Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes is particularly helpful in cases of skin lesions presenting as the first sign of the malignancy, and the underlying malignancy can be treated in a timely manner. Autoimmune paraneoplastic and neoplasm-associated bullous skin syndromes are characterized by blister formation due to an autoimmune response to components of the epidermis or basement membrane in the context of a neoplasm. The clinical manifestations, histopathology and immunopathology findings, target antigens, associated neoplasm, current diagnostic criteria, current understanding of pathogenesis, and treatment options for a selection of four diseases are reviewed. Paraneoplastic pemphigus manifests with clinically distinct painful mucosal erosions and polymorphic cutaneous lesions, and is often associated with lymphoproliferative neoplasm. In contrast, bullous pemphigoid associated with neoplasm presents with large tense subepidermal bullae of the skin, and mild mucosal involvement, but without unique clinical features. Mucous membrane pemphigoid associated with neoplasm is a disorder of chronic subepithelial blisters that evolve into erosions and ulcerations that heal with scarring, and involves stratified squamous mucosal surfaces. Linear IgA dermatosis associated with neoplasm is characterized by annularly grouped pruritic papules, vesicles, and bullae along the extensor surfaces of elbows, knees, and buttocks. Physicians should be aware that these autoimmune paraneoplastic and neoplasm-associated syndromes can manifest distinct or similar clinical features as compared with the non-neoplastic counterparts.

Paraneoplastic Pemphigus and Autoimmune Blistering Diseases Associated with Neoplasm: Characteristics, Diagnosis, Associated Neoplasms, Proposed Pathogenesis, Treatment

Patients with psoriasis are frequently complicated with autoimmune bullous diseases, especially, pemphigoid diseases. It has been known that one-third cases of anti-laminin gamma1 pemphigoid, formerly anti-p200 pemphigoid, are associated with psoriasis whereas bullous pemphigoid is the most frequently associated bullous disease in psoriasis cases regardless of the lack of detectable levels of the accompanying anti-laminin gamma1 autoantibodies. Despite several suggestions, however, the definitive reason of the striking association of psoriasis and these autoimmune bullous diseases remains elusive. In this review, we look over the epidemiological evidence of the association of psoriasis and autoimmune bullous diseases and the information of genetic susceptibilities of each disease, and discuss the possible mechanisms of their complication with reference to the recent understandings of each pathogenesis.

/pdf/interaction-of-psoriasis-and-bullous-diseases-jiz5zt9god.pdf

Interaction of Psoriasis and Bullous Diseases.

Psoriasis is a chronic inflammatory skin disease for which no cure has emerged. Its complex etiology requires the development of an in vitro model representative of the pathology. In this study, we exploited gene profiling analyses on microarray in order to characterize and further optimize the production of a human psoriatic skin model representative of this in vivo skin disease. Various skin substitutes were produced by tissue-engineering using biopsies from normal, healthy donors, or from lesional or non-lesional skin samples from patients with psoriasis, and their gene expression profiles were examined by DNA microarray. We demonstrated that more than 3540 and 1088 genes (two-fold change) were deregulated between healthy/lesional and lesional/non-lesional psoriatic substitutes, respectively. Moreover, several genes related to lipid metabolism, such as PLA2G4E and PLA2G4C, were identified as repressed in the lesional substitutes. In conclusion, gene profiling analyses identified a list of deregulated candidate genes associated with various metabolic pathways that may contribute to the progression of psoriasis.

https://www.mdpi.com/1422-0067/19/10/2923/pdf

The Tissue-Engineered Human Psoriatic Skin Substitute: A Valuable In Vitro Model to Identify Genes with Altered Expression in Lesional Psoriasis.

The coexistence of psoriasis with autoimmune bullous diseases (AIBDs), particularly bullous pemphigoid (BP), has been documented in case reports and series, as well as in epidemiological studies. The onset of psoriasis precedes that of BP in the majority of cases. Patients with concomitant BP and psoriasis are generally younger at the onset of BP and present with fewer erosions and blisters as compared with patients suffering from isolated BP. Intriguingly, it has been speculated that some BP cases with comorbid psoriasis can actually correspond to anti-laminin gamma-1 pemphigoid, a rare form that was recently recognized as a distinct entity and which can mimic BP and/or other subepidermal AIBDs. The pathomechanisms underlying the BP–psoriasis association have not yet been identified, although several hypotheses have been proposed. The most credited among such hypotheses involves the so-called “epitope spreading” phenomenon, with tissue injury secondary to a primary inflammatory process (i.e., psoriasis) leading to the exposure of sequestered antigens evoking a secondary autoimmune disease (i.e., bullous pemphigoid). This narrative review aims to give a brief overview of the association between psoriasis and BP, examining epidemiological, clinical, and immunopathological features, the pathomechanisms underlying this association, the treatments for psoriasis incriminated as potential triggers of BP, and the therapeutic management of patients with psoriasis and BP. 

/pdf/the-intriguing-links-between-psoriasis-and-bullous-252ilvda.pdf

The Intriguing Links between Psoriasis and Bullous Pemphigoid

Pemphigoid gestationis is a skin-specific autoimmune disorder that can sometimes present as the cutaneous manifestation of a multiorgan disease due to potentially common pathogenic mechanisms We report a severe form of pemphigoid gestationis in a 32-year-old primigravida woman, who presented at 22 weeks of gestation with headaches and blurred vision, later developing encephalitis, intrauterine fetal demise and dilated cardiomyopathy

Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder.

Bullous pemphigoid (BP) and psoriasis vulgaris represent two clinically well characterized, inflammatory, chronic skin diseases. A 62 years old female patient, from rural areas, was admitted for the presence of erythematous plaques covered by large tense blisters with clear fluid, located symmetrically on the anterior part of the upper limbs, the trunk, the neck and the lower limbs. Also the lesions were intense itching. Lesions occurred three days before presentation at the clinic. Medical history revealed psoriasis diagnosed 28 years ago, breast cancer treated with surgery, radio and chemotherapy three years ago and Parkinson's disease diagnosed 3 weeks prior to presentation to the dermatology clinic. Histopathology examination revealed: atrophic epidermis with subepidermal presence of a blister containing numerous eosinophils and neutrophils. In the papillary dermis neutrophils and eosinophils predominantly vascular. Bullous pemphigoid has multiple etiology. Bullous pemphigoid is an autoimmune subepidermal bullous dermatosis which may be associated with psoriasis. Medical literature and cases reported in dermatology journals claim that bullous pemphigoid is often associated with psoriasis, though the immunogenetical and immunopathologycal mecanismes are still not known. Our patient has three different diseases but their etiology and pathogenesis can interfere.

Bullous pemphigoid associated with psoriasis, breast cancer and Parkinson's disease.

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Bullous pemphigoid associated with psoriasis, breast cancer and Parkinson's disease.