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PubMed2000

Immunoablative high-dose cyclophosphamide without stem cell rescue in a patient with pemphigus vulgaris.

The use of ablative intravenous cyclophosphamide (50 mg/kg per day for 4 days) without stem cell rescue has been described in patients with refractory autoimmune diseases such as paraneoplastic pemphigus, systemic lupus erythematosus, and aplastic anemia. We describe a 33-year-old patient with pemphigus vulgaris recalcitrant to multiple therapies. The patient presented with numerous oral ulcerations, erosions, and hyperpigmented crusted plaques on his face, trunk, and arms. Findings of a skin biopsy and direct immunofluorescence were consistent with pemphigus vulgaris. The circulating pemphigus vulgaris autoantibodies were present at a titer of 1:640. The patient received immunoablative therapy (50 mg/kg of cyclophosphamide for a total of 4 days) and tolerated the regimen well. Complications such as thrombocytopenia and Pseudomonas septicemia were quickly treated. Four months after the 4-day therapy, his oral and skin lesions completely healed, and his pemphigus titers have decreased to zero. He is no longer receiving prednisone and no new lesions have developed. This provides further evidence that this regimen is relatively safe and provides a potential "cure" for refractory autoimmune diseases such as pemphigus vulgaris.

Adult↗
PubMed1975

Cutaneous immunohistochemistry. The direct immunoperioxidase and immunoglobulin-enzyme bridge methods compared with the immunofluorescence method in dermatology.

Two immunohistochemical methods, using the enzyme horseradish peroxidase, the direct immunoperoxidase (DIP) and the immunoglobulin-enzyme bridge (IEB) method were applied on 129 skin specimens of 81 patients with lupus erythematosus, bullous pemphigoid, pemphigus vulgaris and rosacea. These methods were compared with each other and with the immunofluorescence (IF) method. The DIP method was preferred to the IEB method because of the greater contrast between the specific staining and the nonspecific staining of the former. The results obtained with both peroxidase methods were comparable with those of the IF method.

Fluorescent Antibody Technique↗
PubMed1988

[Immunohistological studies of immune dermatoses on reconstituted paraffin sections. Value and comparison of different methods].

In a study on 26 patients with autoimmune diseases the immunostaining for immunoglobulins and complement in frozen sections was compared with that in formalin-fixed and paraffin-embedded sections. The formalin-fixed material of pemphigus vulgaris, lupus erythematosus, bullous pemphigoid, and dermatitis herpetiformis (Duhring's disease) was reconstituted and stained by means of the standard two-step technique (TST), the peroxidase-antiperoxidase technique (PAP), and the streptavidin-biotin method (SAB). In comparison with frozen sections, immunoglobulins and complement could also be demonstrated in formalin-fixed sections in all cases of pemphigus vulgaris and in 85% of cases of discoid lupus erythematosus, but in only 60% of cases of bullous pemphigoid or Duhring's disease. PAP and SAB proved to be about equally reliable, but TST was significantly less dependable.

Dermatitis Herpetiformis↗
PubMed1996

Penicillamine-induced bullous dermatoses.

The successful therapeutic use of D-penicillamine (DPA) has been hindered by its many adverse effects. Autoimmune bullous syndromes are among the less common adverse DPA reactions; they are not dose dependent and appear late in the treatment of diseases of altered immunity, most often rheumatoid arthritis. The majority of the DPA-induced bullous syndromes belong to the pemphigus spectrum, usually pemphigus foliaceus or erythematosus, have a lower prevalence of demonstrable tissue-fixed or circulating antibodies than spontaneously occurring pemphigus, display abnormal direct immunofluorescent patterns, and have a generally favorable prognosis. However, many cases do exhibit a full-blown chronic disease, unaffected by DPA withdrawal. DPA-induced cicatricial pemphigoid is a severe disease of both mucous and cutaneous involvement with a prognosis similar to the spontaneous disease. Cases of DPA-induced epidermolysis bullosa acquisita and DPA-induced bullous pemphigoid were not sufficiently substantiated by immunofluorescence or immunoprecipitation criteria.

Drug Eruptions↗
PubMed1976

Studies on etiologic factors in pemphigus.

A survey of 234 cases of pemphigus yielded three observations which suggest that different forms of pemphigus may have different etiologies. 1. While the incidence of pemphigus in the age group of 21 and over was essentially the same in males and females (108 and 113) the incidence was significantly higher in females in the age group under 20. Only two of 13 patients in this group were males. Also pemphigus foliaceus and erythematosus was reported in a significantly higher proportion of the cases in the age group of 2 1/2 to 20 thus suggesting that the juvenile form of the disease differs from the adult form. 2. In four of a group of 15 cases identified as pemphigus foliaceus the disease appeared to be provoked by minor physical insults, a frequency which is significantly higher than the five cases with similar histories in the group of 198 cases identified as pemphigus vulgaris. 3. In the entire group of 234 cases two patients with pemphigus both proven by immunofluorescence had relatives with pemphigus. Also a third patient had a blood relative with bullous pemphigoid. This frequency of three per 234 is higher than would be expected by chance. Studies of one family (not included in this survey) with multiple skin antibodies suggest that such familial predispositions may be due to abnormal immune responses.

Adolescent↗
PubMed2001

Connectivity patterns in tuberculosis and leprosy patients are indistinguishable from that of healthy donors.

Connectivity, the self-defined interactions between antigen-recognising molecules in a network system can in part be assessed by measuring the reactivity of a given serum against an ordered set of immunoglobulin (Ig)G F(ab')2 fractions, separated by means of isoelectric focusing so that, the serum reactivity against the whole set of fractions defines a characteristic pattern of connectivity. Deviations from the normal condition (healthy donors) have so far been documented for two autoimmune diseases: systemic lupus erythematosus (SLE) and pemphigus vulgaris, as well as for human immunodeficiency virus (HIV)-1 infection. We tested here if bacterial infections lead to alterations in connectivity. In addition, we wanted to test if two antigenically related bacteria would produce similar or otherwise distinctive connectivity patterns. Connectivity analysis was applied on the sera from tuberculosis and leprosy patients and the sera from healthy donors were used as control. No statistically significant differences between the three groups studied were found. These results have implications for theories that set the origin of autoimmune diseases in microbial infections. To the best of our knowledge, this is the first attempt to analyze the connectivity status in bacterial infections.

Adult↗
PubMed1992

Expression of the alpha 6 beta 4 integrin in lesional skin differentiates bullous pemphigoid (BP) from epidermolysis bullosa acquisita (EBA).

The integrin alpha 6 beta 4 complex is a protein of the membrane of basal keratinocytes, localized at the surface of cells in contact with the basement membrane zone in normal skin. The expression of alpha 6 beta 4 was investigated in several autoimmune blistering skin diseases including bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), bullous systemic lupus erythematosus (BSLE), and pemphigus vulgaris (PV) by an indirect immunofluorescence technique. In lesional bullous skin of BP, alpha 6 beta 4 expression was either absent, or in some cases represented an unusual irregular patchy staining. In contrast, in lesional bullous skin from EBA, BSLE, and PV, alpha 6 beta 4 expression was comparable to that observed in normal skin, i.e., a linear staining of the BMZ. Thus, analysis of the alpha 6 beta 4 integrin reactivity on lesional skin, in conjunction with the typical localization of collagen IV, allows a rapid and accurate distinction between BP and EBA.

Diagnosis, Differential↗
PubMedDate not supplied

Upper aerodigestive tract manifestations of Behçet's disease: review of 30 cases.

Behçet's disease is a chronic inflammatory disorder of undetermined cause. The most frequent manifestations are aphthous ulceration of the upper aerodigestive tract, genital ulcers, ocular inflammation, and arthritis. Less frequent features include involvement of the CNS, thrombophlebitis, and large-vessel arteritis. Of utmost importance is the fact that aphthous ulceration of the upper aerodigestive tract will develop in all patients who have Behçet's disease; thus, they may come to the otorhinolaryngologist for evaluation. This disease must be distinguished from recurrent aphthous ulceration, herpes simplex infection, Stevens-Johnson syndrome, Reiter's disease, pemphigus, systemic lupus erythematosus, and Crohn's disease. Behçet's disease is diagnosed on the basis of clinical features; no specific laboratory abnormalities have been noted. The histopathologic perivasculitis is non-specific. Corticosteroid administration has been the most efficacious treatment. For serious complications, such as uveitis or meningoencephalitis, immunosuppressive agents can be used.

Adolescent↗
PubMed2004

Enhanced diagnostic immunofluorescence using biopsies transported in saline.

BACKGROUND: The demonstration of tissue-bound immunoreactants by direct immunofluorescence microscopy (DIF) is a valuable parameter in the diagnosis of various autoimmune and immunecomplex-mediated skin diseases. For preservation of tissue-bound immunoreactants, biopsies are usually fresh-frozen in liquid nitrogen or transported in Michel's fixative. But even optimally preserved tissue specimens are no guarantee for the correct diagnosis by DIF, especially when weak to moderate IgG fluorescence of the epidermal basement membrane zone is involved. In such cases false negative results are easily obtained due to the relatively high dermal "background" fluorescence produced by polyclonal anti-human IgG fluorescein conjugates. METHODS: In the present study we have compared the use of normal saline (0.9% NaCl) with liquid nitrogen and Michel's fixative as transport medium for skin biopsies. From 25 patients with an autoimmune skin disease (pemphigus, pemphigoid, lupus erythematosus and vasculitis) four matched skin biopsies were obtained and transported in either saline for 24 and 48 hours, liquid nitrogen, or Michel's fixative for 48 hours. RESULTS: Direct IF microscopy showed significant reduction of background fluorescence (p < 0.01) and relatively enhanced desired specific (IgG, IgA) staining in biopsies transported in saline. A conclusive or tentative IF diagnosis was reached in 92% after 24 h saline, 83% after 48 h saline, 68% after freezing in liquid nitrogen, and 62% after 48 h Michel's medium (n = 25). CONCLUSIONS: We conclude that transporting biopsies without freezing in normal saline for 24 hours is an adequate and attractive method for routine IF diagnosis in autoimmune and immune complex-mediated dermatoses. The superior results with saline incubation are explained by washing away of IgG background in dermis and epidermis.

Autoimmune Diseases↗
PubMed2003

Clinical characteristics and mycology of onychomycosis in autoimmune patients.

Onychomycosis is the most common nail disorder in adults. Predisposing factors are immunosuppression, poor peripheral circulation, diabetes mellitus, increasing age, nail trauma, and tinea pedis. Autoimmune patients, who carry many of these predisposing factors, have never been studied. Autoimmune patients, with underlying autoimmune skin diseases; pemphigus, systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), scleroderma, dermatomyositis and cutaneous vasculitis, as well as having abnormal-appearing nail(s) with suspicion of fungal nail infection were included. Clinical information was obtained. The causative organisms were identified by potassium hydroxide preparation and cultured. Duration of onychomycosis in autoimmune patients was twice longer than in non-autoimmune patients. Of those with mycological proven onychomycosis, the autoimmune patients had significantly more affected nails (p < 0.05; chi2, two-sided) compared to the non-autoimmune patients but there was no difference in the affected fingernails or toenails and clinical type of onychomycosis. Candida spp was the most frequently found in autoimmune subjects compared to dermatophytes, Trichophyton rubrum. However, dermatophytes especially Trichophyton rubrum was the most common causative organism in non-autoimmune samples, followed by Candida spp. The causative organisms were more frequently discovered in autoimmune patients, whether by potassium hydroxide (KOH) or culture, than in non-autoimmune patients (p < 0.05; chi2, two-sided).

Adult↗
PubMed1986

Molecular heterogeneity of the bullous pemphigoid antigens as detected by immunoblotting.

Sera from 28 patients with bullous pemphigoid (BP), four patients with cicatricial pemphigoid (CP), and 24 controls (normal volunteers and patients with pemphigus, systemic lupus erythematosus, or other skin diseases) were tested against extracts of human epidermis by immunoblotting techniques. The extraction buffer included 1% SDS, 5% beta-mercaptoethanol, and six protease inhibitors with various specificities. BP sera from individual patients showed different patterns of reactivity with the same epidermal extract, and each pattern consisted of one or more bands. A total of five bands of 240 kD, 200 kD, 180 kD, 97 kD, and 77 kD reacted with BP sera; the 240-kD band reacted with one CP sera, and none of these bands was detected by the control sera. The 240-kD and 180-kD bands reacted very strongly with some sera and were most frequently observed (43% and 29%, respectively). The 200-kD, 97-kD, and 77-kD bands were less frequently observed (25%, 7%, and 7%, respectively), but when present, their reactions were usually strong. Eleven percent of the BP sera did not react with any bands. Contrary to previous reports, this study shows that BP autoantibodies react with several protein bands, as detected by immunoblotting. We have recently shown by immunoelectron microscopy that BP autoantibodies bind to the basal cell hemidesmosomes. It remains to be determined which of these protein bands represent specific hemidesmosomal proteins and which antibody-antigen interactions are relevant to the pathogenesis of this disease.

Antigen-Antibody Reactions↗
PubMed1982

D-Penicillamine--induced pemphigus syndrome.

D-Penicillamine is a chelating agent which is effective in the treatment of Wilson's disease, cystinuria, and lead poisoning. In recent years, it has also been used to treat patients with rheumatoid arthritis with good results. The adverse effects of D-penicillamine are many. These include loss of taste, nephrotic syndrome, lupus erythematosus--like syndrome, polymyositis, dermatomyositis, myasthenia gravis, and agranulocytosis. Beginning in 1969, D-penicillamine was reported to induce a pemphigus eruption. We present a patient with D-penicillamine--induced pemphigus erythematosus and review previously reported cases.

Aged↗