Published Web Locationhttps://doi.org/10.5070/D335v03510
From New York University Department of Dermatology
Elizabeth K. Hale
Dermatology Online Journal 8(2): 9
This 12 year-old girl has had an eruption involving the scalp, trunk, and extremities for six years. The patient first presented to the Charles C. Harris Skin and Cancer Pavilion in March, 1995, with a generalized pruritic eruption of flaccid bullae and crusted erosions. She was otherwise healthy. A biopsy was performed at that time, and a diagnosis was made based on clinical, histopathologic, and immunofluorescence data. Treatment has consisted of prednisone, dapsone, and topical glucocorticoid preparations.
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Hyperkeratotic papules and plaques, in addition to several crusted erosions, were concentrated on her distal extremities. The eruption was less generalized than it was on initial presentation six years ago. There are no intact blisters at this time, and there were no lesions of the oral mucosa.
Hemoglobin was 9.4 mg/dl, hematocrit 31.2%, and the mean corpuscular volume 66.3 fl. A comprehensive metabolic profile, including liver function tests, was normal.
There is an intraepidermal blister with acantholysis of the granular layer. A direct immunofluorescence test shows abnormal intercellular deposits of IgG and C3, which are predominantly in the upper layer of the epidermis. There were no abnormal deposits of IgG, C3, IgM, IgA, or fibrin in the basement-membrane zone or around blood vessels.
Pemphigus foliaceous is also known as superficial pemphigus because the intraepidermal separation occurs superficially in the granular layer. While sporadic pemphigus foliaceous most commonly affects middle-aged individuals and is rare in children, childhood pemphigus foliaceous is more common than pemphigus vulgaris of childhood and generally follows a less aggressive course than does the adult form of the disease. However, a distinct subtype of pemphigus foliaceous known as fogo selvagem is endemic in rural areas of Brazil and occurs most commonly in children and young adults.
The target epidermal antigen in pemphigus foliaceous is desmoglein 1, a 16-kD transmembrane desmosomal glycoprotein, which is a member of the cadherin supergene family. Anti-epidermal autoantibodies have been shown to be pathogenic in passive transfer experiments.
Pemphigus foliaceous is characterized by the presence of scaly, crusted blisters, which often overly an erythematous base. Small, flaccid blisters are often inconspicuous. Oral mucosal lesions are rarely seen. The disease may remain localized for many years; however, pemphigus foliaceous may progress to a severe, generalized, exfoliative dermatitis. Histologically, in addition to acantholysis in the granular layer, subcorneal pustules comprised of neutrophils and acantholytic cells are often present.
Localized pemphigus foliaceous may often be treated adequately with topical therapy. Systemic glucocorticoids remain the mainstay of therapy for more extensive disease although many alternate immunosuppressive steroid-sparing agents have been tried. Because of potential adverse effects, cytotoxic drugs should be used with special precaution in children. However, dapsone, gold, and intravenous corticosteroid pulse therapy followed by immunosuppressive agents (including methotrexate and azathioprine) have been used alone or as ancillary therapy for patients with refractory disease or when prolonged use of high-dose corticosteroids is undesirable.
ReferencesAhmed AR, Salm M. Juvenile pemphigus. J Am Acad Dermatol 8:799, 1983
Basset N, et al. Dapsone as initial treatment in superficial pemphigus: report of nine cases. Arch Dermatol 123:783, 1987
Kanwar AJ, Kaur S. Pemphigus in children. Int J Dermatol 30:343, 1992
Kanwar AJ, Dhar S. Further experience with pemphigus in children. Pediatr Dermatol 11:107, 1994
Lin MS, et al. The desmosome and hemidesmosome in cutaneous autoimmunity. Clin Exper Immunol 107:9, 1997
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