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Diagnostic Patterns and Delays in PV: Experience With 99 Pacientes

By David A. Sirois, D.M.D., Ph.D.
Department of Oral Medicine

Pemphigus vulgaris is a chronic autoimmune disease affecting the mucosa and skin and resulting in epithelial acantholysis, bullae formation, and chronic ulceration.1 Skin lesions of pemphigus vulgaris present clinically with typical bullae formation and ulceration. However, oral mucosal manifestations are less characteristic, typically occurring as multiple, chronic mucosal erosions or superficial ulcerations of various sizes and rarely presenting with intact bullae.2 Although pemphigus vulgaris is widely considered a skin disease, several reports of cases and case series have described it frequently as the initial, and occasionally the exclusive, site of involvement.2, 3 Thus, the unfamiliar features of oral pemphigus vulgaris could result in longer diagnostic and treatment delays than cutaneous pemphigus, which could adversely affect treatment response and prognosis.4, 5 The present study explored the natural history and diagnostic pattern of pemphigus vulgaris among 99 patients, with specific interest in the differences between oral and cutaneous pemphigus.



Patients and Methods

A questionnaire was mailed to the membership of the New York Chapter of the National Pemphigus Foundation and also distributed to attendees at the national conference of the foundation held in Chicago, Ill, in 1998. A total of 99 questionnaires were completed: of the 64 mailed questionnaires, 52 were completed and returned by members (81%) and an additional 47 nonduplicated individuals attending the national meeting. To minimize selection bias, we made the questionnaire available to patients without regard to the site of their lesions or the type of treating clinician. The questionnaire collected the following data: specific diagnosis; present age; age at diagnosis; sex; prevalence of oral, cutaneous, and combined mucocutaneous lesions; site of initial lesions (oral mucosa, skin, other) and site of most problematic lesions; time between onset of skin and oral lesions; the number of clinicians consulted and biopsies performed before a correct diagnosis was established; and present medications.

Results

In Table 1 are the demographic characteristics and lesion chronology of the group. Table 2 summarizes the distribution of lesions and diagnostic patterns. Eighty percent of patients experienced oral lesions as the first sign of pemphigus vulgaris. Throughout the history of their illness, 69% experienced both oral and cutaneous lesions, 24% only oral lesions, and 7% only cutaneous lesions. Oral lesions were more bothersome than skin lesions for 67% of patients, mostly owing to pain while eating.

Conclusions

Eighty percent of patients with pemphigus vulgaris experienced oral lesions first, and the disease remained limited to mucosal surfaces in 1 of 4 patients. Diagnostic delays greater than 6 months were common in oral pemphigus vulgaris, a finding of particular importance since early diagnosis and intervention may prevent disease spreading. Oral pemphigus vulgaris was less commonly recognized than cutaneous pemphigus vulgaris, requiring more clinician consultations associated with increased health care utilization and costs. While patients with skin lesions typically sought help from physicians, those with oral lesions sought help equally from physicians and dentists. Therefore, when a patient presents with multiple, chronic oral ulcerations, pemphigus vulgaris should be included in the differential diagnosis.

David A. Sirois, DMD, PhD
Department of Oral Medicine
New York University College of Dentistry
345 E 24th St
Nueva York, NY 10010
(e-mail: [email protected])

Mahnaz Fatahzadeh, DMD
Rinat Roth, DDS
Newark, NJ

Dominik Ettlin, DMD, MD
Chicago, Ill

References

1. Fine JD. Management of acquired bullous skin diseases. N Engl J Med. 1995;333:1475-1484. MEDLINE

2. Robinson JC, Lozada-Nur F, Frieden I. Oral pemphigus vulgaris: a review of the literature and a report on the management of 12 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1997;84:349-355.

3. Eversole LR. Immunopathology of oral mucosal ulcerative, desquamative, and bullous disease. Oral Surg Oral Med Oral Pathol. 1994;77:555-571. MEDLINE

4. Ding X, Aoki V, Mascaro JM Jr, Lopez-Swiderski A, Diaz LA, Fairley JA. Mucosal and mucocutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles. J Invest Dermatol. 1997;109:592-596. MEDLINE

5. Fitzpatrick RE, Newcomer VD. The correlation of disease activity and antibody titers in pemphigus. Arch Dermatol. 1980;116:285-290. MEDLINE

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