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PubMed · 6986830

Pemphigus: current concepts.

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Abstract

Pemphigus is an autoimmune intraepidermal bullous disease involving the skin and mucous membranes. There are four clinically recognized variants of this disease. The histopathologic hallmark is acantholysis, which is disruption of normal cell-to-cell adhesion. Patients produce an IgG antibody directed against an antigen present in the intercellular substance of the epidermis. The antibody binds to the intercellular spaces in vivo and can be frequently detected in patients' sera. Immunofluorescent examination of perilesional tissue and serum is an extremely valuable diagnostic technique. Recently, pemphigus-like lesions have been produced in skin explants grown in tissue culture media enriched with pemphigus serum. Before corticosteroid therapy was available, pemphigus was a fatal disease. Steroids, and more recently immunosuppressive agents, have drastically improved the prognosis.

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AcantholysisAdrenal Cortex HormonesAutoimmune DiseasesBinding Sites, AntibodyGoldHumansImmunoglobulin GImmunosuppressive AgentsLymphomaMyasthenia GravisPemphigusPenicillamineThymomaThymus NeoplasmsJournal ArticleResearch Support, U.S. Gov't, P.H.S.Review

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1980. Pemphigus: current concepts.. https://doi.org/10.7326/0003-4819-92-3-396

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PubMed1997

Gaining more insight into the pathomechanisms of thiol-induced acantholysis.

Acantholysis is considered the initial and the main pathogenetic event of pemphigus. The first step in drug-induced acantholysis (biochemical and/or immunological) involves binding of the drug to the cell membrane and the formation of 'drug-cysteine' instead of 'cysteine-cysteine' bondings. We suggest that the reaction of D-penicillamine with cystine disulfides that results in cysteine-penicillamine disulfides is not a terminal reaction, but rather a primary initiating step of a chain reaction. It is reasonable to consider that the cysteine-penicillamine disulfide is continuing to be enzymatically reduced by various thiol reductants, in particular glutathione reductase, thereby generating a 'new' penicillamine molecule which, in turn, reacts with other cystine disulfides and does so in an unending cycle. A chain reaction is thus created in which the drug is repeatedly generated so that one molecule of the drug may attack thousands of cystine disulfide bonds. It is highly possible that normal individuals have their own endogenous means of controlling this deleterious chain reaction, whereas pemphigus-prone individuals lack the ability to stop this potentially damaging reaction. Drug-induced pemphigus should thus be added to the ever-growing list of adverse drug reactions related to pharmacogenetic disorders in drug metabolism.

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PubMed1997

Langerhans cells in the development of skin cancer: a qualitative and quantitative comparison of cell markers in normal, acanthotic and neoplastic ovine skin.

The distribution of Langerhans cells in normal, acanthotic and neoplastic ovine epithelium was examined using the enzyme marker Acetylcholinesterase (AchE) and monoclonal antibodies (MoAb) to CD1 (20.27) and MHC Class II (49.1 and 28.1) molecules. In normal skin, where Langerhans cells were regularly spaced within the basal layer, qualitative observations and direct pairwise testing showed that AChE was superior to the MoAb in detecting these cells. Significantly more (P < 0.01) dendritic cells were also detected with MoAb 49.1 than MoAb 20.27 or 28.1, suggesting differential expression of MHC Class II subsets and the presence of CD1- MHC Class II+ granule- dendritic cells in sheep analogous to indeterminate cells of man. In acanthotic skin, compared to normal skin, Langerhans cells were less numerous, irregular and more suprabasal in distribution and their morphology was occasionally swollen and indistinct. No difference was seen in the ability of AChE and MoAb in detecting Langerhans cells, however pairwise testing of markers did demonstrate that significantly more (P < 0.05) cells without dendritic processes were stained with MoAb 49.1 than with 20.27 or 28.1. In all squamous cell carcinomas examined dendritic cells that stained for AChE, CD1 or MHC Class II antigens were concentrated at the peripheral areas of neoplastic epithelium. Many dendritic cells were detected with MoAb to MHC Class II antigens, whereas CD1 and AChE positive dendritic cells were rare in tumor bearing tissue. The quantitative differences in the immunohistochemical staining of Langerhans cells between normal, acanthotic and neoplastic epithelium were consistent with ultrastructural studies. When compared with those of a newborn lamb, which had had very little exposure to antigens or ultraviolet radiation (UVR), the Langerhans cells of the aged sheep were deformed and contained far fewer Birbeck granules. The abnormalities were progressively more severe in acanthotic and neoplastic skin. These observed changes may have resulted from UVR induced damage and may be indicative of impaired function involved in the development of skin cancer.

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