Pemphigus Erythematosus Workup

Updated: Mar 02, 2018
  • Author: Rakesh Bharti, MD, MBBS; Chief Editor: William D James, MD  more...
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Workup

Laboratory Studies

With direct immunofluorescence (see the image below) in pemphigus erythematosus, linear deposits of immunoglobulin G (IgG) and C3 are present in the intercellular space of the epidermis. Granular deposits of C3 and IgG at the dermoepidermal junction are present in 80% of patients, particularly in biopsy specimens from the face or other sun-exposed areas. [7]

Direct immunofluorescence microscopy performed on Direct immunofluorescence microscopy performed on epithelial biopsy specimen obtained from a patient with pemphigus vulgaris detects immunoglobulin G deposits at the epithelial cell surfaces.

With immunoelectron microscopy in pemphigus erythematosus, IgG and C3 deposits are localized to the epidermal cell membranes and the upper dermis.

Patients with pemphigus erythematosus may have other laboratory abnormalities suggestive of systemic lupus erythematosus (SLE); these include anemia, lymphopenia, thrombocytopenia, renal abnormalities, proteinuria, or a positive rheumatoid factor.

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Procedures

In pemphigus erythematosus, select an early vesicle or bulla for skin biopsy. Perilesional skin is tested on immunofluorescence studies.

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Histologic Findings

Intraepidermal superficial bullae are usually within the granular layer or just below it. Acantholysis may occur in the blister floor or roof. Old lesions may have follicular hyperkeratosis with acantholysis and dyskeratosis of the granular layer. See the images below.

Biopsy shows moderate epithelial hyperplasia with Biopsy shows moderate epithelial hyperplasia with a suprabasal cleft that shows suprabasal acantholysis. The rest of the epithelium shows spongiosis with neutrophils. The submucosa has a moderately dense mixed perivascular infiltrate of lymphocytes and neutrophils. At places, the epithelium is missing, and the surface is covered by fibrin and necrotic inflammatory cells. Photo courtesy of Dr. Uday Khopkar.
Biopsy shows upper epidermal acantholytic blisteri Biopsy shows upper epidermal acantholytic blistering dermatitis involving the granular and upper spinous layer. The blister contains plasma, RBCs, and few acute inflammatory cells. The epidermis at the periphery of the blister shows mild spongiosis with neutrophils. In the roof of the blister, a few elongated acantholytic cells can be seen. Underlying dermis shows superficial and mid perivascular mixed infiltrate of neutrophils and lymphocytes. Photo courtesy of Dr. Uday Khopkar.
Biopsy shows upper epidermal acantholytic blisteri Biopsy shows upper epidermal acantholytic blistering dermatitis involving the granular and upper spinous layer with absence of roof of blister. The epidermis shows mild spongiosis with neutrophils. Underlying dermis shows superficial and mid perivascular mixed infiltrate of neutrophils and lymphocytes. Photo courtesy of Dr. Uday Khopkar.
Biopsy shows sparse superficial and deep perivascu Biopsy shows sparse superficial and deep perivascular infiltrate of lymphocytes. The papillary dermis is edematous and there is extravasation of RBCs. Basal layer shows vacuolization and interface infiltration by lymphocytes. Reticular dermis shows small amount of mucin. Photo courtesy of Dr. Uday Khopkar.
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