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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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Medicines

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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Practice pearl: gargling with cholinergic ophthalmic drops for treating the oral lesions of pemphigus vulgaris
From Journal of Drugs in Dermatology, 9/1/04 by M.R. Namazi

Painful oral lesions are seen in almost all patients with pemphigus vulgaris and are the presenting complaint in 50-70% of patients (1). Mucosal lesions are very recalcitrant. often healing after skin lesions when immunosuppressive therapy is given, and they may persist even though skin lesions are controlled. Topical corticosteroids or prostaglandin E2 has been claimed to be effective (2).

Below, based on the role of muscarinic receptors in keratinocyte biology and the encouraging results of the recent trials with cholinergic drugs in the treatment of pemphigus, a novel therapeutic approach for this troublesome condition is presented:

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 autontibodies 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 topical suramin sodium and systemic Mestinon (pyridostigmine bromide), two cholinomimetic agents, have been employed successfully in the treatment of pemphigus (3,5). Moreover, gargling with diphenhydramine syrup, used for alleviating the pain of pemphigus oral lesions, has recently been claimed to slow down the healing of the lesions due to the anticholinergic effect of this agent (7).

Stimulation of the keratinocyte-acetylcholine axis may lead to a therapeutic effect through any of the following mechanisms: (a) stimulating keratinocyte cell-to-cell attachment; (b) accelerating reepithelialization; and (c) competing with the disease-causing pemphigus antibodies, preventing them from attachment to keratinocytes (6).

Cholinergic ophthalimic drops, such as pilocarpine 4% drops, have been exploited by psychiatrists for the treatment of xerosthomia induced as the anti-cholinergic side effect of some psychiatric medications such as tricyclic antidepressants. I recommend gargling with these drops for expediting the healing of pemphigus oral lesions.

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 edn. Vol.4. Oxford: Blackwell Scientific Publications. 1998; 1847-65.

2. Scully C. The Oral Cavity. In: Rook/Wilkinson/Ebling Textbook of Dermatology (Champion RH. Burton JL. Burns DA. Breatnach SM, eds). 6th edn. Vol4. Oxford; Blackwell Scientific Publications. 1998; 3083-4.

3. Nguyen VT, et al. The pathophysiological significance of nondesmoglein targets of pemphigus autoimmunity Arch Dermatol 1998; 134:971-80.

4. Nguyen VT, et al. 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.

6. Grando SA. New approaches to the treatment of pemphigus. J Invest Dertmatol Symp Proc. 2004; 9(1):84-91.

7. Namazi MR. Diphenhydramine gargling may slow the healing of oral lesions of pemphigus vulgaris J Drugs Dermatol. 2004; 3(1):12.

8. Grebb JA. Biological Therapies. In: Kaplan and Sadock's Comprehensive Textbook of Psychiatry (Sadock BJ. Sadock VA, eds). 7th edn. Vol 2. Lippincott Williams & Wilkins. 2000; 2240.

M.R. Namazi, MD

P.O.Box: 71955-687

Shiraz. Iran

Email: namazi_mr@yahoo.com

COPYRIGHT 2004 Journal of Drugs in Dermatology, Inc.
COPYRIGHT 2005 Gale Group

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