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Clinical PRACTICE Blistering Mucocutaneous Diseases of the Oral Mucosa — a Review: Part 2

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Clinical PRACTICE Blistering Mucocutaneous Diseases of the Oral Mucosa — a Review: Part 2 Clinical PRACTICE Blistering Mucocutaneous Diseases of the Oral Mucosa — A Review: Part 2. Pemphigus Vulgaris Contact Author Mark R. Darling, MSc (Dent), MSc (Med), MChD (Oral Path); Dr.Darling Tom Daley, DDS, MSc, FRCD(C) Email: mark.darling@schulich. uwo.ca ABSTRACT Oral mucous membranes may be affected by a variety of blistering mucocutaneous diseases. In this paper, we review the clinical manifestations, typical microscopic and immunofluorescence features, pathogenesis, biological behaviour and treatment of pemphigus vulgaris. Although pemphigus vulgaris is not a common disease of the oral cavity, its potential to cause severe or life-threatening disease is such that the general dentist must have an understanding of its pathophysiology, clinical presentation and management. © J Can Dent Assoc 2006; 72(1):63–6 MeSH Key Words: mouth diseases; pemphigus/drug therapy; pemphigus/etiology This article has been peer reviewed. he most common blistering conditions captopril, phenacetin, furosemide, penicillin, of the oral and perioral soft tissues were tiopronin and sulfones such as dapsone. Oral Tbriefly reviewed in part 1 of this paper lesions are commonly seen with pemphigus (viral infections, immunopathogenic mucocu- vulgaris and paraneoplastic pemphigus.6 taneous blistering diseases, erythema multi- forme and other contact or systemic allergic Normal Desmosomes reactions).1–4 This paper (part 2) focuses on Adjacent epithelial cells share a number of the second most common chronic immuno- connections including tight junctions, gap pathogenic disease to cause chronic oral junctions and desmosomes. Desmosomes are blistering: pemphigus vulgaris. specialized structures that can be thought of as spot welds between cells. The intermediate Pemphigus keratin filaments of each cell are linked to focal Pemphigus is a group of diseases associated plaque-like electron dense thickenings on the with intraepithelial blistering.5 Pemphigus inside of the cell membrane containing pro- vulgaris (variant: pemphigus vegetans) and teins called plakoglobins and desmoplakins. pemphigus foliaceous (variant: pemphigus Some of these proteins are linked to glycopro- erythematosus) are the classically recognized teins, desmogleins and desmocollins, which clinical variants, but others are now known, extend through the cell plasma membrane to a such as IgA pemphigus, paraneoplastic pem- widened zone between the 2 cells called the phigus and herpetiform pemphigus. Drug- desmoglea. In this space, the desmogleins and induced pemphigus vulgaris-like lesions are desmocollins join by homophilic binding to known to be caused by D-penicillamine, but link the 2 cell membranes together (Fig. 1). similar lesions have also been reported for The expression of desmogleins varies between JCDA • www.cda-adc.ca/jcda • February 2006, Vol. 72, No. 1 • 63 ––– Darling ––– inflammatory infiltrate in the adjacent connective tissue2 (Figs. 2c and 2d). Immunofluorescence Features Direct immunofluorescence shows binding of IgG and C3 between epithelial cells, forming a “chicken wire” or “fish net” appearance. There is no staining in the base- ment membrane zone (BMZ). Indirect immunofluores- cence is positive in the same pattern in almost all cases indicating the presence of circulating autoantibodies.2 Figure 1: Schematic of a normal Pathogenesis desmosome between 2 adjacent epithelial cells. In mucous membrane pemphigus vulgaris, IgG autoantibodies against desmoglein 3 are produced and are hypothesized to hinder sterically the homophilic extracellular linkage of adjacent cells in the desmoglea of 6 oral epithelium and skin: oral epithelium contains mostly the desmosome (Fig. 2e). Consequently, cells do not desmoglein 3, whereas skin contains both desmoglein 3 adhere to each other and the epithelium falls apart. and desmoglein 1. Hemidesmosomes at the basement membrane are not affected because they do not contain desmoglein 3, so Pemphigus Vulgaris basal cells remain attached to the basement membrane. In Pemphigus vulgaris is a blistering disease of skin and mucocutaneous pemphigus vulgaris, IgG autoantibodies sometimes of the mucous membranes. It affects both men to both desmoglein 1 and 3 are produced, causing lesions and women over a wide age range and is extremely variable on both mucous membranes and skin.5–7 Circulating in severity. Two immune variants are recognized: the autoantibodies against desmoglein 3 or 1, or both, are mucous membrane predominant type (anti-desmoglein 3 usually found and the severity of disease is roughly pro- only) and the mucocutaneous type (anti-desmoglein 1 portional to the serum concentration. This fact allows the and 3). In the mucocutaneous type, oral ulcerative lesions disease to be monitored by assessing changes in autoanti- are often seen before the disease inevitably affects the skin. body serum concentration. The condition is seen in a greater proportion in certain Clinical Course ethnic groups, such as those of Mediterranean origin and Severe cases of pemphigus vulgaris affecting skin and Ashkenazi Jews, suggesting a genetic predisposition. The mucous membranes are fatal if not treated. Localized disease is less common than mucous membrane pem- lesions in the oral cavity are not life threatening but may phigoid (MMP), occurring in about 0.1–0.5 patients per be associated with considerable morbidity if untreated, 100,000 population a year.7 and patients must be carefully monitored for spread of Patients complain of painful, persistent ulcers and the condition to other mucous membranes and skin. sloughing (Fig. 2a), which may affect any part of the oral cavity but is commonly seen first in the buccal mucosa, Pemphigus vulgaris typically has a rapid onset, but pro- palatal mucosa and lips7 (Fig. 2b). Occasionally blisters are gression is highly variable. Remission is common after an seen, but these usually rupture quickly and are often unpredictable time period, and apparent cure of the dis- unnoticed. The Nikolsky test is positive. The ulcerations ease may be seen. About 75% of patients undergo remis- may affect other mucous membranes, including the con- sion after 10 years of therapy. Induction of remission junctiva, nasal mucosa, pharynx, larynx, esophagus and depends on the initial severity of disease and response to 7 genital mucosa, as well as the skin where intact blisters are therapy. more commonly seen.8 Treatment Microscopic Appearance Corticosteroids are the primary drugs used in the Biopsy of a blister shows an intraepithelial vesicle con- treatment of pemphigus vulgaris.7–9 Mild localized lesions taining floating, rounded keratinocytes (Tzanck cells) that of oral mucous membrane pemphigus in patients with have become detached from surrounding cells (acanthol- low titres of circulating autoantibodies may be controlled, ysis). The basal layer cells are still firmly attached to the at least temporarily, with topical corticosteroid rinses or connective tissue, although they may show separation creams, including agents such as clobetasol proprionate.7 along their lateral plasma membranes. The roof of the Intralesional triamcinolone may be used for resistant blister is tenuous and of variable thickness, or may be local lesions. However, patients with multifocal disease or absent. Long rete pegs lined by a single layer of basal cells severe localized disease require systemic corticosteroids, are often present, and there is a mild to moderate chronic typically starting at 60–80 mg prednisone a day. If no con- 64 JCDA • www.cda-adc.ca/jcda • February 2006, Vol. 72, No. 1 • ––– Pemphigus Vulgaris ––– 2a: This female patient with oral pem- Figure 2b: Sloughing lesions of the soft phigus vulgaris has typical ragged palate are commonly seen, as in this female epithelial lesions of the gingiva. The patient with pemphigus vulgaris. arrow points to the superficial epithe- lial layers that have sloughed off. Blisters are rarely seen intraorally. Figure 2c: Microscopically, a blister occurs within the epithelium as individual cells become detached and the epithelium separates. This lesion has lost the roof of the blister, which now contains hemorrhage, but the clinging basal layer is still present (arrow). (Hematoxylin and eosin staining, magnification ×100) Figure 2d: Higher magnification shows Figure 2e: Schematic diagram of the the clinging basal layer (arrows) as well immunopathogenesis of oral pemphigus as detached (Tzank) cells (curved vulgaris. arrows) floating in the blister space. (Hematoxylin and eosin staining, magnification ×500) trol is obtained in the first week, the dose is increased to Paraneoplastic Pemphigus 120 mg a day for a week, then up to 240 mg a day if neces- Paraneoplastic pemphigus is a rare blistering and sary to prevent new blisters. Once control is achieved, the ulcerating disease of sudden onset of the skin and mucous dose is reduced by half, to 120 mg a day for a week, then to membranes and always affects the oral mucosa. Oral 80 mg a day for a week until a level of 40 mg a day is lesions are very painful and consist of widespread, irreg- reached. The dose is then decreased every 4 months.9 In ular shallow ulcers at multiple oral sites. Characteristically, most cases, low maintenance doses, usually every other they extend on the lip vermilion causing hemorrhagic, day, are needed for years. High-dose pulses of corticos- crusting blisters and erosions (Fig. 3a). The conjunctiva are typically severely affected with inflammation, ulcera- teroids may be delivered for relapsing or resistant cases, tion and pain (Fig. 3b).
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