Phemphigus Vulgaris
Neha Bansal
MBBS, KEM Hospital, Mumbai, India
First Created: 01/02/2013 

Introduction

Definition:

Phemphix means bubbles or blisters in Greek. Pemphigus Vulgaris refers to the blistering auto-immune disease of the skin and mucous membranes.1

Pathogenesis:

It is caused due to the auto-antibodies to the desmogleins (desmoglein 3), which are intracellular adhesion proteins.1-3 This results in the damaged cell to cell adhesions and hence, the formation of blisters and ulcers.2 Even though it is thought to be genetically transmitted, some of the etiological agents considered are garlic in diet, drugs with sulfhydryl radical, cosmetics, Human herpesvirus -8 (HHV-8) and is often associated with myasthenia gravis.2

Signs & Symptoms:

May start with ocular symptoms, though long term complications rarely occur. Oral mucosa is most commonly involved with ulcerations and blister formation1,2, and skin blisters show Nikolsky sign (peeling of epithelium on tangential pressure).1 The milder form known as Pemphigus foliaceous affects the more superficial layer and the blisters crust over the face, upper chest, and usually don't involve the mucosa.1,3 It should be considered in cases of chronic mucositis in children and adolescent population.

Age groups affected:

Usually affects older age group (40-60 yrs) and is rare in children. However, cases have been reported. A transient form is seen in neonates of affected mothers. It is most commonly seen in the Ashkenazi Jewish population.1,2

Phemphigus Vulgaris - Differential Diagnosis

Stevens-Johnson syndrome, aphthous stomatitis, herpes simplex virus infection, impetigo, contact dermatitis, Behcet's disease

Phemphigus Vulgaris - Diagnosis

Perilesional or oral mucosal biopsy. IgG or complement detection by immunofluorescence in intercellular cells of the epidermis.1 ELISA for antibodies to Dsg-1 & Dsg-3 is now available.2

Treatment

It was fatal before the development of immune-suppressants due to dehydration or secondary infections.1, 2 High dose steroids (1-6 mg/kg/day) are usually the drug of choice.1,2 Adjuvant therapy with other immune-suppressant drugs like cyclosporine, cyclophosphamide, methotrexate, dapsone, IVIG can be tried.1,2 For milder forms, topical steroid therapy can suffice.2


1. Merchant S, Weinstein M. Pemphigus Vulgaris: The Eyes Have It. Pediatrics 2003; 112; 183-185.
2. Scully C, Challacombe SJ. Pemphigus vulgaris: update on etiopathogenesis, oral manifestations, and management. Crit Rev Oral Biol Med. 2002; 13: 397-408.
3. Mahoney MG, Wang Z, Rothenberger K, Koch PJ, Amagai M, Stanley JR. Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. J Clin Invest. 1999; 103: 461-468.


Phemphigus Vulgaris Phemphigus Vulgaris 01/02/2013
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