Bullous pemphigoid and psoriasis vulgaris
Published Web Location
https://doi.org/10.5070/D37g85p6rfMain Content
Bullous pemphigoid and psoriasis vulgaris
Patrick E Burnett MD PhD
Dermatology Online Journal 9(4): 19
From the Ronald O. Perelman Department of Dermatology, New York University
Abstract
A 55-year-old man with psoriasis presented with erythematous, edematous plaques, tense bullae, and erosions. Direct immunofluorescence and histopathologic examination confirmed the diagnosis of bullous pemphigoid. Both dermatologic conditions responded to azathioprine. The clinical presentation, etiology, and treatment options are reviewed.
Clinical summary
History.—A 55-year-old man has a 3-year history of a dermatosis involving the trunk and extremities. He presented in June 1999 to the dermatology service of the Department of Veterans Affairs New York Harbor Health Care System, where he was already under treatment for psoriasis, with a 1-week history of circumscribed, pruritic, red, blistering plaques on his hands and feet. The patient gave a history of recently working with cement and various solvents, which in the past had given him similar, pruritic, red blisters on his hands. A biopsy was performed, and the patient was treated with betamethasone dipropionate 0.1 percent cream. Subsequently he was treated with oral prednisone and azathioprine. In the past 3 years his disease has had periods of relative quiescence alternating with severe flares. A biopsy was performed in January 2002 from a well-demarcated, erythematous plaque with thick but loose adherent scale with adjacent tense bullae. Both dermatologic conditions have subsequently responded to azathioprine.
Physical examination.—Scattered on the trunk, and proximal extremities were well-demarcated, erythematous plaques with thick, loose, adherent scale. In a similar distribution were tense bullae and round erosions with an erythematous, edematous bases. Lateral pressure did not extend the bullae. No mucosal membrane lesions were noted.
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Laboratory data.—A comprehensive metabolic panel and complete blood count were normal.
Histopathology.—There is a subepidermal blister with a detached overlying epidermis and a superficial, perivascular infiltrate predominantly of eosinophils and neutrophils. There are numerous eosinophils within the blister cavity. The adjacent epidermis is irregularly hyperplastic with prominent hypergranulosis and compact orthokeratosis.
Diagnosis.—Bullous pemphigoid and psoriasis vulgaris.
Comment
Bullous pemphigoid is characterized by tense, subepidermal bullae with a predilection for the groin, axillae, and flexor surfaces. The autoimmune nature of this disorder has been confirmed with the identification of IgG antibodies to bullous pemphigoid antigen 1, a 230-kd protein and bullous pemphigoid antigen 2, a 180-kd molecular weight transmembrane protein, both of which are localized to the hemidesmosome. These antigens account for 90 percent of patients with bullous pemphigoid.
Reports of patients with both psoriasis and a blistering disease have been published; however, bullous pemphigoid is the most common association [1]. A predilection for blistering diseases in patients with a history of psoriasis has not been studied beyond case reports. Bullous pemphigoid does have an increased prevalence in patients with diabetes mellitus, rheumatoid arthritis, dermatomyositis, ulcerative colitis, and myasthenia gravis; no association with malignant conditions has been noted.
Treatment of bullous pemphigoid consists of immune suppression. For localized or even extensive disease, topical glucocorticoids alone may be sufficient [2]. Azathioprine, methotrexate, IVIg, and mycophenolate mofetil have been demonstrated to be effective [3, 4]. Two independent cases of psoriasis associated with bullous pemphigoid have been successfully treated with azathioprine and cyclosporine [5, 6].
References
1. Tomasini D, et al. Development of pemphigus foliaceus in a patient with psoriasis: A simple coincidence? Eur J Dermatol 1998;8:56.2. Joly P, et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002;346:321.
3. Ahmed AR. Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immunosuppressive treatment. J Am Acad Dermatol 2001;45:825.
4. Grundmann-Kollmann M, et al. Mycophenolate mofetil: A new therapeutic option in the treatment of blistering autoimmune diseases. J Am Acad Dermatol 1999; 40:957.
5. Primka EJ III, Camisa C. Psoriasis and bullous pemphigoid treated with azathioprine. J Am Acad Dermatol 1998;39:121.
6. Boixeda JP, et al. Bullous pemphigoid and psoriasis: Treatment with cyclosporine. J Am Acad Dermatol 1991;24:152.
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