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Thiopronine, a D-penicillamine-like agent, is being prescribed increasingly for RA. Among side effects, only benign skin reactions have been mentioned to date. A single case of pemphigus erythematosus induced by this drug was recently reported. We describe here 3 other patients who developed pemphigus vulgaris during long-term thiopronine therapy and who improved after the drug was stopped.
A simple technique using immunoperoxidase-labelled antibodies is reported for the ultrastructural localization of fixed immunoglobulins in tissue lesions. This technique allows the immunolabeling of frozen sections of 15 mu mounted on glass slides. These sections are then closed over for ultrastructural examination, using an Epon-filled gelatin capsule, and then returned to the glass slide. After polymerization, the capsule containing the tissue section is separated from the glass by immersion in liquid nitrogen. This technique is applied to the study of fixed Ig in the skin lesions of lupus erythematosus, pemphigus, bullous pemphigoid and dermatitis herpetiformis. The results obtained show a fine localization of fixed Ig, good tissue conservation and easy orientation of the biological material. It can be applied to the study of tissue antigens.
BACKGROUND: This is the first epidemiologic study of pemphigus in the southern region of Saudi Arabia. METHODS: Data were collected from patient records in the outpatient and inpatient departments of Asir Central Hospital during the period 1990-99. The diagnosis of pemphigus was based on the clinical, histopathologic, and direct immunofluorescence patterns which were consistent with pemphigus. RESULTS: The results showed that the mean incidence of the disease in the general population was 0.16/100 000; the mean incidence of the disease in the population above 20 years of age was 0.27/100 000. The prevalence of the disease at the end of the study period was 1.56/100 000 for the total population, and 2.7/100 000 for the population above 20 years of age. The male to female ratio was 2.2 : 1. The age of onset of the disease ranged from 23 to 67 years, except for one case at an age of 9 years, giving an average of 43.1 +/- 13.4 years and a median of 40 years. Pemphigus vulgaris was the most commonly encountered type, followed by pemphigus erythematosus (94.7% and 5.3%, respectively). Mucosal involvement occurred in 84.2% of cases. No association with neoplasia was found in the current study. CONCLUSIONS: Pemphigus in the southern region of Saudi Arabia is as common as in other parts of the world, but with higher mucosal involvement.
Two sisters developed pemphigus vulgaris and pemphigus erythematosus within 3 years. The diagnosis was confirmed by clinical, histologic and immunofluorescent antibody studies. One of the sisters experienced a common cold before the pemphigus developed and displayed a positive macrophage migration inhibition (MIF) test to a combination drug compounded of paracetamol, caffeine, chlorpheniramine maleate and phenylephrine HCl, which she had received 2 weeks prior to the appearance of the cutaneous lesions. It is suggested that her pemphigus was triggered by the drug. Although the patient had a strong genetic and familial predisposition to pemphigus, her clinical symptoms did not become evident until they were activated through an exogenous factor, namely, the causative drug. This case offers an example of a possible interaction between endogenous, genetic factors, and exogenous, triggering factors in the development of full-blown disease.
A case of pemphigus foliaceus which initially developed at sites of preexisting psoriasis and a case of pemphigus erythematosus that developed on the lesions of photosensitive erythemas are described. Inflammatory dermatoses can be considered to be local factors which facilitate the autoimmune response of pemphigus, which in turn may induce blistering cutaneous lesions in subclinical patients with autoantibodies.
BACKGROUND AND DESIGN: Immunofluorescence detection of stippled IgA in dermal papillae has been considered the gold standard in the diagnosis of dermatitis herpetiformis (DH). We have developed an immunohistochemical technique using the avidin-biotin-peroxidase complex that is equally effective as direct immunofluorescence in detecting IgA. We retrospectively studied 43 paraffin-embedded biopsy specimens obtained from patients with DH and a variety of other diseases for the presence of IgA along the basement membrane zone. RESULTS: Eleven immunofluorescence-proved cases of DH were found positive for IgA with the avidin-biotin-peroxidase method. One biopsy specimen originally classified as DH was identified and reclassified as linear IgA bullous disease based on the immunoperoxidase findings. All the samples that were positive on direct immunofluorescence were positive with the avidin-biotin-peroxidase method. Control samples of bullous pemphigoid, discoid lupus erythematosus, pemphigus vulgaris, and dermatitis were all negative for IgA deposition. CONCLUSION: The diagnosis of DH on formalin-fixed tissue is possible with the use of an avidin-biotin-peroxidase method, which is convenient and cost-effective.
A review of canine pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus, and bullous pemphigoid shows them to share many clinicopathologic similarities with their human counterparts. Because of their numerous analogies in clinical presentations, histopathologic and immunopathologic findings, and therapeutic results, canine and human pemphigus and pemphigoid may represent a valuable area for comparative dermatologic research.
The association of pemphigus and thymoma has been investigated in a recent personally observed case and data from nineteen further cases published in the literature have been reviewed. From these twenty cases several prominent features emerge. Clinically, pemphigus of Senear-Usher type (pemphigus erythematosus) is most commonly found (11/20 cases). There is a male preponderance (at least 13/20 cases) although associated myasthenia gravis which occurred in thirteen cases was more common in females. Thymoma probably preceded pemphigus in all cases, although this was certain in only nine. Evidence of autoimmunity was marked; in addition to antibodies to intercellular substance, anti-striated muscle antibodies were frequent and antinuclear factor was positive in ten out of twelve cases. The prognosis was usually good although thymectomy was generally ineffective or occasionally seemed to have an adverse effect.
Biopsies from the oral mucosa of three patients (two with pemphigus vulgaris and one with pemphigus erythematosus) were taken for both freeze-fracturing and thin-sectioning studies. Oral mucosa from a clinically healthy volunteer was used as control. The samples collected from both the involved and uninvolved oral mucosa of pemphigus patients showed widened intercellular spaces and fewer than normal desmosomes. Thin sections showed a random distribution of tonofilaments. However, in freeze-fracture replicas tonofilaments were arranged in thick bundles with a slightly coiled orientation. In freeze-fracture replicas of samples from a pemphigus patient in remission no difference was found in the number of particles on both fracture faces.
The observation of pemphigus foliaceus out of endemic zone in a six-year-old child is reported. The rate of anti-ICS antibodies is very high (1/6,400). The direct immunofluorescence shows the common aspect of epidermic network between the cells. Fluorescence basement membrane has been found with monospecific anti-C3 sera, and after remission with the monospecific anti-IgG sera. The clinical, histological and immunological relationship between pemphigus foliaceus in its sporadic and endemic forms and pemphigus erythematosus are discussed. The clinical and immunological regression is obtained by association of prednisone and immunodepressive therapy. After two years of treatment the maintenance dosis is not yet reached. A review of ten previous reported cases of pemphigus foliaceus in children in its sporadic form is presented.
The association of HLA antigens with pemphigus in Jewish patients of various ethnic origins was investigated. Of 45 Ashkenazi patients, 31 (68.6%) had A26 (p less than 10(-5) and 32 (7175) had BW38 ( less than 10(-5)0. Among the 11 non-Ashkenazi patients, 7 (63.6%) had A26 and only 1 had BW38. The question whether the antigen A26 or BW38 is linked with the disease is discussed. Our findings may suggest that the primary association of pemphigus is with A26. This association may be stronger in Jewish male patients than in females. In view of the recent literature and our results it may be presumed that pemphigus in Jews is associated with antigens of Loci A and DR. No differences between pemphigus vulgaris and pemphigus erythematosus were observed.
In examination of the ultrastructure of excised tissue and other material from patients with various diseases, principally dermatovenerological affections and with malignant tumors, and in healthy control subjects, patterns suggesting mycoplasma were repeatedly found in certain diseases only. Specific attempts to isolate mycoplasma from the material were made, in which previously patterns of a mycoplasma type had frequently been seen. It was possible to culture mycoplasma-like organisms in vitro from patients with the following diseases: malignant melanoma, mycosis fungoides, prickle cell carcinoma, squamous cell carcinoma, non-specific urethritis, pemphigus erythematosus, panarteritis nodosa and vasculitis allergica cutis.
The development of pemphigus foliaceus in three patients and pemphigus erythematosus in one patient with rheumatoid arthritis treated with penicillamine is described.
This report describes the immunofluorescence (IF) findings in sequential skin biopsies and serum obtained during a follow-up study of a patient who developed pemphigus lesions after D-penicillamine treatment of arthritis. The distribution in the skin of IgG, IgM and C3 varied with the date, type and site of the biopsy sample. Thus IF patterns showed features of (a) pemphigus vulgaris, (b) pemphigus foliaceus and (c) pemphigus erythematosus. Indirect IF examination indicated the presence of circulating intercellular epithelial antibody of both the IgG and IgM class.
The behavior of the complement and of its components C4 and C3 has been studied in serum and in blister fluid both in patients with pemphigus vulgaris and other bullous dermatoses and in healthy subjects with experimentally induced blisters. The results are suggestive of local activation of the complement, in the blister fluid of patients with pemphigus vulgaris, pemphigus erythematosus and bullous pemphigoid according to the classical enzymatic sequence.
BACKGROUND: Despite the high incidence of pemphigus in the Jewish population, data on the epidemiology and etiology of the disease in Israel are sparse. OBJECTIVE: This study was conducted to identify clinical and epidemiologic features of pemphigus patients in Israel, while searching for risk factors that induce or exacerbate the disease. METHODS: Demographic and clinical information was recorded from the charts of 55 pemphigus patients treated over a 5 year period. A sample of 22 patients was compared to 22 age and gender-matched controls by means of a questionnaire querying details on lifestyle, including occupation, diet, sun exposure, and smoking. RESULTS: The findings show that the typical Israeli pemphigus patient is middle-aged, married, and of East European or Middle Eastern origin. The most common diagnosed clinical variant was pemphigus vulgaris, followed by pemphigus erythematosus. Some 70% of patients were treated with two or more immunosuppressive drugs and 62% entered long-lasting remission. Twenty-three percent of patients were exposed through their work to chemical substances, mainly pesticides, at the beginning of the disease and 18% of patients were continually exposed to ultraviolet radiation 5 years prior to onset of the disease. CONCLUSIONS: There is a possible correlation between occupational exposure to pesticides and UV radiation, and pemphigus induction.
We describe a case of the coexistence of psoriasis and pemphigus erythematosus. The coexistence of these two diseases may be more than coincidental: the effects of one immune-mediated skin disease may influence the occurrence of another. Possible mechanisms include antigen modification, altered regulation of T-cell activity, and plasminogen activator abnormalities.
BACKGROUND: Direct immunofluorescence (DIF) is a necessary examination tool for the diagnosis of pemphigus. The suction-blister-method splits the skin at the lamina lucida and it is possible with a scalpel to separate the entire epidermis from the dermis. OBJECTIVE: The study was to determine whether DIF is reliable on epidermal sheets separated using a suction apparatus. METHODS: Thirteen patients were selected for this study: (nine with pemphigus vulgaris (PV), one with paraneoplastic pemphigus (PP), and three with pemphigus erythematosus (PE). Frozen epidermal sheets, separated from the dermis with a scalpel, were used as a substrate. Diagnosis with routine fluorescein isothiocyanate (FITC) antibodies was made. RESULTS: In all patients a pericellular deposition of IgG was evident and in eight of these patients a pericellular deposition of C3 was present. In two cases of PE and one of PP, the C3 deposits were also present in the lower part of basal keratinocytes. CONCLUSION: This diagnostic method without skin biopsy is easy to perform and, together with the histology and clinical aspects, could be a useful tool in the diagnosis of pemphigus. We recommend this method when the patient is allergic to local anaesthetics, the patient easily produces hypertrophic scars, or in follow-up of already biopsied patients.