Multiple crusted plaques in a woman with systemic lupus erythematosus. Pemphigus erythematosus (PE).
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A case that exhibits the pathologic features of cutaneous lupus erythematosus and the immunopathologic characteristics of pemphigus erythematosus following myasthenia gravis and thymoma is presented, and the literature pertaining to these disease associations is reviewed. The findings in this case were analyzed with respect to the accepted diagnostic criteria for lupus erythematosus, pemphigus, and pemphigus erythematosus. The pathogenic mechanisms that lead to acantholysis in pemphigus are examined in an attempt to explain why acantholysis did not occur in this case.
Pemphigus erythematosus occurred in a patient with rheumatoid arthritis who was treated with D-penicillamine. The skin lesions appeared 4 months after the onset of D-penicillamine treatment and persisted 14 years after cessation of this drug. Topical betamethasone dipropionate applications resulted in complete regression of the cutaneous lesions.
A 34-year-old woman was admitted to our hospital because of ptosis, dysarthria, muscle weakness of upper limbs and skin lesions. At the age of 22 years, she was diagnosed as having systemic lupus erythematosus (SLE) due to the presence of arthritis and high titer of antinuclear antibody. On admission, the high antiacetylcholine receptor antibody titer, along with the positive tensilon test and electromyography established a diagnosis of myasthenia gravis (MG). The demonstration of anti-intercellular antibodies both in cutaneous tissue and blood confirmed the diagnosis of pemphigus. MRI showed hypertrophic thymus. After thymectomy, the myasthenic symptoms aggravated and SLE and pemphigus erythematosus relapsed despite anti-cholinesterase treatment with plasmapheresis. She was then placed on corticosteroid therapy with an improvement of her all symptoms. This very rare case of MG associated with SLE and pemphigus erythematosus suggests that these diseases share common immunological abnormalities.
The expression of the pemphigus foliaceus (PF), pemphigus erythematosus (PE), and pemphigus vulgaris (PV) antigens in 16 different regions of normal human skin was evaluated by indirect immunofluorescence by using sera with a high titer of PF, PE, and PV antibodies. Regional variations were observed in the expression of all these antigens. The expression of the PF and PE antigens, as measured by endpoint titer of antibody reactivity, was highest in skin specimens obtained from the upper torso, and lowest in those from the buccal mucosa, lower torso, and scalp. This distribution pattern differed from that of PV antigen, whose expression was highest in buccal mucosa and scalp. These patterns correlate with, and may provide a partial explanation for, the different distribution of skin lesions in these different forms of pemphigus.
Topical 0.1% tacrolimus was used for treatment of localized lesions associated with 10 cases of discoid lupus erythematosus (DLE) and two cases of pemphigus erythematosus (PE) either as a sole therapy (n=2) or as an adjunctive treatment (n=10). Eight of 10 dogs with DLE and both dogs with PE were improved following 8 weeks of topical application. In six of the eight dogs that improved, other medications were discontinued. No adverse effects in clinical or laboratory parameters were noted throughout the study.
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An association between pemphigus erythematosus and systemic lupus erythematosus is well recognized. However, most reports strongly imply that the clinical manifestations of lupus erythematosus are usually minimal or only serologically significant. We describe the case of a 27-year-old black woman who experienced life-threatening systemic lupus erythematosus while recovering from pemphigus erythematosus. Unique histopathologic findings show the presence of both disorders in the same lesion.
An 82-year-old female with pemphigus erythematosus had the head of femur replaced. In her case, systemic lupus erythematosus and myasthenia gravis, typical complications of pemphigus erythematosus, were not present. As even slight friction easily causes blisters in patients with pemphigus, we paid attention mainly to fragility of the skin and the mucus membranes and were careful to minimize physical stimulation of the skin. Because the mucosa of the pharynx or oral cavity could easily be damaged and form blisters during intubation, which in turn might make the management of the upper airway more difficult, we selected spinal anesthesia. No serious perioperative complications, except a few blisters around the wound were observed. We thus successfully managed anesthesia of a patient with pemphigus erythematosa.
We present a 36-year-old woman with pemphigus erythematosus that showed histopathologically supra-basilar acantholysis and lichenoid tissue reaction. To our knowledge this is the first case of pemphigus erythematosus with such unusual histopathologic presentation to be reported.
Four patients with pemphigus erythematosus, three male and one female, are presented and the literature is reviewed. One of the patients had penicillamine-induced pemphigus erythematosus. The mean age of onset was 66 years. Direct immunofluorescent examination of perilesional skin demonstrated immunoglobulin and/or complement deposition in both the intercellular cement substance (ICS) of the epidermis and at the dermoepidermal junction (DEJ) in all four patients. The lupus band test was positive in three of the four patients when biopsies from uninvolved skin were studied. Indirect immunofluorescent examination of the sera for anti-ICS antibodies was positive in all patients. All four patients had positive antinuclear antibody tests (ANA), but anti-deoxyribonucleic acid (DNA) and anti-extractable nuclear antigens (ENA) were negative. One patient had a positive rheumatoid factor. Serum creatinine and total complement were normal in all four patients. The patients were treated with various combinations of oral corticosteroids, topical corticosteroids, dapsone, and oral therapy, which consisted of hydrogen peroxide, benadryl elixir, and decadron elixir. Three patients were in complete remissions and the fourth patient was in incomplete remission after 4 to 10 months of follow-up (mean, 6 months). Patients with pemphigus erythematosus require significantly lower doses of systemic corticosteroids for control of disease. Dapsone is an important agent for adjuvant therapy and facilitates use of lower doses of systemic corticosteroids.
A 5-year-old girl had pemphigus erythematosus based on clinical and immunohistologic criteria. The rare occurrence in childhood of the pemphigus group of disorders, and of pemphigus erythematosus in particular, is discussed. We also review current knowledge concerning the delineation of the different pemphigus subsets and the treatment of pemphigus erythematosus.
A patient affected by rheumatoid arthritis developed pemphigus erythematosus after 14 months of treatment with thiopronine. The lesions healed spontaneously after 4 weeks' withdrawal of the drug. Whereas the pemphigus erythematosus induced by D-penicillamine is a quite rare but well documented side effect, we believe our case to be the first one reported as being due to thiopronine. It is very interesting to note that the two drugs are very similar with regard to chemical structure, mechanism of action, therapeutic indications and also side effects.
Desmoplakin I and II are important components of the attachment plaque of the desmosome which mediates cell to cell adhesion, in epithelial cells. In this study we used well-characterized antibody against desmoplakin I and II immunohistochemically and immunoelectron microscopically on two cases of pemphigus vulgaris and one case of pemphigus erythematosus and two cases each of Hailey-Hailey's disease and Darier's disease. In the normal human epidermis the desmosomes were demonstrated in a dotted pattern along cell periphery. In pemphigus vulgaris and pemphigus erythematosus acantholytic cells and the perilesional cells exhibited normal dotted pattern along the cell periphery. In Hailey-Hailey's disease and Darier's disease, the dotted pattern is lost in acantholysed and perilesional areas and anti-desmoplakin I + II positive proteins were observed diffusely in the cytoplasm. Immunoelectron microscopical findings correspond to these light microscopical observations. It is concluded that in autoimmune acantholytic disease such as pemphigus vulgaris and pemphigus erythematosus, desmoplakins are intact even in acantholytic cells, whereas in genodermatoses such as vulgaris and pemphigus erythematosus, desmoplakins are intact even in acantholytic cells, whereas in genodermatoses such as Hailey-Hailey's disease and Darier's disease primary or secondary abnormalities abnormalities of desmosomes may be involved in their pathogenesis.
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