eMedicine Specialties > Dermatology > Bullous Diseases

Pemphigus Foliaceus

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Slawomir Majewski, MD, Professor and Director, Department of Dermatology and Venereology, Warsaw School of Medicine, Poland; Sebastian S Majewski, MD, Consulting Staff, Department of Dermatology, Military Institute of Health Services, Warsaw, Poland
Contributor Information and Disclosures

Updated: May 5, 2009

Introduction

Background

Pemphigus foliaceus (PF) is generally a benign variety of pemphigus. It is an autoimmune skin disorder characterized by the loss of intercellular adhesion of keratinocytes in the upper parts of the epidermis (acantholysis), resulting in the formation of superficial blisters. It is typified by clinical involvement of healthy-appearing skin that blisters when rubbed (the Nikolsky sign; commonly but incorrectly spelled Nicholsky), a finding named after Dr Piotr Nikolsky, who first described this sign in 1896.1 Pemphigus foliaceus is characterized by a chronic course, with little or no involvement of the mucous membranes.

Pierre Louis Alphee Cazenave, founder of the first journal dedicated entirely to dermatology, documented the first description of pemphigus foliaceus in 1844 in this journal. The description was of a 47-year-old woman who consulted him at l'Hopital Saint Louis in Paris for a generalized eruption of several years' duration. Nikolsky described lateral extension of the preexisting erosion due to lifting up the collarette (and when applying a lateral pressure to the clinically intact skin), whereas Asboe-Hansen described extension of the intact blister due to pressure that is applied to its roof.

Pemphigus foliaceus has the following 6 subtypes: pemphigus erythematosus (PE), pemphigus herpetiformis (PH), endemic pemphigus foliaceus, endemic pemphigus foliaceus with antigenic reactivity characteristic of paraneoplastic pemphigus (but with no neoplasm), immunoglobulin A (IgA) pemphigus foliaceus, and drug-induced pemphigus foliaceus. See Pemphigus Erythematosus; Pemphigus Herpetiformis; Pemphigus, Paraneoplastic; and Pemphigus, IgA for more information.

Senear and Usher originally described PE in 1926 as an unusual type of pemphigus with features of lupus erythematosus. PE (also known as Senear-Usher syndrome) is best viewed as a localized form of pemphigus foliaceus. Chorzelski et al2 determined its immunopathology in 1968.

Another pemphigus foliaceus variant with pruritic, flaccid vesicles in an annular pattern has been characterized as IgA pemphigus foliaceus, with antibodies of IgA class providing the basis for diagnosis.

Jablonska and associates3 coined the term pemphigus herpetiformis for the pemphigus foliaceus variant that often begins as small clusters of pruritic papules and vesicles mimicking dermatitis herpetiformis.

Endemic pemphigus foliaceus, or fogo selvagem (formerly known as Brazilian pemphigus foliaceus because it is evident mainly in the river valleys of rural Brazil), has also been described in Columbia, El Salvador, Paraguay, Peru, and recently in Tunisia. Fogo selvagem (Portuguese for wild fire) displays immunopathologic findings of pemphigus and a distinctive epidemiology suggestive of a disorder triggered by an infectious insect-borne agent (see Fogo Selvagem). A focus of endemic pemphigus foliaceus also exists in El Bagre, Columbia and shares features with Senear-Usher syndrome but occurs in an endemic fashion.4,5 Heterogeneous antigenic reactivity was observed as in paraneoplastic pemphigus but with no evidence of association with neoplasia. This endemic pemphigus disease in El Bagre had immunologic features similar to pemphigus foliaceus or erythematosus.

Chorzelski et al in 19996 described paraneoplastic pemphigus with cutaneous and serologic features of pemphigus foliaceus in a patient with an underlying lymphoma. The authors are not aware of any similar patients with these highly unusual findings.

Drug-induced pemphigus foliaceus is mostly associated with penicillamine, nifedipine, or captopril, medications with a cysteinelike chemical structure.

A transition from pemphigus vulgaris (PV) to pemphigus foliaceus, or vice versa, is not likely. However, in the experience at the Medical University of Warsaw, PV in the remission period may resemble pemphigus foliaceus. About 7% of patients with pemphigus foliaceus may have the initial features of PH. This figure was 35% in patients with endemic pemphigus foliaceus in Tunisia (see Pemphigus Vulgaris).

Pathophysiology

Superficial blisters in pemphigus foliaceus are induced by immunoglobulin G (IgG) (mainly IgG4 subclass) autoantibodies directed against a cell adhesion molecule, desmoglein 1 (160 kd), expressed mainly in the granular layer of the epidermis. Desmoglein 1 is also a major autoantigen in cases of PH, suggesting that most cases of both PE and PH are clinical variants of pemphigus foliaceus. The mechanism of acantholysis induction by specific autoantibodies may involve phosphorylation of intracellular proteins associated with desmosomes. Complement activation does not play a pathogenic role in pemphigus foliaceus.

Antibodies against desmoglein 3 are also present in patients with paraneoplastic pemphigus (PNP), a severe condition associated with various antibodies against different components of the cell adhesion complex. Other target antigens, including the acetylcholine receptor, have also been postulated to be relevant in the pathogenesis of pemphigus foliaceus.7,8,9,10

Cholinergic control of epidermal cohesion may be important.11 The regulation of keratinocyte cell-to-cell and cell-matrix adhesion is an important biological function of cutaneous acetylcholine. Recent progress in therapy of pemphigus using cholinergic drugs supports this concept.

Precipitating factors include medications and ultraviolet light radiation. It was recently suggested that both enhanced autoantibody epidermal binding and preferential neutrophil adhesion to UV-irradiated epidermis contribute to acantholysis development in photo-induced pemphigus foliaceus.

Endemic pemphigus foliaceus seems to have an environmental cause. The prevalence of antibodies against desmoglein 1 is high in people residing in endemic areas of Brazil, with disease onset preceded by a sustained antibody response due to an as yet unknown environmental factor.

The role of genetic factors is evident in fogo selvagem in which a strong association exists with some human leukocyte antigen DRB1 (HLA-DRB1) haplotypes, including DRB1*0404, 1402, 1406, and 1401. In France, persons with DRB1*0102 and 0404 are at an increased risk of pemphigus foliaceus.

Pemphigus trigger factors have been meticulously analyzed by Ruocco and Ruocco12 , who have delineated an exhaustive list and stressed the need to detect environmental provoking or precipitating factors. As a superb memory device to facilitate thorough patient evaluation, Ruocco13 has cleverly observed that PEMPHIGUS should encourage the physician to consider pesticides (PE), malignancy (M), pharmaceuticals (P), hormones (H), infectious agents (I), gastronomy (G), ultraviolet light (U), and stress (S).

Rarely, a change in pemphigus subtype may occur, accompanied by qualitative and quantitative changes in the anti-Dsg autoantibody profile as detected using antigen-specific enzyme-linked immunosorbent assays (ELISAs). Thus, the antibody profile defines the clinical phenotypes of pemphigus, and that intermolecular "epitope spreading" may be the immunological mechanism underlying a shift between pemphigus foliaceus and PV.14

Frequency

International

The incidence of pemphigus foliaceus varies depending on the population studied. Pemphigus foliaceus is rare and sporadic worldwide. In contrast to PV, no predominance of pemphigus foliaceus is found in Jews and in people of Mediterranean descent. An increased incidence of pemphigus foliaceus was noted in Tunisian women (6.6 cases per million per year), whereas, in Western Europe, the incidence of pemphigus foliaceus is about 0.5-1 case per million per year.15

Endemic pemphigus foliaceus, or fogo selvagem, occurs with a high frequency in central and southwestern Brazil and in Colombia. The Terena reservation in Brazil, a recently identified focus, has a prevalence of 3.4% of the population. In endemic regions of Brazil, as many as 50 cases per million per year are seen.16 Other foci may be present in the Maghreb; one was described in Morocco.

Mortality/Morbidity

Pemphigus foliaceus tends to persist for months to years. PE may coexist with thymoma, myasthenia gravis, lupus erythematosus, and other autoimmune bullous diseases.

Race

Pemphigus foliaceus has been described in all races.

Sex

In general, the prevalence of pemphigus foliaceus in men and women is about equal; however, in the Sousse region of Tunisia, an overwhelming predominance of women are affected. The peak incidence of endemic pemphigus foliaceus in women aged 25-34 years in the Sousse region of Tunisia is 15.5 cases per million per year. In El Salvador, a similar female and age predisposition may also be evident.

Age

The mean patient age at onset of pemphigus foliaceus is about 50-60 years; however, it may occur at any age, from infancy onward. Fogo selvagem often occurs in children, young adults, and genetically related family members. The mean patient age at onset is about 20-30 years. The peak incidence of endemic pemphigus foliaceus occurs in women aged 25-34 years in the Sousse region of Tunisia. An increased incidence in genetically related family members does not appear to exist.

Clinical

History

  • The bullae usually start on the trunk. The course of the disease is long-term, with the patient's general health being satisfactory.
  • Spontaneous remission sometimes occurs, but the lesions can persist for several years.
  • A unique clinical pattern may occur in children, with individual lesions appearing as arcuate, circinate, or polycyclic.17
  • Eyelid skin involvement without conjunctival changes occurs occasionally in patients with pemphigus foliaceus.18

Physical

  • The primary lesions are small, superficial blisters; however, these flaccid bullae are difficult to find because they are transient and transform into erosions.
    • Typical pemphigus foliaceus has scaly, crusted erosions on an erythematosus base confined mainly to so-called seborrhoic areas (eg, face, scalp, upper part of the trunk).
    • The Nikolsky sign is the finding that physical trauma can shear the pathologic epidermis of the skin of patients with pemphigus foliaceus, resulting in clinical lesions. The Nikolsky sign should probably be regarded as a moderately sensitive but highly specific tool for the diagnosis of pemphigus.19
    • The erosions can become numerous, showing a tendency to generalize.
    • Occasionally, erythrodermia develops.
    • Atrophic changes of the nails and the hair are sometimes evident.
    • The erosions may be accompanied by a burning sensation and local pain.
    • In contrast to PV, in pemphigus foliaceus, little or no involvement of the mucous membranes occurs.
Middle-aged American woman of Mexican lineage wit...

Middle-aged American woman of Mexican lineage with superficial bullae characteristic of pemphigus foliaceus.

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Middle-aged American woman of Mexican lineage wit...

Middle-aged American woman of Mexican lineage with superficial bullae characteristic of pemphigus foliaceus.


Pemphigus foliaceus. Middle-aged American woman o...

Pemphigus foliaceus. Middle-aged American woman of Mexican lineage with superficial bullae formation.

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Pemphigus foliaceus. Middle-aged American woman o...

Pemphigus foliaceus. Middle-aged American woman of Mexican lineage with superficial bullae formation.


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A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.

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A 41-year-old woman of Puerto Rican origin with a...

A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.


A 41-year-old woman of Puerto Rican origin with a...

A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.

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A 41-year-old woman of Puerto Rican origin with a...

A 41-year-old woman of Puerto Rican origin with a 9-year history of pemphigus foliaceus, often with erythroderma flares.

  • IgA pemphigus foliaceus begins as pruritic, flaccid vesicles in an annular pattern.
  • PH commences as intensely pruritic, grouped papules and vesicles suggestive of dermatitis herpetiformis. Erythematous patches with peripheral vesicles may be present. Sometimes, oral erosions are seen.
  • PE starts as erythematous patches with border vesiculation, often in a butterfly distribution on the cheeks and the forehead, with similar patches on the sternal and interscapular skin. Crusted plaques may appear in the healing phase.
  • PNP is a subset of pemphigus combining the clinical features of PV variably associated with those of erythema multiforme, bullous pemphigoid, and lichen planus. Chorzelski and associates6 in 1999 described a most unusual case of PNP with the immunopathologic findings of pemphigus foliaceus. The clinical pattern appears to be correlated with that of the antibody profile; therefore, patients with antibodies directed against desmoglein 1 tend to have the clinical features of pemphigus foliaceus.

Causes

  • Endemic pemphigus foliaceus, or fogo selvagem, seems to be induced by a viral infection transmitted by insects.
  • In some patients, pemphigus foliaceus may be precipitated by extensive UV exposure or burns and by various drugs (eg, penicillamine,20,21 inhibitors of angiotensin convertase, nonsteroid anti-inflammatory agents).22 Pemphigus foliaceus induced by bucillamine has been described.23

More on Pemphigus Foliaceus

Overview: Pemphigus Foliaceus
Differential Diagnoses & Workup: Pemphigus Foliaceus
Treatment & Medication: Pemphigus Foliaceus
Follow-up: Pemphigus Foliaceus
Multimedia: Pemphigus Foliaceus
References
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References

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Further Reading

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Keywords

superficial pemphigus, fogo selvagem, PF, pemphigus erythematosus, PE, pemphigus herpetiformis, PH, endemic PF, endemic pemphigus foliaceus, immunoglobulin A PF, IgA PF, IgA pemphigus foliaceus, immunoglobulin A pemphigus foliaceus, drug-induced PF, drug-induced pemphigus foliaceus

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Slawomir Majewski, MD, Professor and Director, Department of Dermatology and Venereology, Warsaw School of Medicine, Poland
Disclosure: Nothing to disclose.

Sebastian S Majewski, MD, Consulting Staff, Department of Dermatology, Military Institute of Health Services, Warsaw, Poland
Disclosure: Nothing to disclose.

Medical Editor

Robin Travers, MD, Assistant Professor of Medicine (Dermatology), Dartmouth University School of Medicine; Staff Dermatologist, New England Baptist Hospital; Private Practice, SkinCare Physicians
Robin Travers, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Informatics Association, Massachusetts Medical Society, Medical Dermatology Society, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Julia R Nunley, MD, Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center
Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society
Disclosure: Johnson and Johnson stock holder dividends; Amgen stock holder dividends; Forest Lab, Inc stock holder dividends; Galaxo Smith Klein stock holder dividends; Covidien stock holder dividends; Novartis Grant/research funds Consulting; Biolex  sub-investigator

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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