Immunopathology of iga pemphigus request pdf researchgate. The clinical and histologic lesions of iga pemphigus are unique amongst the pemphigus group, producing pustules in addition to the characteristic pauciinflammatory blister. Iga pemphigus is a subtype of pemphigus with two distinct forms. The distribution of iga pemphigus antigen in human skin and the role of iga anticell surface antibodies in the induction of intraepidermal acantholysis. Immunoglobulin a iga pemphigus is a group of newly characterized immunemediated intraepidermal blistering skin diseases. In our department of oral and maxillofacial surgery, nuh, pemphigus vulgaris is an uncommon cause of chronic oral mucosal ulceration. Pemphigoid is a group of rare autoimmune blistering skin diseases. The conditions may therefore affect areas of the body such as.
David weedon ao md frcpa fcaphon, in weedons skin pathology third edition, 2010. Clinical, histopathological and immunological distinction in two. Iga pemphigus is a rare pustular autoimmune disease with exclusive iga anti. Background iga pemphigus is a rare neutrophilic acantholytic autoimmune disease. Iga pemphigus is an autoimmune, intraepidermal vesiculobullous eruption, with variable acantholysis and the presence of intercellular deposits of iga within the epidermis. Of those patients with pemphigus vulgaris, nearly half were chinese, 5 indians and 1 malay. Induction of pemphigus in neonatal mice by passive transfer of igg from patients with the disease. Both occur in middleaged or elderly persons with a slight female predominance. Pemphigus foliaceus masquerading as iga pemphigus and. Pemphigus vegetans causes thick sores in the groin and under the arms. Pemphigoid is more common than pemphigus, and is slightly more common in women than in men. While most of the reported cases describe vesicular andor pustular cutaneous eruption, only four cases had mucosal involvement 1. Apr 18, 2017 histopathology demonstrates an intraepidermal blister, which may be located subcorneally spdtype iga pemphigus, suprabasally, or at mid epidermis ientype iga pemphigus.
Common subepidermal, blistering, autoimmune disease of skin due to igg antibodies to the hemidesmosomal antigens bullous pemphigoid antigen 1 and 2. Subcorneal pustular dermatosis also known as sneddonwilkinson disease and pustulosis subcornealis is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils 203 this is distinct from and not to be. The four major types of the pemphigus include pemphigus vulgaris, pemphigus foliaceus, iga pemphigus, and paraneoplastic pemphigus. Iga pemphigus is a heterogeneous variant of pemphigus and must be differentiated from other blistering diseases including dermatitis herpetiformis, pemphigus herpetiformis, pemphigus foliaceus, pemphigus erythematosus, pemphigus vulgaris, pemphigus vegetans, paraneoplastic pemphigus or linear iga bullous dermatosis. Currently, igap is subdivided based on histopathological and. The diagnosis of iga pemphigus requires a skin biopsy for histology and direct immunofluorescent testing. Iga pemphigus the pustules tend to coalesce in an annular or circinate pattern with central crusting, though a sunflower configuration is more characteristic for ien type. Pemphigus vulgaris pathogenesis, clinical features. Target antigens are the cellcell adhesion molecules referred to as desmogleins. Discrepancies among clinical, histological and immunological findings in iga pemphigus. Bruckner al, fitzpatrick je, hashimoto t, weston wl, morelli jg.
Subcorneal pustular dermatosis type a case report with vesicle and pustular eruption for years cured with dapsone in 3 weeks yungchuan liu1 tienyi tzung1 chibin lin2 peiyu lo3 iga pemphigus is a newly characterized group of autoimmune intraepidermal blistering. Iga pemphigus was first described by wallach, foldes, and cottenot in 1982 under the name subcorneal pustular dermatosis and monoclonal iga. Request pdf on nov 1, 2001, takashi hashimoto and others published immunopathology of iga pemphigus find, read and cite all the research you need on. Subepidermal blister with eosinophils and superficial dermal edema.
Iga pemphigus, he 40x 4300 iga pemphigus, anti iga, fitc 20x 6168. In iga pemphigus, a skin biopsy reveals an intraepidermal blister. While dif is still the diagnostic method of choice for linear iga bullous disease and iga pemphigus, elisa is a more accurate, costeffective and less invasive method of diagnosis for several aibds including pemphigus vulgaris and foliaceus, based on currently available evidence. In china, the number of the patients with pf and pnp are lower than that of pv, perhaps because of the limitation of diagnostic methods. This type can also cause small bumps with pus inside. In the classical textbooks of skin pathology 14 it is stated that. Dermatitis herpetiformis and linear iga bullous dermatosis dermatology. Pemphigus vulgaris pathology outlines and treatment. Among them, iga pemphigus is characterized by tissuebound and circulating iga antibodies targeting desmosomal or nondesmosomal cell surface components in the epidermis.
Request pdf clinical, histopathological and immunological distinction in two cases of iga pemphigus two cases of iga pemphigus, one of the subcorneal. Iga pemphigus, he 40x 4300 iga pemphigus, antiiga, fitc 20x 6168. Granular or fibrillar iga at the tips of the dermal papillae, along bmz. Histopathologic examination of iga pemphigus shows slight acantholysis and neutrophilic infiltration in the epidermis. Patients present with tense bullae, which do not rupture easily, on an erythematous base. In our hospital, from 1989 to present, we have detected 32 patients with pemphigus. Histopathology demonstrates an intraepidermal blister, which may be located subcorneally spdtype iga pemphigus, suprabasally, or at mid epidermis ientype iga pemphigus.
Discrepancies among clinical, histological and immunological. The word pemphigus comes from the greek word pemphix which means blister or bubble. The examination of skin biopsies by light microscopy and immunofluorescence remains the standard for diagnosis today. The presence of iga in the epidermis was first reported by varigos. Conclusions the present report represents the largest. The average age at the time of diagnosis was 49 years. Pictures iga pemphigus, after several years followed by myeloma he and anti iga immunofluorescence. The diagnosis requires confirmation by immunofluorescence differential diagnosis against subcorneal pustular dermatosis or pemphigus foliaceus and pemphigus vulgaris. Paraneoplastic pemphigus is a rare type of pemphigus. Pemphigus patients develop mucosal erosions andor blisters, erosions, or small bumps that fill with pus or fluid.
Note the subcorneal pustule in a, and micropustule in the entire epidermis in b hematoxylin and eosin, bar 100. Summary iga pemphigus igap is a rare, distinct variant of pemphigus. Unlike typical immunoglobulin g iggmediated pemphigus, iga pemphigus is characterized by tissuebound and circulating iga autoantibodies that target the desmosomal proteins of the epidermis. Comparison of histopathology, immunofluorescence, and. Jan 19, 20 iga pemphigus was first described by wallach, foldes, and cottenot in 1982 under the name subcorneal pustular dermatosis and monoclonal iga. Atypical igaigg pemphigus involving the skin, oral mucosa, and colon in a child. This type of pemphigus is caused by an antibody called iga. Traditional histopathology and tissue based techniques have played a pivotal role in the understanding of pemphigus and in highlighting the diversity within this group of diseases. The problem of histopathological differentiation of bullous diseases has been focused on pemphigus, dermatitis herpetiformis, and erythema multiforme. The blisters look like those in pemphigus foliaceus. B, histopathologic analysis of lesional skin that shows a subcorneal blister.
In vivo, it is characterized by linear deposition of igg, iga, or c3 along the epithelial basement membrane zone. Pemphigus vulgaris is an autoimmune mucocutaneous condition occurring as a result of inappropriate production of autoantibodies against the desmosomes holding the epithelial cells together. Pictures iga pemphigus, after several years followed by myeloma he and antiiga immunofluorescence. Pv is the most common disease and represents this kind of potentially lifethreatening disease. The onset of iga pemphigus is reported to be subacute. Subcorneal pustular dermatosis also known as sneddonwilkinson disease and pustulosis subcornealis is skin condition that is a rare, chronic, recurrent, pustular eruption characterized histopathologically by subcorneal pustules that contain abundant neutrophils. Pemphigus is a lifethreatening autoimmune blistering disease. Pemphigus vulgaris pv is an immunobullous intraepithelial disease characterized by vesicle formation on the skin and mucous membrane. Iga pemphigus is a rare autoimmune blistering disease characterized by epidermal iga immunoglobulin deposits. As its name indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells. Iggiga pemphigus with autoantibodiesto desmoglein 1 and. Discrepancies between clinical, histopathologic, and immunologic features make it difficult to finally establish the subtype of iga pemphigus.
We only diagnose these disorders by clinical symptoms, histopathology and immuno. As its name indicates, pemphigoid is similar in general appearance to pemphigus, but, unlike pemphigus, pemphigoid does not feature acantholysis, a loss of connections between skin cells pemphigoid is more common than pemphigus, and is slightly more common in women than in men. Pemphigus is a group of organspecific autoimmune disorders, including pemphigus vulgaris pv, pemphigus foliaceus pf and paraneoplastic pemphigus pnp. Iga pemphigus of the subcorneal pustular dermatosis type is a rare autoimmune blistering disease in the pemphigus spectrum. Pemphigus is the term used to describe a group of autoimmune diseases that cause the skin and mucous membranes to become blistered.
Subcorneal pustular dermatosis type a case report with vesicle and pustular eruption for years cured with dapsone in 3 weeks yungchuan liu1 tienyi tzung1 chibin lin2 peiyu lo3 iga pemphigus is a newly characterized. Atypical iga igg pemphigus involving the skin, oral mucosa, and colon in a child. Histopathology pemphigus vulgaris the earliest changes are intercellular edema and. Pemphigus vulgaris pv pv is a serious autoimmune disorder with mucocutaneous manifestations characterized by the development of blisters on the skin andor mucosal membranes. Typically there is a more prominent subcorneal neutrophilic.
The histopathology of iggiga pemphigus is also variable, and may exhibit acantholysis, subcorneal and intraepidermal. Direct immunofluorescence of a skin biopsy of healthy appearing skin close to the area of blistering reveals iga deposition in epidermal keratinocyte cell membranes. Subcorneal pustular dermatosistype iga pemphigus with. Histopathology demonstrates silent or absent acantholysis and is not characteristically observed as in classic pemphigus. Rare 1 2% of pemphigus cases variant of pemphigus vulgaris characterized by verrucous plaques in the flexural regions oral cavity is commonly affected. In this paper i am presenting detailed histopathological study of pemphigus foliaceus. Histologic examination of iga pemphigus demonstrates subcorneal blisters with massive neutrophilic. There is a mixed dermal inflammatory infiltrate, with intraepidermal neutrophil infiltration. Histopathology of pemphigus foliaceus jama dermatology. Patients are clinically characterized by extensive erythemas that.