Paraneoplastic pemphigus with negative direct immunofluorescence in epidermis or mucosa but positive findings in adnexal structures

Barnadas, M. A.; Curell, R.; Alomar, A.; Gelpí, Carmen

doi:10.1111/j.1600-0560.2008.00993.x

Cited by 16 publications

(12 citation statements)

References 19 publications

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“…Our findings of positive reactivity in dermal blood vessels, especially in the upper dermis and neurovascular skin appendices are suggestive of possible antigenicity for these structures in ABDs. Finally, recent studies have shown positive reactivity to eccrine and sebaceous glands in ABDs utilizing IIF and DIF [31][32][33][34][35]. In regard to the positive patterns seen in the dermis(possibly associated with cell junctions and/or telocytes), we were able to recently demonstrate activation of both multiple proteases and protease inhibitors following the same patterns of positivity we are describing here [36].…”

Section: Followed the Same Pattern As Fibrinogensupporting

confidence: 81%

Immunohistochemistry versus immunofluoresence in the diagnosis of autoimmune blistering diseases

Velez¹,

Howard²

2013

Our Dermatol Online

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Introduction: In patients with autoimmune skin blistering diseases (ABDs), the diagnostic gold standard has classically been direct and indirect immunofluorescence (DIF and IIF), despite inherent technical problems of autofluorescence. Aim: We sought to overcome autofluorescence issues and compare the reliability of immunofluorescence versus immunohistochemistry (IHC) staining in the diagnoses of these diseases. Methods: We tested via IHC for anti-human IgG, IgM, IgA, IgD, IgE, Kappa light chains, Lambda light chains, Complement/C3c, Complement/ C1q, Complement/C3d, albumin and fibrinogen in 30 patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia (El Bagre-EPF), and 30 control biopsies from the endemic area. We also tested archival biopsies from patients with ABDs whose diagnoses were made clinically, histopathologically and by DIF/IIF studies from 2 independent dermatopathology laboratories in the USA. Specifically, we tested 34 patients with bullous pemphigoid (BP), 18 with pemphigus vulgaris (PV), 8 with pemphigus foliaceus (PF), 14 with dermatitis herpetiformis (DH) and 30 control skin samples from plastic esthetic surgery reduction surgeries. Results: The diagnostic correlation between IHC and DIF-IIF was almost 98% in most cases. IHC revealed evidence of autofluorescence around dermal blood vessels, dermal eccrine glands and neurovascular packages feeding skin appendices in ABDs; this autofluorescence may represent a non-specific immune response. Strong patterns of positivity were seen also in endothelial-mesenchymal cell junction-like structures, as well as between dermal fibrohistiocytic cells. In PV, we noted strong reactivity to neurovascular packages supplying sebaceous glands, as well as apocrine glands with edematous changes. Conclusions:We suggest that IHC is as reliable as DIF or IIF for the diagnosis of ABDs; our findings further suggest that what has previously been considered DIF/IIF autofluorescence background may be of relevance in ABDs. Our discovery of reactivity against edematous dermal apocrine glands may be related to the fact that PV has a vegetant form, with lesions present in anatomic areas where these glands exist.

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Section: Followed the Same Pattern As Fibrinogensupporting

confidence: 81%

Immunohistochemistry versus immunofluoresence in the diagnosis of autoimmune blistering diseases

Velez¹,

Howard²

2013

Our Dermatol Online

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“…Antibodies against envoplakin and periplakin are believed to be the most sensitive and specific indicators of disease 39 . However, the development of plakin autoantibodies has been reported as delayed, limited, or absent in a number of cases of PAMS 40–42 . Both desmoglein (Dsg) 1 and 3 antibodies also have been found in patients with PAMS 43 .…”

Section: Pathophysiologymentioning

confidence: 99%

Paraneoplastic autoimmune multiorgan syndrome: 20 years after

et al. 2011

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The purpose of this review is to provide insight and clarification in the quandary of classification and delineate clinical and histological features and pathophysiology of paraneoplastic pemphigus. This is a paraneoplastic disease of epithelial autoimmunity and adhesion originally described by Dr. Anhalt in 1990. Paraneoplastic pemphigus represents only one manifestation of the heterogeneous autoimmune syndrome in which patients, in addition to small airways occlusion, may display a spectrum of at least five clinical variants of the mucocutaneous disease [i.e. pemphigus-like, pemphigoid-like, erythema multiforme-like, graft-versus-host disease-like, and lichen planus-like, termed paraneoplastic autoimmune multiorgan syndrome (PAMS)]. There is a need for the expanded, inclusive classification of diverse mucocutaneous and respiratory presentations of PAMS. Multiple specific effectors of humoral and cellular autoimmunity mediating epithelial damage have been identified. An update of advances in clinical and basic research on PAMS and in management and overall prognosis of PAMS is provided.

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“…This finding raises the question of whether periadnexal direct immunofluorescence (DIF) findings could offer reasonable, proxy diagnostic information in situations where the epidermis or DEJ cannot be evaluated. Although this hypothesis has not been specifically investigated previously, a prior study reported three patients with paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) who were found to have intercellular immunoglobulin (Ig) G and IgM deposits surrounding adnexal structures on DIF testing, when DIF findings of the DEJ were absent …”

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confidence: 99%

Diagnostic utility of direct immunofluorescence findings around hair follicles and sweat glands in immunobullous disease

Lehman

Camilleri

2012

J Cutan Pathol

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Paraneoplastic pemphigus with negative direct immunofluorescence in epidermis or mucosa but positive findings in adnexal structures

Abstract: Negative DIF findings in epithelia do not rule out the diagnosis of PNP, and the presence of IgG and/or C3 at the intercellular level of adnexal structures can help establish this diagnosis.

Cited by 16 publications

References 19 publications

Immunohistochemistry versus immunofluoresence in the diagnosis of autoimmune blistering diseases

Immunohistochemistry versus immunofluoresence in the diagnosis of autoimmune blistering diseases

Paraneoplastic autoimmune multiorgan syndrome: 20 years after

Diagnostic utility of direct immunofluorescence findings around hair follicles and sweat glands in immunobullous disease

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