Pemphigus Foliaceus Clinical Presentation

Updated: Mar 30, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Presentation

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. [36] Eyelid skin involvement without conjunctival changes occurs occasionally in patients with pemphigus foliaceus. [37]

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Physical Examination

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. [38]

The erosions can become numerous, showing a tendency to generalize. Occasionally, erythrodermia develops. Thus, pemphigus foliaceus may be evident as a an erythroderma or a psoriasiform erythroderma. [35, 39] 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. Note the images below.

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

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 associates [8] 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.

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Complications

Monitor pemphigus foliaceus (PF) patients for other autoimmune disorders, particularly thymoma and myasthenia gravis.

New cutaneous pain or new constitutional symptoms, change in primary morphology, rapid disease progression, or failure to respond to appropriate therapies may suggest a concurrent cutaneous viral infection such as by herpes simplex or cytomegalovirus. [40]

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