemic diseases such as pemphigus vulgaris. The authors present a case that illustrates the importance of a specific diagnosis in patients with desquamative gingival lesions previously treated for 6 months as classical gingivitis. Gingival biopsy showed histologic patterns typical of pemphigus vulgaris. The patient was treated with systemic and topical corticosteroids in association with miconazole. The patient is now under control with low-dose systemic corticosteroids. Proper recognition of lesions in the oral mucosa leads, in several situations, to an early diagnosis of a systemic disease.

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“…Azathioprine has been the most commonly used adjuvant. In children 16 years of age or younger, seven cases have been treated with azathioprine as an adjuvant, all without side‐effects 36–42 . Many of these cases were approximately 15 years of age.…”

Section: Treatmentmentioning

confidence: 99%