Pemphigus is an autoimmune bullous disease clinically characterized by blistering and erosions of the skin and/or mucous membranes. The most common forms are pemphigus vulgaris (PV) and pemphigus foliaceus (PF), accounting for 70 and 20 % of cases, respectively.
Clinical Presentation - PV may involve one or more epithelial tissues, most commonly the oral mucosa. Involvement of the conjunctival, laryngeal, esophageal, genital and nasal mucosae may also occur.
Genetic Susceptibility - it is more prevalent in people from Jewish or Mediterranean ancestry, familial cases, increased prevalence of other autoimmune diseases in the first-degree relatives of pemphigus patients, and detection of anti-Dsg antibodies. Higher incidence of PV and earlier age at onset for pemphigus seen in Indian population have been attributed to higher frequency of DSG3*TCCCC in Indian population.
Diagnosis - Diagnosis involves clinical presentation, serology, direct immunofluorescence microscopy of a perilesional biopsy, and histopathology of a lesional biopsy.
Treatment - Corticosteroids are considered the mainstay of treatment. These are often combined with oral azathioprine or a mycophenolate compound to achieve disease control. In recent years, rituximab (RTX), an anti-CD20 immunobiological drug, has challenged the traditional treatment principles for pemphigus and is now being suggested as a first-line option, along with corticosteroids.
Did you know about such bullous disorder? How have you dealt with such patient in you clinical practice?
Pemphigus Vulgaris is a condition where the body’s immune system mistakenly attacks healthy skin cells, causing painful blisters and sores on the skin and mouth. It’s an autoimmune disease, meaning your body’s defense system is attacking itself. Blisters and sores can be painful and affect daily life. Treatment helps manage symptoms and prevent flare-ups.
Reading about such disorder for the first time and it is quite informative. There are many factors which can trigger this autoimmune response thereby causing this disorder to occur, including some unknown triggers, certain drugs such as penicillamine, ACE inhibitors and some environmental/ regional factors as well.
Thank you for this clear overview. Pemphigus is indeed a challenging autoimmune condition, especially when oral or mucosal involvement makes eating and daily activities painful. The shift toward earlier use of rituximab has been encouraging, since many patients respond well and need fewer steroids.
During my internship period and DVL posting, i have seen pemphigus patient there.
Initially, pemphigus is an autoimmune disorder, which means our own immune fails to recognise our own self. That resulted in destory our own cells.
Generally pemphigus characterized by the blisters symptoms. It produce immunse pain for them.
In our hospital, pemphigus was treated with corticosteroid ( which helps to act as immune suppressant and also reduce inflammation) such as prednisolone or dexamethasone along with cyclophosphamide (DCP therapy).
Well explained with the introduction the mechanism, symptoms, diagnosis and then treatment. Honestly speaking this is very brand new information for me where I got to know about a new diseases which was very informative indeed.
Pemphigus is indeed one of the more challenging autoimmune bullous disorders to manage, especially because patients often present late with severe pain, mucosal involvement, and emotional distress. In clinical practice, early recognition and prompt referral to dermatology are crucial. Supportive care pain control, infection prevention, nutrition monitoring, and psychological reassurance plays a major role alongside medical treatment. Many patients struggle with fear, body image concerns, and anxiety due to the chronic and unpredictable nature of the disease, so a multidisciplinary approach is essential. Rituximab has truly transformed outcomes in recent years, offering faster remission and better quality of life for many individuals.
Pemphigus vulgaris is an autoimmune skin disease characterised by the formation of blisters and erosions on the skin and mucous membranes. Patients are often more concerned about their physical appearance, which can significantly affect their confidence and mental health. Hence, psychological counselling and reassurance are important parts of management.
Since it is an autoimmune condition, complete healing may take a long time, and relapses can occur. Proper blister care and infection prevention are crucial to avoid complications. Corticosteroids are most commonly prescribed.
Absolutely, pemphigus often reveals its first signs in the oral cavity, thus making the role of dentists and oral physicians key in early detection. Buccal mucosa, palate, gingiva, or tongue will have painful blisters or erosions, which may precede skin involvement by months. These lesions can interfere with eating, speaking, and overall nutrition. All this takes a toll on the patient’s quality of life. Thus, early recognition and biopsy confirmation are essential. Thus, it is vital to restore both comfort and confidence in patients by managing pain and oral hygiene alongside systemic therapy.
This is a very insightful article on pemphigus. Yes, I’ve seen patients with this condition in clinical practice, most present with painful oral or skin erosions, and early steroids with immunosuppressants or rituximab make a big difference. Careful monitoring, infection control and counseling about the chronic nature of the disease are essential.