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PubMed2003

A case of co-incident phenylketonuria, pemphigus foliaceus, and tinea amiantacea treated with tetracycline and nicotinamide.

I report a patient with phenylketonuria who presented with pemphigus foliaceus or tinea amiantacea. The rash resolved with treatment with tetracycline and niacinamide. This article outlines the uses and mechanisms of this therapy with particular attention to its use in pemphigus, treatments, and scalp findings of pemphigus foliaceus and the dermatological manifestations of PKU.

Adult↗

PubMed1997

[Brazilian pemphigus foliaceus (fogo selvagem)].

Most of the clinical, histological and immunohistological features of fogo selvagem resemble those of idiopathic pemphigus foliaceus (PF). Both diseases are clinically characterized by small flaccid bullae evolving into to scaly and crusted lesions, sometimes with pustules, mainly in seborrheic areas of the skin. Mucosal surfaces are mostly spared. The main histologic feature of endemic pemphigus foliaceus is a subcorneal acantholytic blister. Standard immunofluorescence studies demonstrate intercellular IgG deposits throughout the entire epidermis. These IgG antibodies are mainly of the IgG4-subclass. Almost all patients have circulating IgG-autoantibodies in their serum directed against stratified epithelial desmosomes. The fogo selvagem autoantibodies and the PF antibodies are directed against the 160 kD desmosomal glycoprotein desmoglein 1 which together with plakoglobin (85 kD) forms a complex of adhesion proteins with desmosomes of stratified epithelia. Fogo selvagem occurs in endemic foci in some areas of Brazil and possibly in neighbouring South American countries, very often in children, adolescents and young adults. The etiology of fogo selvagem is still unknown. The frequent association with insect bites has lead to the concept of fogo selvagem being a transmissible disease with acquired immunity in adulthood. However, the infectious agent and possible vectors have not yet been identified.

Adolescent↗

PubMed2001

Pemphigus foliaceus developing after metastasis of cutaneous squamous cell carcinoma to regional lymph nodes.

We describe a patient in whom pemphigus foliaceus developed after cutaneous squamous cell carcinoma (SCC) metastasized to regional lymph nodes. Immunologic analysis revealed that production of anti-desmoglein 1 autoantibodies started when SCC metastasized, and the SCC expressed desmoglein 1, suggesting a pathogenic role of metastasized SCC in developing pemphigus foliaceus.

Aged↗

PubMed1989

IgA pemphigus foliaceus. Report of two cases and a review of the literature.

The cases of two patients with vesiculobullous lesions were diagnosed clinically and histopathologically as pemphigus foliaceus; unexpectedly, both revealed intercellular IgA, but not IgG, in the upper epidermis by direct immunofluorescence. Such histologic and immunofluorescence findings have been reported in eight other cases. In our cases no circulating IgA or IgG intercellular antibodies could be detected; in four of eight other reported cases IgA antibodies showed intercellular staining like that of pemphigus antibodies. Subcorneal acantholytic lesions occurred in both our cases; of the other cases reported, five had essentially identical histopathologic findings. The clinical and histopathologic features of pemphigus, as well as the recent findings of circulating IgA intercellular antibodies alone or with IgG antibodies, appear to place this disease into the spectrum of pemphigus. The 10 IgA pemphigus cases reported to date fall into one of two groups, the IgA pemphigus foliaceus (including our two cases) and IgA pemphigus of the intraepidermal neutrophilic type, which seems to be less common.

Adult↗

PubMed2001

Pemphigus foliaceus with prominent neutrophilic pustules.

We describe four patients with generalized scaly and pustular skin lesions showing extensive neutrophilic infiltration in the subcorneal region of the epidermis. Immunofluorescence, immunoblot and enzyme-linked immunosorbent assay analyses detected IgG antibodies reacting exclusively with desmoglein 1, the pemphigus foliaceus antigen. This study indicates that pemphigus foliaceus may show prominent neutrophilic pustular skin lesions.

Adult↗

PubMed1979

[Detection of intercellularsubstance-autoantibodies after antigen-antibody-complex splitting in pemphigus foliaceus (author's transl)].

One patient suffering from pemphigus foliaceus exhibited the typical intercellular deposits of immunoglobulins in the subcorneal layers of the affected and not affected skin by direct be detected by means of several antigensubstrates. They could only be found after splitting of the antigen-antibodies-complexes. The laboratory findings exclude a more distinct deviation of the humoral and cellular immune reactivity.

Antigen-Antibody Complex↗

PubMed2004

Cytokine gene polymorphisms in endemic pemphigus foliaceus: a possible role for IL6 variants.

Endemic pemphigus foliaceus (EPF) is a complex autoimmune disease characterized by the presence of antibodies against desmoglein 1, which lead to the loss of adhesion among keratinocytes (acantholysis). Variants of HLA class II genes have been the only genetic factors found to modulate susceptibility to EPF. This study aims at investigating the influence of cytokine genetic variants in the pathogenesis of EPF, since they may affect the expression levels of these immunomodulatory molecules. The sample included 168 patients and 189 controls and was comprised of mostly Caucasoids and Mulattos. The approach consisted of a case-control association study and the alleles were identified by mismatched PCR-RFLP. No associations were found with variants of IL1A, IL1B, IL1RN, IL4R and IL10. There was a weak negative association with the haplotype -1082G -592C (OR=0.49) of the IL10 gene in Mulattos. In regard to polymorphism -590 of the IL4 gene, a positive association with the T/T genotype (OR=2.71) and a negative association with the C variant (OR=0.37) were found. Associations with IL6 -174 variants suggest that the C/C genotype has a protective effect (OR=0.13) while carriers of the G allele are more susceptible (OR=7.66) to EPF.

Black People↗

PubMed1996

Binding of autoantibodies is not restricted to desmosomes in pemphigus vulgaris: comparison of 14 cases of pemphigus vulgaris and 10 cases of pemphigus foliaceus studied by western immunoblot and immunoelectron microscopy.

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases characterized by a loss of cell-cell adhesion and by autoantibodies directed against epidermal cadherins. PF antigen has been established as desmoglein I which is located strictly on the desmosome, whereas the precise ultrastructural localization of PV antigen remains unclear and controversial to date. To further investigate this question, we compared the location of immune deposits in 14 patients with PV and 10 patients with PF by both direct and indirect immunoelectron microscopy (IEM). Inclusion criteria were based upon clinical features, histological level of cleavage and characterization of circulating antibodies by Western blot on epithelial bovine tongue extracts. IEM was performed on unfixed 0.7-mm slices of skin for the direct technique or on normal skin for the indirect technique using peroxidase labelling. In PF, by both direct and indirect IEM, immune deposits were located on the extracellular part of desmosomes (desmoglea) in all the samples studied. In PV, by both direct and indirect IEM, deposits were situated on the desmoglea and along large portions of the keratinocyte membrane without desmosomal structures in 15 of the 18 samples studied and only on the desmoglea in 3 samples. These results suggest that, in contrast to PF, the target antigen in PV is not always restricted to desmosomes. As various types of adherens junctions have been reported to mediate cell adhesion in the epidermis, the PV antigen could be a component of desmosomes and of other focal adhesions.

Adult↗

PubMed1982

[Myasthenia gravis and pemphigus foliaceus].

Myasthenia gravis associated with thymoma affecting a 76-year-old white woman is reported. Two years later, after an immunosuppressive treatment with prednisone and irradiation of the tumor, bullous lesions appeared on the skin sparing the mucosae. The histopathologic features and the direct and indirect immunofluorescence tests indicated the existence of pemphigus foliaceus. The association of Myasthenia Gravis with pemphigus foliaceus is very rare, and the present case is actually the third reported in the literature.

Aged↗

PubMed2001

A polymorphic variant of the gene coding desmoglein 1, the target autoantigen of pemphigus foliaceus, is associated with the disease.

Two polymorphic markers were identified on the desmoglein 1 gene which encodes the autoantigen targeted by pathogenic antibodies in pemphigus foliaceus (PF), a cutaneous autoimmune blistering disease. The first marker, made of a variant haplotype of five mis-sense mutations located on the part of the gene encoding the fourth and fifth extracellular domains of the protein, is not associated with the disease. The second marker consists of a single silent T to C transition at position 809 and was found to be significantly more frequent (P = 0.015) in Caucasian PF patients (n = 36) than in controls (n = 98). Thus, pemphigus foliaceus constitutes another example of autoimmune disease in which the autoantigen polymorphism contributes to disease susceptibility.

Amino Acid Sequence↗

PubMed1988

South American pemphigus foliaceus: study of an epidemic in El Bagre and Nechi, Colombia 1982 to 1986.

Between 1982 and 1986 we have had the chance to study 21 patients with pemphigus foliaceus of the 'fogo selvagem' type. The patients came from El Bagre and Nechi, rural areas of Colombia with some gold mining. This is the first outbreak of South American pemphigus foliaceus reported in Colombia. The majority of the patients were mestizo men, who worked as farmers or miners or both, with an average age of 44. Five patients were relatives. Eleven patients (52%) had mild disease, three (14%) moderate disease and seven (33%) severe disease. During hospitalization, three patients died due to complications or as a result of immunosuppressive treatment. Of the remaining 18 patients, 10 were in remission with treatment, one was in remission without treatment, while no information was available on the remaining seven.

Adult↗

PubMed2000

Pemphigus foliaceus induced by exposure to sunlight. Report of a case and analysis of photochallenge-induced lesions.

BACKGROUND: Although there are many reports on photo-induced pemphigus, careful analysis in the development of acantholytic blister have rarely been performed in the lesions induced by photochallenge. OBJECTIVE: The study was intended to elucidate the mechanisms by which ultraviolet light (UV) radiation causes the skin lesions of pemphigus foliaceus. METHODS: Photochallenge-induced lesions were examined histologically and immunohistochemically over the time course with three biopsy specimens. Neutrophil adhesion assay was performed using the specimens prepared from the photochallenged lesions as substrates. RESULTS: A small number of neutrophils were seen at 5 h after photochallenge in the dermis and epidermis, and slight acantholysis was detected at 72 h. In neutrophil adhesion assay, greater numbers of neutrophils adhered to the upper epidermis of the skin obtained at 5 h after challenge. CONCLUSIONS: Our results suggest that both enhanced binding of autoantibodies to the epidermis after UV radiation and preferential adhesion of neutrophils to the UV-irradiated epidermis contribute to the development of acantholysis in photo- induced pemphigus foliaceus. Intercellular adhesion molecule-1 (ICAM-1) expression on keratinocytes is not primarily responsible for the epidermal migration of neutrophils.

Cell Adhesion↗

PubMed2001

Diagnosing Pemphigus foliaceus: a retrospective analysis of clinical, histological and immunological criteria.

BACKGROUND: Clinical, histological and immunological criteria distinguish pemphigus foliaceus (PF) from pemphigus vulgaris (PV), but whether and how often they are concordant in the same patient is unknown. METHODS: Seven clinical records were selected from two hospital settings for having a diagnosis of PF and the initial serum and histopathological specimens still available. Controls were 8 PV records selected in the same way. Histopathological slides were re-evaluated. Stored sera were studied by indirect immunofluorescence (IIF), Western blot and ELISA. RESULTS: Acantholysis was superficial in all PF patients and deep in all PV patients. Mucosal lesions were not exclusive of PV. IIF was positive in 43% of PF patients. Western blot revealed desmoglein 1 in 86% of PF patients and in 25% of PV. ELISA revealed anti-desmoglein-1 antibodies in up to 71% of PF and in 62% of PV patients, in 1 failing to detect anti-desmoglein-3 antibodies. CONCLUSIONS: Histopathology remains the most reliable criterion for diagnosing PF. Western blot and ELISA, especially in combination, may be only of confirmatory value.

Acantholysis↗

PubMed1979

Pemphigus foliaceus in a patient with rheumatoid arthritis and Sjögren's syndrome. A case report.

A 74-year-old male patient with rheumatoid arthritis and Sjögren's syndrome developed pemphigus foliaceus. Clinically healthy skin tissue revealed a fluorescent band of IgM along the basal membrane and an intercellular deposit of C3 and IgG in the deepest areas of the epidermis. Similar investigations of diseased skin revealed no deposits of IgM, and C3 and IgG were spread over a large area of the epidermis, though following the same pattern. In sero-positive rheumatoid patients with and without vasculitis, C3 and IgM are frequently found in small dermal vessels immediately below the epidermis in apparently normal skin. The fluorescent band of IgM along the basal membrane is thought to be an expression of the patient's rheumatoid arthritis, in spite of the fact that this IgM site is not typically associated with rheumatoid arthritis. The change in the pattern of deposition is thought to be due to pemphigus foliaceus.

Aged↗

PubMed1999

The treatment of mild pemphigus vulgaris and pemphigus foliaceus with a topical corticosteroid.

Seven patients with mild pemphigus vulgaris (n = 3) or pemphigus foliaceus (n = 4) were treated with a very potent topical corticosteroid alone. Clobetasol propionate 0.05% cream was applied to mucosal lesions and involved skin twice a day for at least 15 days, then progressively tapered. Pemphigus was considered to be controlled if healing of lesions was obtained, with a 75% decrease in the number of new lesions per week without addition of any systemic treatment. In all seven patients, the disease was controlled initially with healing of cutaneous lesions within 15 days, while healing of mucosal lesions took at least 1 month. In four patients, remission was maintained with topical corticosteroid alone for a mean 19-month follow-up. In three patients, relapse occurred after 2-11 months, requiring a systemic treatment.

Administration, Topical↗

PubMed2006

Sensitivity of indirect immunofluorescence and ELISA in detecting intercellular antibodies in endemic pemphigus foliaceus (Fogo Selvagem).

BACKGROUND: Since 1967 dermatology has used the classic technique of indirect immunofluorescence (IFI) for the detection of autoantibodies against antigens of the skin in diseased people with endemic pemphigus foliaceus. Thirty years later enzyme-linked immunosorbent assays--ELISA (rDsg1 and rDsg3) appeared as a viable option. A group of highly recognized researchers have concluded that ELISA is a simple, sensitive and highly specific method, allowing for diagnostic differentiation between pemphigus vulgaris (PV) and endemic pemphigus foliaceus (EPF). Scientific literature certifies that both ELISA and IIF bear high sensitivity in spite of the fact that a direct comparison between the ELISA and IIF tests has never been performed. OBJECTIVES: This study was conducted to compare the sensitivity of these tests in detecting antibodies in the EPF. MATERIAL AND METHODS: Thirty-two serum samples were collected from patients with EPF. The control serum of 15 healthy individuals was tested to detect the presence of antibodies of EPF by indirect immunofluorescence and ELISA (rDsg1 and rDsg3). The IIF was performed, taking human skin as a substrate. RESULTS: Antibodies in patients with EPF were detected more commonly by the ELISA (rDsg1) (91%) compared with IIF (81%). CONCLUSIONS: The ELISA (rDsg1) is slightly more sensitive than IIF in detecting antibodies related to EPV. However, according to our results, we do not currently possess a test with 100% accuracy in differentiating EPF from PV. Although previous studies have associated Dsg3 with PV, the tests performed during this study showed that 12% (4/32) of patients with EPF (cutaneous diseases only) also had Dsg3 antibodies.

Autoantibodies↗

PubMed1993

Pemphigus foliaceus induced by nifedipine.

We report a case of pemphigus foliaceus caused by nifedipine. In this case casual rechallenge with nifedipine confirmed that this drug was the causative agent. The skin lesions cleared quickly after withdrawal of the drug and treatment with a short course of oral steroids. To our knowledge, this is the first reported patient with nifedipine-induced pemphigus.

Drug Eruptions↗

PubMed1986

Childhood pemphigus foliaceus: case report and review of the literature.

Childhood pemphigus foliaceus typically causes erythema and scaling of the scalp. Sometimes, blisters and oozing are present, which often are misdiagnosed as either impetigo or seborrheic dermatitis. The eruption may progress to involve the trunk and limbs, the lesions often having an arcuate pattern. The diagnosis can be established by histologic and immunologic evaluation. In children, the disease seems to be less severe than pemphigus vulgaris, as is the case with adults, and we recommend a trial of topical steroids before embarking on systemic therapy.

Administration, Topical↗