From the Guidelines
Pemphigus vulgaris can rarely occur without oral or mucosal lesions, but this is uncommon. In the vast majority of cases, pemphigus vulgaris begins with oral mucosal lesions before skin involvement develops, as the disease typically involves autoantibodies targeting desmoglein-3, which is predominantly found in mucosal tissues 1. Some patients may present with cutaneous lesions only, without any mucosal involvement, but this represents a minority of cases. The British Association of Dermatologists' guidelines for the management of pemphigus vulgaris do not specifically address the occurrence of the disease without oral or mucosal lesions, but they do emphasize the importance of a thorough examination of all mucosal surfaces in suspected cases 1.
Key Points to Consider
- The pathophysiology of pemphigus vulgaris involves autoantibodies targeting desmoglein proteins, which are essential components of cell adhesion structures 1.
- The distribution of these target antigens helps explain the typical presentation pattern, with mucosal surfaces usually affected first or most prominently.
- When evaluating a suspected case of pemphigus vulgaris without mucosal lesions, clinicians should perform a thorough examination of all mucosal surfaces, as subtle lesions may be present, and consider other pemphigus variants in the differential diagnosis.
- The management of pemphigus vulgaris typically involves the use of corticosteroids, with or without adjuvant immunosuppressants, as outlined in the British Association of Dermatologists' guidelines 1.
Clinical Implications
- Clinicians should be aware of the possibility of pemphigus vulgaris occurring without oral or mucosal lesions, although this is uncommon.
- A thorough examination of all mucosal surfaces is essential in suspected cases, and other pemphigus variants should be considered in the differential diagnosis.
- The management of pemphigus vulgaris should be guided by the British Association of Dermatologists' guidelines, which emphasize the importance of corticosteroids and adjuvant immunosuppressants in inducing and maintaining remission 1.
From the Research
Pemphigus Vulgaris Without Oral or Mucosal Lesions
- Pemphigus vulgaris is a rare mucocutaneous autoimmune disease that typically presents with oral lesions, but it can also occur without them 2.
- The disease is characterized by the presence of circulating IgG autoantibodies against desmoglein 3, a protein found in the desmosomes of stratified squamous epithelium 2.
- While oral lesions are often the first sign of pemphigus vulgaris, some patients may not develop them, or they may develop cutaneous lesions first 3, 2.
- There is no clear evidence to suggest that pemphigus vulgaris can occur without any mucosal or cutaneous lesions, as the disease is defined by the presence of autoantibodies against desmoglein 3 and the resulting blistering lesions 3, 2.
Clinical Presentation and Diagnosis
- Pemphigus vulgaris can present with a range of symptoms, including vesiculobullous lesions, ulcers, and erosions on the skin and mucous membranes 4, 2.
- The diagnosis is typically confirmed by biopsy and histological examination, as well as immunostaining to detect the presence of autoantibodies against desmoglein 3 2.
- While the presence of oral lesions can be a clue to the diagnosis of pemphigus vulgaris, their absence does not rule out the disease, and other diagnostic tests may be necessary to confirm the diagnosis 4.
Treatment and Management
- The treatment of pemphigus vulgaris typically involves systemic immunosuppression with corticosteroids, azathioprine, or other agents 5, 6.
- In severe cases, combination therapy with immunoadsorption, rituximab, and pulsed dexamethasone may be necessary to induce remission 6.
- The goal of treatment is to control the disease, prevent relapses, and minimize adverse events associated with immunosuppressive therapy 5.