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Pemphigus

Pemphigus is an autoimmune disorder that causes blistering and raw sores on skin and mucous membranes. As with other autoimmune disorders, it is caused when the body's defenses mistake its own tissues as foreign, and attack the cells. more...

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Types

There are three types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus.

The most common form of the disorder is pemphigus vulgaris (ICD-10 L10.0). It occurs when antibodies attack Desmoglein 3, a protein that keeps cells bound together. Thus, cells simply fall apart, causing skin to slough off. Although pemphigus vulgaris may occur at any age, it is quite rare in children, and most common in the middle aged and elderly. Sores often originate in the mouth, making eating difficult and uncomfortable.

Pemphigus foliaceus (ICD-10 L10.2) is the least severe of the three varieties. Desmoglein 1, the protein that is destroyed by the body's immune system is only found in the top dry layer of the skin, so mouth sores do not occur. Pemphigus foliaceus is characterized by crusty sores that often begin on the scalp, and may move to the chest, back, and face. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as dermatitis or eczema.

The least common and most severe type of pemphigus is the neoplastic variety. This disorder is usually found in conjunction with an already-existing malignancy. Very painful sores appear on the mouth, lips, and the esophagus. A diagnosis of neoplastic pemphigus may prompt a search for an existing tumor. Sometimes, the tumor is not malignant. In these cases, tumor removal may lead to a remission of the pemphigus.

Treatment

If not treated, pemphigus is usually fatal, due to overwhelming systemic infection. The most common treatment is the administration of oral steroids, especially prednisone. Mild cases sometimes respond to the application of topical steroids. All of these drugs may cause severe side effects, so the patient should be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects.

If skin lesions do become infected, antibiotics may be used for treatment. In addition, talcum powder is helpful to prevent oozing sores from adhering to bedsheets and clothes.

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Diphenhydramine gargling may slow the healing of oral lesions of pemphigus vulgaris - Letters to the Editor - Letter to the Editor
From Journal of Drugs in Dermatology, 1/1/04

Oral lesions are seen in almost all patients with pemphigus vulgaris and are the presenting complaint in 50-70% of patients (1). As these lesions are painful and cause much discomfort, especially while eating some clinicians advise patients to gargle diphenhydramine syrup, which is an effective local anesthetic (2). Based on the role of muscarinic receptors in keratinocyte biology, this letter contests the appropriateness of this therapeutic approach.

It has been shown that in addition to anti-desmosome autoantibodies, the pemphigus serum contains autoantibodies to keratinocyte muscarinic cholinergic receptors (3). These receptors, being expressed mainly in the lower layers of epidermis, control keratinocyte adhesion (4) and their inactivation by pemphigus autoantibodies is believed to elicit intracellular signals that cause disassembly of desmosomes, contributing to acantholysis and blistering (3).

Acetylcholine, synthesized by keratinocytes, regulates keratinocyte adhesion and reverses pemphigus IgG-induced acantholysis in keratinocyte monolayers (2,5). Of note is that suramin sodium, a drug acting on acetylcholine receptors, has been employed to treat pemphigus (3).

Conclusively, given that keratinocyte muscarinic receptors control keratinocyte adhesion and that diphenhydromine possesses potent anti-muscarinic effects (2), gargling of diphenhydramine syrup may slow the healing of the oral lesions of pemphigus and therefore it is best replaced with other topical anesthetics devoid of anticholinergic activity.

References:

1. Wojnarowska F, Eady RAJ, Burge SM. Bullous Eruptions. In: Rook/Wilkinson/Ebling Textbook of Dermatology (Champion RH, Burton JL, Burns DA, Breatnach SM, eds). 6th ed. Vol. 4. Oxford, Blackwell Scientific Publications 1998; 1847-65.

2. Burkohalter A, Frick OL. Histamine, Serotonin & the Ergot Alkaloids. In: Basic & Clinical Pharmacology (Katzung BG, editor). 5th ed. Connecticut: Prentice-Hall International Inc., 1992; 233-7.

3. Nguyen VT, Lee TX, Ndoye A, Shultz LD, Pittelkow MR, Dahl MV, et al. The pathophysiological significance of nondesmoglein targets of pemphigus autoimmunity. Arch Dermatol 1998; 134:971-80.

4. Nguyen VT, Anedondo J, Chernyavsky AI, Kitajima Y, Grad SA. Keratinocyte acetylcholine receptors regulate cell adhesion. Life Sci 2003; 72(18-19):2081-5.

5. Grando SA, Dahl MV. Activation of keratinocyte muscarinic acetylcholine receptors reverses pemphigus acantholysis. J Eur Acad Dermatol Venereol 1993; 2:72-86.

Corresponding address:

M R Namazi MD

Dermatology Department

Shiraz University of Medical Sciences

PO Box 71955-687

Shiraz, Iran

Email: namazi_mr@yahoo.com

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