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Pemphigoid Nodularis

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The text is the summary of recent articles on Pemphigoid Nodularis at 75 thresold from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.


Considering these clinical, histologic, and immunofluorescence findings, the diagnosis of a Nodular subtype of bullous pemphigoid was made [1]. The diagnosis of Purpuric BP was made on the basis of history, clinical presentation, histopathology report, direct and indirect immunofluorescence studies the diagnosis of Purpuric BP was made [2]. Patients with pemphigoid nodularis have clinical features of prurigo nodularis in combination with clinical or immunologic characteristics of Bullous pemphigoid [3]. We report the case of a 71-year-old Woman with pemphigoid nodularis [4]. The diagnosis was suspected clinically and established by positive indirect immunofluorescence (IIF) findings characteristic of pemphigoid [5].

Direct immunofluorescence examination, indirect immunofluorescence examination using skin split with 1 mol/L sodium chloride, and immunoblotting analysis using extracts of normal human Dermis gave results compatible with EBA [6]. The patient had Chronic Dermatitis and later developed bullous pemphigoid [7]. These lesions were confirmed by Biopsy, Direct Immunofluorescence, and enzyme-linked immunosorbent assay of the 180 kd bullous pemphigoid antigen to be pemphigoid nodularis [8]. The extracellular portion of collagen XVII is constitutively shed from the cell surface by ADAMs (proteinases that contain adhesive and metalloprotease domains) [9]. Cell biological analyses suggest that collagen XVII functions as a cell-matrix adhesion molecule through stabilization of the hemidesmosome complex [10].

This concept is supported by investigations into human diseases of the dermoepidermal junction, in which collagen XVII is either genetically defective or absent (as in some forms of nonlethal junctional epidermolysis bullosa) [11]. Autoantibodies against collagen XVII (BP180) are seen in bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid, linear IgA disease, lichen planus pemphigoides and pemphigoid nodularis [12]. In vivo and in vitro studies provide evidence for a pathogenic role of these autoantibodies, and suggest that the serum level and epitope specificity of these antibodies influences disease severity and phenotype [13]. The condition is characterized by the presence of prurigo nodularis-like lesions, possible history of blistering, and immunohistochemical findings of bullous pemphigoid [14]. Western immunoblotting of EDTA-separated epidermal extracts revealed that the 230-kD bullous pemphigoid (BP) antigen was recognized by circulating autoantibodies in the patient sera, but the 180-kD BP antigen was not [15].

The disease spectrum of LPP and pemphigoid nodularis differs from that of classical BP phenotype, and their presentations are often indolent [16]. LPP may predominantly affect a younger age group and is responsive to standard treatments used in acquired autoimmune Bullous diseases, while pemphigoid nodularis is more common in elderly Women and is relatively resistant to therapy [17]. We describe a patient who had LPP for nearly two decades and subsequently developed a Nodular Eruption with a concurrently detected antibullous pemphigoid antigen 2 (BP180) autoantibody [18]. In pemphigoid nodularis, the immunopathological findings are identical to those of bullous pemphigoid (BP) [19]. We describe a patient with pemphigoid nodularis that fulfilled the criteria of bullous pemphigoid by histopathologic examination and direct and indirect immunofluorescence studies [20].

Bullous pemphigoid complicated by internal malignancy has been well documented [21]. We report a 49-year-old Japanese male with clinical and histopathological features of pemphigoid nodularis including circulating and in vivo-bound IgG antibasement membrane zone Antibodies and IgA anti-intercellular Antibodies [22]. NC16a domain is now considered to be the most pathogenic site of bullous pemphigoid [23]. Compared with the results of typical bullous pemphigoid patients, Localized pemphigoid sera detected the 180 kD bullous pemphigoid antigen less frequently, and sera from both Localized pemphigoid and pemphigoid nodularis showed a lower end point of titer of Antibodies to NC16a domain [24]. Direct Immunofluorescence was positive in all cases, with Linear basement membrane zone deposition of IgG and C3 [25].

Circulating IgG against the basement membrane was also detected by Indirect Immunofluorescence [26]. By Western immunoblotting the 230 kd bullous pemphigoid antigen was recognized by circulating autoantibodies [27]. Patients were females aged 76, 71 and 50 years, and all had features of bullous pemphigoid together with prurigo-like lesions at some stage of their illness [28]. Direct Immunofluorescence in all cases was positive, with linear basement membrane zone deposition of IgG and C3 [29]. Indirect Immunofluorescence studies disclosed circulating anti-basement membrane zone Antibodies [30].

Several variants have been described, including pemphigoid nodularis, which may mimic or evolve from or into prurigo nodularis [31]. The casual relationship between prurigo nodularis and Bullous pemphigoid is unknown [32]. We describe a patient with prurigo nodularis and no immunologic evidence of pemphigoid who subsequently developed Bullae [33]. Direct immunofluorescence then confirmed the diagnosis of bullous pemphigoid [34]. Direct Immunofluorescence studies of a Nodular lesion revealed Linear deposition of IgG and C3 at the Epidermal basement membrane zone (EBMZ) [35].

Indirect immunofluorescence studies of serum revealed IgG pemphigoid antibasement membrane zone Antibodies in 1:1,280 titer [36].

References: 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ,

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