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At least 19 recordsLinked to original sources
PubMed1991

IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis.

A patient with clinical features of herpetiform pemphigus of the foliaceus type had histologic findings consistent with pemphigus. Intercellular IgA deposits in a pattern like that of IgG in pemphigus were present. Circulating pemphigus-type IgA-class antibodies reacted first only with guinea pig and later in the disease also with monkey esophagus sections. IgG-class pemphigus antibodies were blocked by the IgA-class antibodies of this patient. In addition, the IgA-class pemphigus antibodies in this patient were blocked by the IgG-class pemphigus antibodies in tests on guinea pig and monkey esophagus. This indicates that the IgA-class antibodies in this patient were directed either to the same antigen as the IgG-class pemphigus foliaceus antibodies or to one that is close enough to it to give steric hindrance. The skin lesions responded poorly to systemic corticosteroid therapy. Dapsone therapy initially produced dramatic improvement, but the condition flared to the point that plasmapheresis, in addition to high doses of corticosteroids and cyclophosphamide, had to be used to control it.

Acantholysis↗
PubMed1978

Fate of pemphigus antibody following successful therapy. Preliminary evaluation of pemphigus antibody determinations to regulate therapy.

Six of 17 pemphigus patients (35%) treated during a six-year period with immunosuppressive agents and/or corticosteroids have had prolonged clinical and immunologic remissions off all therapy. All were treated until serum and tissue bound pemphigus antibodies could no longer be detected. The length of remission has ranged from 1 1/2 years to 4 years. Three of these six patients relapsed after being clinically and serologically free of pemphigus for 19, 20, and 48 months. Seven additional patients are clinically free of disease with insignificant pemphigus antibody titers of 10 or less. Therapy now is being discontinued gradually in these patients. This preliminary study demonstrates that (1) a large percentage of pemphigus patients may have a prolonged clinical and immunological remission after successful therapy; (2) maintenance therapy may not be required to preserve the remission; (3) monitoring serum and in vivo bound pemphigus antibody is of value in regulating therapy in pemphigus patients.

Adrenal Cortex Hormones↗
PubMed1992

Pemphigus vulgaris in siblings: HLA-DR4 and HLA-DQw3 and susceptibility to pemphigus.

BACKGROUND: The association of pemphigus vulgaris with the HLA serotypes, DR4 and DRw6, and with the DQ-beta chain alleles, DQw1 and DQw3, suggests that there is a genetic predisposition to this disease. However, familial cases of pemphigus vulgaris are exceedingly rare. OBJECTIVE: We studied two siblings with pemphigus vulgaris and three unaffected family members to determine whether an HLA allele was associated with the development of pemphigus vulgaris in this family. METHODS: We utilized restriction fragment length polymorphism methods using the HLA-DR beta 1, HLA-DQ alpha, and HLA-DW beta 1 genes as cDNA probes. RESULTS: We found that the affected siblings share the haplotype HLA-DR4 and the DQw.3.2 allele. CONCLUSION: Our findings of gene sharing among siblings with pemphigus vulgaris lend support to previous studies of unrelated Caucasian patients, which implicated DQw3 allele polymorphisms in conferring susceptibility to pemphigus.

Adult↗
PubMed1979

Appearance of "pemphigus acantholysis factor" in human skin cultured with pemphigus antibody.

These studies deal with the mechanism of pemphigus IgG-induced epidermal acantholysis. When normal human skin was culted with defined medium containing IgG from pemphigus serum, extensive epidermal acantholysis developed and heat-labile proteolytic enzyme(s) were recovered in the culture medium. The enzyme(s) displayed maximal activity at pH 6.5 when a 3H-amino acid-labeled, insoluble epidermal cell material was used as substrate. The enzyme activity increased during the first 3 days of culture and the appearance of maximal activity coincided with the time of onset of acantholysis. Acantholysis did not occur in control cultures incubated with normal IgG and the enzyme did not appear in the medium or in aqueous extracts of cultured tissues. The enzyme(s) is probably not of lysosomal origin because low pH-active proteases characteristic of these organelles remained within the cells. The effects of puromycin on appearance of enzyme activity, acantholysis and cell viability was studied. At cytotoxic concentrations, the appearance of the enzyme(s) and acantholysis were prevented, whereas at less toxic concentrations enzyme activity and acantholysis were not prevented. Because inhibition of protein synthetic rates by puromycin could not be dissociated from the cytotoxic effects, it is uncertain whether enzyme appearance and acantholysis were dependent upon living tissue or on specific protein synthesis. After pemphigus IgG was removed from the conditioned medium by DEAE cellulose and affinity column chromatography, the remaining material contained enzyme activity and caused acantholysis in fresh skin explants. Similar activities were not present in normal IgG-containing conditioned medium or unfractionated epidermal extracts from normal skin. These data indicate that when the pemphigus IgG autoantibody interacts with epidermal cell surface antigens, the cell responds by synthesis or activation of a non-IgG "pemphigus acantholysis factor" (PAF) which may be a nonlysosomal proteolytic enzyme. It is suggested that PAF causes loss of adhesion between keratinocytes and ultimately produces the characteristic acantholytic cells of pemphigus.

Acantholysis↗
PubMed1977

Characterization of cryoprecipitates in pemphigus: demonstration of pemphigus antibody activity in cryoprecipitates using the immunofluorescent technique.

Cryoproteins observed in patients with pemphigus have been shown to contain IgG, complement components, and other serum proteins. The IgG moiety possessed pemphigus antibody activity and contained kappa and lambda light chains. Cryoproteins appeared in the clinically active stage and disappeared subsequent to clinical improvement. These observations indicate that immune complexes may exist in the sera of patients with pemphigus.

Adult↗
PubMed1979

In vivo bound pemphigus antibodies in a stillborn infant. Passive intrauterine transfer of pemphigus vulgaris?

Pemphigus vulgaris developed in a 23-year-old woman during the first trimester of pregnancy. In the 33d week she had a stillbirth of a female child who showed extensive skin lesions clinically suggestive of pemphigus. The application of direct immunofluorescence staining to skin tissue sections showed deposition of IgG in the intercellular spaces of the epidermis of both mother and child. Our findings raise the possibility of transplacentar passage of pemphigus antibody, which may have been responsible for the fetal skin lesions.

Adult↗
PubMedDate not supplied

Levels of serum IgE in patients with Brazilian pemphigus foliaceus and pemphigus vulgaris.

The level of serum IgE in thirty patients with pemphigus (19 with Brazilian pemphigus foliaceus (BPF) and 11 with pemphigus vulgaris (PV)) were determined by means of a solid phase radioimmunoassay. A significant (P less than 0.01) increase in the level of IgE was observed in BPF patients compared to the level of IgE in PV patients and healthy adults. The possible relationship of an elevated level of IgE with respect to other aberrations of immunologic responsiveness and the suggested infectious etiology of BPF are considered.

Humans↗
PubMed1977

Organ culture studies of pemphigus antibodies. II. Ultrastructural comparison between acantholytic changes in vitro and human pemphigus lesions.

The ultrastructural and light microscopic features of acantholysis produced in organ culture were compared with those of human pemphigus lesions. In both, an intraepidermal split was seen an typical suprabasal acantholytic cells were present. These cells contained small bundles of tonofilaments, usually located away from the cell periphery. Desmonsomal plaques with inserted tonofilaments frequently remained along the periphery of acantholytic cells and along the upper portion of the periphery of basal cells. The ultrastructural similarity between in vitro and in vivo lesions provides additional evidence to suggest that organ cultures may provide a valid model for studying the dynamics of pemphigus lesion formation.

Aged↗
PubMed1975

Herpetiform pemphigus, a variable pattern of pemphigus.

A patient with the clinical features of dermatitis herpetiformis and the histological features of pemphigus, subcorneal dermatosis and dermatitis herpetiformis did not respond to treatment with pyridine or sulfones. Systemic steroids and azothioprine were effective. Immunofluorescence studies demonstrated circulating pemphigus antibodies and IgG bound in vivo in the intercellular spaces of the epidermis.

Abscess↗
PubMed1979

Association of pemphigus chronicus with pregnancy.

The evolution of pemphigus chronicus has been studied in 3 pregnant women. In the first patient, pemphigus vulgaris appeared 2 years prior to pregnancy. In the other 2 patients the disease began during pregnancy. Both women were treated with corticosteroids during their pregnancy which was normal as was delivery. A complete remission was seen in the first case 7 years following cortocosteroid therapy, and 8 months after therapy in the second case. Prolonged corticosteroid administration had no adverse effects on the fetus. The occurrence of pemphigus chronicus in the Republic of Macedonia during the past 15 years was only 0.14% of all patients referred to the Department of Dermatology. Female patients with this disease slightly outnumbered males. All forms of pemphigus seen in this period were diagnosed both clinically and histologically. The total number of cases was 53, of which pemphigus vulgaris was 74.4%, pemphigus vegetans 7%, pemphigus foliaceus 9.3% and pemphigus seborrhoicus 9.3%. We present our cases of pemphigus in pregnant women with regard to the fact that only few such cases have been published.

Adrenal Cortex Hormones↗
PubMed1978

Absence of intercellular antigens in the deep layers of the epidermis in pemphigus foliaceus.

12 patients with pemphigus foliaceus, a form of pemphigus with lesions that arise in the intercellular substance in the superficial layers of the epidermis, and 7 patients with pemphigus vulgaris, where lesions are in the deep layers, were studied by immunofluorescence. Circulating antibodies to intercellular antigens (IC antibodies) were found in 11 pemphigus foliaceus and 5 pemphigus vulgaris patients. On direct immunofluorescence of skin lesions 75% (9 of 12), pemphigus foliaceus patients had intercellular deposits of IgG localized solely or predominantly in the superficial epidermal layers, whereas this was not the case in any of the patients with pemphigus vulgaris. Over 70% of the pemphigus foliaceus patients with predominantly superficial IgG deposits lacked in their lesions normal intercellular antigens usually expressed in the deep layers of the epidermis. This was shown by the inability of IC antibodies in autologous or allogeneic sera to bind to intercellular antigens in the lower epidermis of patient's skin, even though the same sera could bind to intercellular antigens in all layers of normal allogeneic skin. Lack of normal intercellular antigens deep in the epidermis may result in circulating IC antibodies binding to the superficial layers, a site which corresponds to, and thus in some patients may account for, the anatomical location of lesions in pemphigus foliaceus.

Antibody Specificity↗
PubMed2026

When Neurodevelopment Meets Autoimmunity: Pemphigus Foliaceus in Rett Syndrome Expands the Clinical Spectrum-A Case Report.

Rett syndrome (RTT, OMIM 312750) is a complex multisystem neurodevelopmental disorder. Evidence suggests that RTT may have an autoimmune component and inflammatory activation. However, the autoimmune manifestations remain poorly described. Pemphigus foliaceus is a debilitating autoimmune blistering condition caused by IgG autoantibodies that target desmoglein-1 (Dsg1), resulting in widespread skin blistering and lesions. We report a case of pemphigus foliaceus in a 20-year-old female with RTT and discuss its clinical implications. Clinical data obtained from electronic health records were extracted and reviewed. Genetic testing was performed to identify the specific methyl-CpG-binding protein 2 (MECP2) mutation and on an expanded panel of 55 genes associated with pemphigus foliaceus and related blistering disorders. The individual had pemphigus foliaceus, which required immunosuppression, intravenous immunoglobulin (IVIg) therapy, and Rituximab. The disease trajectory was complicated by infections, aspiration pneumonia, and hypoxic cardiac arrest. There was progressive functional decline, and disease control was difficult to achieve, with frequent flares. Genetic testing confirmed a heterozygous pathogenic MECP2 variant (NM_001110792.1:c.952C>T; p.(Arg318Cys)). HLA genotyping identified alleles consistent with the HLA-DRB1*04:02-HLA-DQA1*03:01-HLA-DQB1*03:02 (DR4/DQ8) haplotype. Furthermore, genetic analysis identified a heterozygous DSG1 variant rs12967407. This study reports the first case of pemphigus foliaceus in RTT, expanding the clinical spectrum of RTT beyond its neurodevelopmental phenotype. The DR4/DQ8 haplotype, previously associated with pemphigus susceptibility, supports a background of genetic susceptibility in this individual. No causal association between RTT and pemphigus foliaceus can be inferred from this single case. Rather, this case demonstrates that a rare autoimmune disorder such as pemphigus foliaceus can co-occur with a pathogenic MECP2 mutation. The coexistence of a genetic and autoimmune disease can result in a more complex clinical presentation and treatment course. The case further emphasises the need for increased vigilance in identifying new and emerging systemic pathology alongside RTT.

Humans↗
PubMed1976

Production of epidermal acantholysis in normal human skin in vitro by the IgG fraction from pemphigus serum.

Normal human skin was maintained in organ cultures for several days in Ham's F-10 medium with good preservation of the epidermal cells. When the partially purified IgG fraction from the pooled sera of patients with pemphigus vulgaris or pemphigus foliaceous was added to this culture system, after 24 hr some evidence of epidermal acantholysis was seen. By 72 hr, extensive suprabasilar epidermal acantholysis had occurred in which the acantholytic cells were indistinguishable histologically from the acantholytic cells in biopsies from skin lesions of patients with pemphigus vulgaris. In the control cultures (i.e., F-10 medium or F-10 medium + normal human serum IgG), none of these changes was seen. Direct immunofluorescent staining of these explants using fluorescein-labeled goat antihuman IgG showed that by 6 hr binding of the pemphigus IgG had occurred in the intercellular cement substance of the epidermis. The staining intensity was maximal by 18 to 20 hr. When the pemphigus serum was fractionated by DEAE-cellulose column chromatography, three major IgG-containing peaks (presumably IgG) were eluted which bound to the epidermoid intercellular substance and caused acantholysis in culture. The complement system did not play a role in the antibody-induced acantholysis since complement was not included in this system and heating the reconstituted F-10 + pemphigus IgG for 1 hr at 58 degrees C did not destroy the acantholytic activity. Autoradiographic experiments showed that after about 2 days in culture the rates of incorporation of RNA and protein precursors in the suprabasilar cells in the presence of pemphigus IgG were reduced to less than 10% of the normal IgG controls, whereas these synthetic activities of the basal cells were only slightly affected. These observations lead to the proposal that it is the interaction of the pemphigus autoantibody(s) with the suprabasilar epidermal cell which initiates and possibly substains the process(es) of acantholysis.

Acanthocytes↗
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↗