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Clinical Features

  • Pemphigus vulgaris (Figures 185-1, 185-2, 185-3, and 185-4)—Classical lesions are flaccid bullae that rupture easily, creating erosions. Since bullae are short-lived, erosions are the more common presenting physical finding (Figure 185-13). Lesions are typically tender and heal with postinflammatory hyperpigmentation that resolves without scarring. A positive Asboe-Hansen or Nikolsky sign may be present, but neither sign is diagnostic. A positive Asboe-Hansen sign occurs when a bulla extends to surrounding skin while pressure is applied directly to the bulla. The Nikolsky sign is positive when skin shears off while lateral pressure is applied to unblistered skin during active disease. Sometimes the Asboe-Hansen sign is also attributed to Nikolsky and called a Nikolsky sign, too.
  • Pemphigus vegetans is a variant of PV where healing is associated with vegetating proliferation of the epidermis (Figures 185-5 and 185-6).
  • Pemphigus foliaceous: Multiple red, scaling, crusted, and pruritic lesions described as “corn flakes” are seen. Shallow erosions arise when crusts are removed, but intact blisters are rare as the disease is superficial (Figures 185-7, 185-8, 185-9, 185-10, 185-11, and 185-12).
  • PNP (Figure 185-14)—Lesions are similar to PV, although lichen planus, morbilliform, or erythema multiforme-like lesions also may be seen in addition to blisters and erosions. Another distinctive feature is the presence of epithelial necrosis and lichenoid changes in the lesions. Pulmonary involvement secondary to acantholysis of bronchial mucosa is seen in 30% to 40% of cases of PNP.

Figure 185-13

Pemphigus vulgaris involving the face and oral mucosa. The erosions are deeper than those seen in pemphigus foliaceous. The oral involvement points to pemphigus vulgaris. (Courtesy of Richard P. Usatine, MD.)

Figure 185-14

Paraneoplastic Pemphigus with severe erosions covering practically the entire mucosa of the oral cavity with partial sparing of the dorsum of the tongue. Lesions are extremely painful, interfering with adequate food intake. This patient had non-Hodgkin lymphoma as the underlying malignancy. (Courtesy of Wolff K, and Johnson RA. Fitzpatrick's Color Atlas & Synopsis of Clinical Dermatology, Sixth Edition. New York: McGraw-Hill; 2009, Fig.18-19.)

Typical Distribution

  • PV—Common mucosal site is oral mucosa, although any stratified squamous epithelium may be involved. Mucosal lesions may be followed by skin lesions after weeks to months usually on scalp, face, and upper torso. PV should be suspected if an oral ulcer persists beyond a month (Figures 185-1, 185-3, and 185-15).
  • Pemphigus vegetans—Usually seen in intertriginous areas like the axilla, groin, and genital region (Figures 185-5 and 185-6).
  • PF—Initially affects face and scalp, though may progress to involve chest and back (Figures 185-7, 185-8, 185-9, 185-10, 185-11, and 185-12). When the facial involvement in PF is in a lupus-like pattern, this is called pemphigus erythematosus (Figure 185-16).
  • PNP—Common sites include oral mucosa and conjunctiva. (Figure 185-14) Columnar and transitional epithelia may also be involved besides stratified squamous epithelium.

Figure 185-15

Pemphigus vulgaris involving the tongue and lips of a young woman. This is severely painful, making it difficult to eat or drink. (Courtesy of Richard P. Usatine, MD.)

Figure 185-16

Pemphigus erythematosus creating a lupus-like pattern of facial involvement. Note how the pemphigus foliaceous lesions involve the malar areas bilaterally. (Courtesy of Richard P. Usatine, MD.)

Laboratory Studies

  • Circulating desmoglein antibodies levels may be measured in the blood using indirect immunofluorescence. This is usually not necessary unless the diagnosis is in question and further data are needed.
  • Complete blood count and a comprehensive metabolic profile including liver function tests, creatinine, and glucose will be needed as a baseline, as all the systemic therapies have significant toxicities.
  • Patients at risk for steroid-induced osteoporosis should have a dual-energy x-ray absorptiometry (DEXA) scan performed.

Biopsy

  • Skin biopsy is essential for accurate diagnosis. The depth of acantholysis and site of deposition of antibody complexes help differentiate pemphigus from other bullous diseases. Two specimens should be sent. Perform a shave of the edge of the bulla to include the surrounding normal appearing epidermis. This biopsy should be of the freshest lesion with an intact bulla, if possible. Cut the specimen in half and send the portion with the bulla in formalin for routine histopathology. The second half should be perilesional adjacent normal skin. This is sent on a gauze pad soaked in normal saline or Michel solution for direct immunofluorescence (DIF). Routine histopathology demonstrates suprabasal acantholysis and DIF shows antibody deposition in the intercellular spaces of the epidermis. The pattern of the DIF fluorescence is described as chicken wire (Figure 185-17).

Figure 185-17

Direct immunofluorescence against immunoglobulin (Ig) G antibodies surrounding cells of the epidermis in a patient with pemphigus vulgaris. Note the chicken-wire appearance. (Courtesy of Martin Fernandez, MD, and Richard P. Usatine, MD.)

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