Pemphigus in Hartford County, Connecticut, from 1972 to 1977

Simon, D. G.

doi:10.1001/archderm.116.9.1035

Cited by 34 publications

(27 citation statements)

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“…1 The most common variant is pemphigus vulgaris. 2 Epidemiological studies from Israel 3 and the United States 4 suggest that Ashkenazi Jews have an increased risk for pemphigus, with annual incidence rates of 20 per 1million to 30 per 1 million, compared with other investigated populations such as those of Saudi Arabia 5 (2 per 1 million) and Bulgaria 6 (5 per 1 million).…”

Section: Discussionmentioning

confidence: 99%

“…The increased ORs seen with increasing exposure support this observation. Previous retrospective surveys on pemphigus have been limited to a single geographic region 4,5 or hospital ward. 21 The strengths of this study are its population-based design, the inclusion of case patients diagnosed and treated in the community, and the use of complete data capture of prescribed medications and diagnosis.…”

Section: Commentmentioning

confidence: 99%

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Pemphigus Variant Associated With Penicillin Use

Heymann¹,

Chodick²,

Kramer³

et al. 2007

Arch Dermatol

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Section: Discussionmentioning

confidence: 99%

Section: Commentmentioning

confidence: 99%

Pemphigus Variant Associated With Penicillin Use

Heymann¹,

Chodick²,

Kramer³

et al. 2007

Arch Dermatol

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“…Those with Jewish ancestry also seem to have a particularly high incidence. 2 All ages can be affected by pemphigus, though it is most common in middle age, and both sexes are affected equally. Several studies on the effect of smoking on pemphigus seem to suggest that, at least in some populations, it has a protective effect.…”

Section: Epidemiologymentioning

confidence: 99%

“…1 The most common primary sites in men are lung (24%), colon (19%) and oral squamous cell carcinoma (12%) and in women breast (69%), colon (19%) and ovary (4%). 2 Lesions appear as firm, red or blue nodules, often multiple and close to the site of the primary tumour. The scalp is a well documented site of metastasis for breast, lung and renal cancer.…”

Section: Cutaneous Metastasis/direct Tumour Spreadmentioning

confidence: 99%

Pemphigus: a brief review

Groves

2009

Clinical Medicine

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Pemphigus is an uncommon autoimmune blistering disease, mediated by antibodies directed against desmosomal adhesion proteins (most particularly desmogleins 1 and 3) that are responsible for maintaining integrity of the epidermis. This article will discuss its epidemiology, pathogenesis, clinical variants and approaches to treatment. EpidemiologyRobust epidemiologic data on pemphigus incidence are scarce but a recent study from the UK suggests an incidence of 0.68 cases per 100,000 person years. 1 Incidence varies in different parts of the world, being more common in the Near and Middle East than in Western Europe and North America. Those with Jewish ancestry also seem to have a particularly high incidence. 2 All ages can be affected by pemphigus, though it is most common in middle age, and both sexes are affected equally. Several studies on the effect of smoking on pemphigus seem to suggest that, at least in some populations, it has a protective effect. 3 A rare endemic form of pemphigus foliaceus (fogo selvagem) has been identified in parts of Brazil and in North Africa. 4 The cause remains to be identified, but cases seem to cluster in rural villages around rivers and resolve when patients move away from the endemic areas. It has been suggested that the disease may be transmitted by insects living in the area.Before the advent of systemic steroid therapy pemphigus was often a lifethreatening disease. Morbidity is now more often a result of therapy than related to the underlying disorder. Disease duration varies widely, though remission rarely occurs in less than three years. Some patients require ongoing therapy for over 30 years. GeneticsThe genetics of pemphigus are complex. 5,6 Familial cases are uncommon but there are strong associations with certain genes in the MHC complex on chromosome 6, some of which vary according to the population studied. Thus, in Jewish populations DRB1*0402 is highly overexpressed whereas this is less common in Western Europeans. Conversely, the DRB1*1401 allele is highly overexpressed in European and Japanese groups. 6 Interestingly, genetic polymorphisms have recently been identified in the desmoglein 3 antibody target gene that associate with the disease and are in epistasis with the MHC associations. 7 PathogenesisThe primary pathogenic event in all forms of pemphigus is acantholysis, the separation of epidermal keratinocytes from each other. This leads to blister formation within the epidermis and is a key histological diagnostic feature of the disease (Fig 1).The immunopathology of pemphigus is increasingly well understood. 8,9 Antibodies develop against various elements of the desmosomes, particularly desmogleins 1 and 3. Desmoglein 1 is found particularly in the superficial layers of the epidermis; antibodies against this protein alone result in pemphigus foliaceus. Desmoglein 3 is more widely distributed in the lower parts of the epidermis and in mucosal epithelia. Thus, antibodies against this protein are associated with pemphigus vulgaris, typically presenting with muc...

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“…1 The most common pemphigus form is PV, which affects the skin and mucus membranes with an incidence that varies from 0.42 to 1.6 cases per 100 000 individuals; it is more frequent among Ashkenazi Jews. 2 It typically appears in the fourth or fifth decade of life, particularly in women (66 vs 44%, for women and men, respectively). 3,4 Typically, PV is characterized by suprabasal acantholysis and immunoglobulin G (IgG) and C3 deposits in the intercellular substance, evidenced by direct immunofluorescence or indirect immunofluorescence, with the appearance of a honeycomb.…”

Section: Introductionmentioning

confidence: 99%