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Pemphigus David S JOURNAL OF INSURANCE MEDICINE Copyright Q 2004 Journal of Insurance Medicine J Insur Med 2004;36:267±268 GRAPHICS Pemphigus David S. Williams, MD Pemphigus vulgaris and the related pemphigus vegetans, pemphi- Address: Ohio National Financial gus foliaceus and pemphigus erythematous, are associated with the Services, One Financial Way, Cin- highest mortality among primary skin diseases. Typical ®ndings are cinnati, Ohio 45242. illustrated. Correspondent: David S. Williams, MD, FACP. Key words: Mucous membranes, blisters, systemic infection, immu- nosuppressants. emphigus is an acquired autoimmune The natural untreated course of this dis- P disorder of the skin and mucous mem- ease is slow progression with extensive de- branes most often affecting those between the nudation leading to ¯uid and electrolyte im- ages of 30±70 years. The basic abnormality balance, metabolic derangements, sepsis, and lies in the destruction of the mucopolysaccha- death. Diagnosis is made by skin biopsy of ride protein complex of intercellular cement. early vesicles for routine histologic exam. Di- The tono®brils within cells become disorga- rect immuno¯uorescence shows deposits of nized and detached from desmosomes, fol- immunoglobulins (usually IgG) and/or C3 in lowed by a dissolution of intercellular bridges the intercellular spaces around keratinocytes. in the epidermis just above the basal cell lay- Antibodies to the intercellular areas of the er. This leads to a separation of epidermal epidermis may also be found in the serum of cells so that bullae are formed within the epi- patients. dermis, with a minimal amount of in¯am- Pemphigus is classi®ed by the level and mation. type of splitting in the epidermis and by dif- The disease in its more aggressive form ferences in the course of the disease. The commonly presents with mucous membrane more aggressive pemphigus vulgaris and its lesions of the mouth, tongue and cheeks. Su- variant pemphigus vegetans are character- per®cial ulcerations with peripheral extension ized by a suprabasilar split. The latter is so are more often seen than intact blisters. The known because it heals with hypertrophic blisters are initially sparse, but soon become ``vegetative'' surfaces. Pemphigus foliaceus more generalized. The thin, ¯accid, ¯uid- and its variant pemphigus erythematosus are ®lled, super®cial bullae quickly rupture to characterized by a subcorneal split. These become painful, denuded, bleeding, weeping, two more indolent forms of pemphigus tend non-healing erosions. Often times the normal to involve the scalp and face in a seborrheic skin between lesions can be removed simply dermatitis-like eruption, which often simu- by ®rm stroking. lates the butter¯y rash of systemic lupus er- 267 JOURNAL OF INSURANCE MEDICINE prabasilar epidermal split. Involvement is more common in ¯exural areas (neck, axillae, groin), and may be precipitated by warm weather or super®cial bacterial infections. It differs from other forms of pemphigus in its genetic pattern, absence of mouth lesions, be- nign course, and absence of intercellular an- tibodies. Untreated, pemphigus vulgaris is invari- ably fatal in an average time of 14 months. Pemphigus vegetans runs a more prolonged Figure 1. Oral erosions usually precede the onset of skin course than pemphigus vulgaris, but early blisters by weeks or months. From: Habif T, Campbell J Jr, death without treatment remains the rule. In Quitadamo M, Zug K. Skin Disease Diagnosis and Treatment. St. Louis, Mo; Mosby Inc: 2001. Reprinted with contrast, pemphigus foliaceus and erythe- permission from Mosby an af®liate of Elsevier Science. matosus may follow a relatively benign q2001 by Mosby, Inc. course often lasting over a decade. High dos- es of systemic steroids (100±200 mg of Pred- nisone per day) over prolonged periods usu- ally controls the disease. Methotrexate and other cytotoxic drugs are useful as steroid- sparing agents. Lower doses of medication will usually control the more indolent vari- ants of pemphigus. Exacerbations of familial benign pemphigus may respond to systemic and/or topical antibiotics alone. The mortal- ity of the more aggressive pemphigus vari- ants remains high even with systemic steroid and immunosuppressive treatment. It may decrease over time once a remission is sus- Figure 2. Flaccid blisters rupture easily because the roof, tained. which consists only of a thin portion of the upper epidermis, is very fragile. Healing is with brown pigmentation but without scarring. From: Habif T, Campbell J Jr, Quitadamo REFERENCES M, Zug K. Skin Disease Diagnosis and Treatment. St. Louis, Mo; Mosby Inc: 2001. Reprinted with permission 1. Stewart W, Danto J, Maddin S. Dermatology: Diag- q from Mosby an af®liate of Elsevier Science. 2001 by Mos- nosis and Treatment of Cutaneous Disorders. St. Louis, by, Inc. Mo: The C.V. Mosby Company; 1978:127±145. 2. Parker F. Skin Diseases. In: Wyngaarden J, Smith L, eds. Textbook of Medicine. Philadelphia, Pa: W.B. ythematosis. Likewise, they seldom involve Saunders Company; 1988:2328±2332. the mucous membranes. Lastly, familial be- 3. Brackenridge R. Miscellaneous Impairments. In: nign pemphigus, or Hailey-Hailey disease, is Brackenridge R, Elder J, eds. Medical Selection of Life a dominantly inherited disorder with a su- Risks. New York, NY: Stockton Press; 1998:937±938. 268.
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