Pemphigus vulgaris presenting as an isolated crusted plaque of the cheek
1. Dermatology Department, Habib Thameur Hospital, Tunisia. email@example.com
A healthy 45-year-old man presented with a three-month history of an isolated enlarging eroded plaque on the left cheek. Pemphigus vulgaris was diagnosed by biopsy and positive direct immunofluorescence. The patient was lost to follow up, but returned two months later with generalized cutaneous involvement without mucosal involvement. He responded rapidly to oral corticosteroid therapy.
A previously healthy 45-year-old man presented to our outpatient clinic with a three-month history of an enlarging crusted plaque on the face that appeared impetiginized. The plaque was non-tender but pruritic. The patient denied medications and denied having applied any substance; his medical history was otherwise unremarkable.
Physical examination revealed a 7 x 4 cm well-circumscribed, crusted plaque of the left cheek surrounded by erythema (Fig. 1). The mucous membranes and the rest of the skin were unaffected.
A biopsy specimen was sent for histopathological examination (Fig. 2). Microscopic examination of the biopsy specimen revealed intraepidermal acantholysis supporting the diagnosis of pemphigus vulgaris. Direct immunofluorescence showed intense intercellular staining with immunoglobulin (IgG) and complement (C3) in the epidermis. Indirect immunofluorescent testing demonstrated IgG antibody in the patient's serum that also bound to the epidermal intercellular areas. The patient was lost to follow up for two months. He returned with extensive erosions and blistering of the trunk and extremities. The mucous membranes were spared. The patient subsequently underwent systemic corticosteroid therapy with prednisone at a dose of 1 mg/kg/day.
The skin lesions regressed rapidly. The glucocorticoid dosage was slowly decreased over several months and no recurrence of pemphigus was observed during the following six months.
Pemphigus is an autoimmune bullous disorder characterized by the appearance of cutaneous and mucosal vesicles and bullae. Histologically, keratinocyte acantholysis is the hallmark of the disease. Direct immunofluorescence detects deposits of autoantibodies in the intercellular areas. Indirect immunofluorescence detects circulating antibodies against surface antigens of the epidermal cells, found mainly to be the transmembrane glycoprotein, desmoglein 3, found within the desmosome. The trigger or mechanism of antibody development is unclear .
Pemphigus can be a difficult clinical diagnosis to make if there is no mucosal or generalized cutaneous involvement. Generally, pemphigus vulgaris manifests initially with mucosal lesions that may later be accompanied by cutaneous blisters and erosions . Approximately 60 percent of patients present with oral lesions as the initial manifestation of their disease . Initial presentation with localized cutaneous pemphigus is less common [4, 5]. When first seen, our patient exhibited a localized crusted plaque on the cheek that suggested bacterial infection.
The localized form of pemphigus vulgaris may remain limited to a single site for months before spreading . Typically, the time between onset of oral lesions and subsequent generalization of the disease is about five months .
Any area of the skin may be the initial site of involvement. However, the scalp, axilla, and face (as in our patient) are the most commonly affected sites . The differential diagnosis of the localized early lesions of pemphigus vulgaris, especially on the face, includes impetigo, actinic keratosis, and discoid lupus erythematosus [4, 8]. In endemic regions, like our country Tunisia, cutaneous leishmaniasis must also be considered in the differential diagnosis of a well-demarcated erythematous and crusted area of the face.
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