Pemphigus Vulgaris Clinical Presentation

Updated: Jun 14, 2018
  • Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Mucous membranes

Pemphigus vulgaris presents with oral lesions in 50-70% of patients, and almost all patients have mucosal lesions at some point in the course of their disease. Mucosal lesions may be the sole sign for an average of 5 months before skin lesions develop, or they may be the sole manifestation of the disease. The diagnosis of pemphigus vulgaris should be considered in any patient with persistent oral erosive lesions.

Skin

Most patients with pemphigus vulgaris develop cutaneous lesions. The primary lesion of pemphigus vulgaris is a flaccid blister, which usually arises on healthy-appearing skin but may be found on erythematous skin. New blisters usually are flaccid or become flaccid quickly. Affected skin often is painful but rarely pruritic.

Drug-induced pemphigus vulgaris

Drugs reported most significantly in association with pemphigus vulgaris include penicillamine, captopril, cephalosporin, pyrazolones, nonsteroidal anti-inflammatory drugs (NSAIDs), and other thiol-containing compounds. [23] Rifampin, emotional stress, thermal burns, ultraviolet rays, and infections (eg, coxsackievirus, Herpesviridae family) have also been reported as triggers for pemphigus vulgaris. [24]

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Physical Examination

Mucous membranes typically are affected first in pemphigus vulgaris. Mucosal lesions may precede cutaneous lesions by weeks or months. Patients with mucosal lesions may present to dentists, oral surgeons, or gynecologists. [34, 35]

Mucous membranes

Intact bullae are rare in the mouth. More commonly, patients have ill-defined, irregularly shaped, gingival, buccal, or palatine erosions, which are painful and slow to heal. The erosions extend peripherally with shedding of the epithelium.

The mucous membranes most often affected in pemphigus vulgaris are those of the oral cavity, which is involved in almost all patients with pemphigus vulgaris and sometimes is the only area involved. Erosions may be seen on any part of the oral cavity. Erosions can be scattered and often are extensive. Erosions may spread to involve the larynx, with subsequent hoarseness. The patient often is unable to eat or drink adequately because the erosions are so uncomfortable.

In juvenile pemphigus vulgaris, stomatitis is the presenting complaint in more than 50% of the cases.

Other mucosal surfaces may be involved, including the conjunctiva, [2] esophagus (causes odynophagia and/or dysphagia), [3] labia, vagina, cervix, vulva, [4] penis, urethra, nasal mucosa, and anus.

Skin

The primary lesion of pemphigus vulgaris is a flaccid blister filled with clear fluid that arises on healthy skin or on an erythematous base, as shown in the images below.

Early, small blister filled with clear fluid arise Early, small blister filled with clear fluid arises on healthy skin.
Flaccid blister filled with clear fluid arises on Flaccid blister filled with clear fluid arises on healthy skin.

The blisters are fragile; therefore, intact blisters may be sparse. The contents soon become turbid, or the blisters rupture, producing painful erosions, which is the most common skin presentation and is shown in the image below. Erosions often are large because of their tendency to extend peripherally with the shedding of the epithelium.

An erosion. An erosion.

Vegetating pemphigus vulgaris

Ordinary pemphigus vulgaris erosions may develop vegetation. Lesions in skin folds readily form vegetating granulations. In some patients, erosions tend to develop excessive granulation tissue and crusting, and these patients display more vegetating lesions. This type of lesion tends to occur more frequently in intertriginous areas and on the scalp or face. The vegetating type of response can be more resistant to therapy and can remain in one place for long periods.

Nails

Acute or chronic paronychia, subungual hematomas, and nail dystrophies affecting one or several fingers or toes have been reported with pemphigus vulgaris. [5, 6] Patients with paronychial pemphigus usually also have oral involvement.

Pemphigus in pregnancy

Pemphigus vulgaris occurring in pregnancy is rare. When present, maternal autoantibodies may cross the placenta, resulting in neonatal pemphigus. Neonatal pemphigus is transient and improves with clearance of maternal autoantibodies. [36] Treatment of pemphigus vulgaris in pregnancy is with oral corticosteroids; however, prednisone and its metabolites cross the placenta and have been associated with low birth weight, prematurity, infection, and adrenal insufficiency.

Signs

For the Nikolsky sign in patients with active blistering, firm sliding pressure with a finger separates normal-appearing epidermis, producing an erosion. This sign is not specific for pemphigus vulgaris and is found in other active blistering diseases.

For the Asboe-Hansen sign, lateral pressure on the edge of a blister may spread the blister into clinically unaffected skin.

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Causes

The cause of pemphigus vulgaris remains unknown; however, several potentially relevant factors have been identified.

Genetic factors

Predisposition to pemphigus is linked to genetic factors. [25] Certain major histocompatibility complex (MHC) class II molecules, in particular alleles of human leukocyte antigen DR4 (DRB1*0402) and human leukocyte antigen DRw6 (DQB1*0503), are common in patients with pemphigus vulgaris. [26, 27, 28, 29, 30, 31]

Age

Peak age of onset is from 50-60 years. Infants with neonatal pemphigus remit with clearance of maternal autoantibodies. The disease may develop in children or in older persons.

Disease association

Pemphigus occurs in patients with other autoimmune diseases, particularly myasthenia gravis and thymoma. [32] A study of 110 patients with pemphigus found 4 patients with autoimmune thyroid disease and 3 patients with rheumatoid arthritis. In this study, however, autoimmune diseases were no more common in 969 first-degree relatives of patients with pemphigus than in the general population. [33]

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