Variable response of Hailey-Hailey disease to naltrexone: A case series

Hailey-Hailey disease (HHD), or familial benign pemphigus, is a heritable skin condition characterized by chronic waxing and waning blisters in intertriginous areas. The disease is caused by a mutation of the ATP2C1 gene, encoding a Ca+2-ATPase. Many treatments have been used for the disease, yet there is no effective treatment due to the lack of gene therapy. There are several reports of the use of low-dose naltrexone (LDN) to treat HHD. We add 3 cases of biopsy-proven HHD treated with naltrexone to the current literature.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Source Type: research

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Authors: Pezzolo E, Naldi L Abstract Introduction: "Chronic inflammatory immune-related skin disease" (ISDs) is an umbrella term grouping together heterogeneous entities characterised by chronic inflammation potentially involving the whole skin. We are not covering all ISDs in this review, but take a few as the most representative, including nonbullous and bullous diseases. The question we are aiming to address can be summarised as follows: "despite the differences, is it possible to define some unifying epidemiologic characteristics and shared progression pathways which can guide the organization of...
Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research
We present a case of EBVMCU in the gingiva of a 59-year-old male patient with long-standing pemphigus vulgaris. The initial presentation was suspicious for oral cavity cancer, which was ruled out by biopsy.
Source: International Journal of Oral and Maxillofacial Surgery - Category: ENT & OMF Authors: Tags: Case Report Source Type: research
Zhou and colleagues detected ectopic lymphoid structures (ELS) that resemble tertiary lymphoid organs in pemphigus vulgaris and pemphigus foliaceous lesions. ELS were characterized by B cells reactive to the desmoglein 3 autoantigen, and were associated with active disease and B cell expansion. B cell differentiation in ELS was supported by the detection of centroblasts, plasmablasts, and plasma cells in these lesions. Chemokines that may induce B cell migration to pemphigus lesions were also detected in ELS.
Source: Journal of Investigative Dermatology - Category: Dermatology Tags: Editorial Source Type: research
Pemphigus is an autoimmune bullous disease characterized by IgG production against desmogleins. The major sites of autoantibody production are thought to be lymph nodes, spleen, and bone marrow. Previously, it has been suggested that autoreactive B cells might exist in the skin lesions in pemphigus and produce autoantibodies. In their report, Zhou et  al. expanded their previous studies and reported that ectopic lymphoid-like structures were found in pemphigus skin lesions, wherein B-cell differentiation and lesional B-cell expansion might progress.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Commentary Source Type: research
Pemphigus is a chronic autoimmune blistering disorder, characterized by (muco-)cutaneous erosions due to autoantibodies against desmoglein 3 and/or 1. Pemphigus induction might be associated with drugs, malignancy or radiation therapy (RT); the latter being only rarely described. A rigorous literature review revealed around 30 cases of RT-associated pemphigus, which had been primarily treated with topical and/or systemic steroids, in some cases also dapsone or few other immunosuppressive agents were given. The most common underlying cancer type was breast cancer. We here present a 63-year-old male patient, who was pre-trea...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
PMID: 31957012 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
Publication date: Available online 18 January 2020Source: Gynecologic Oncology ReportsAuthor(s): Bijan Morshedi, Kari RingAbstractPemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma.The patient is a 54-year-old female with a past medical history of arthritis who presented with worsening fatigue in November 2017 and an itchy, blistering, erythematous annular plaque that first appeared on her...
Source: Gynecologic Oncology Reports - Category: OBGYN Source Type: research
Source: Journal of the American Academy of Dermatology - Category: Dermatology Authors: Source Type: research
In this study, we identified new molecular mechanisms of the synergistic cooperation of AuAbs to M3AR and SPCA1 in inducing acantholysis in the anti-Dsg 1/3 AuAb-negative PV patients. Anti-M3AR AuAb was found to play an important role in determining the level of intraepidermal split just above the basal cells, caspase to mediate early pro-apoptotic events triggered by anti-SPCA1 AuAb, and the neonatal Fc receptor (FcRn) to contribute to the pathobiological actions of both anti-M3AR and anti-SPCA1 AuAbs. Altogether, these novel results support our original hypothesis that pemphigus acantholysis is a complex disease process ...
Source: International Immunopharmacology - Category: Allergy & Immunology Authors: Tags: Int Immunopharmacol Source Type: research
Authors: Pires CAA, Einecke YS, Pereira NG, Costa CCC, Cunha ACDS, Moreira AG, Rodrigues FDN, Xavier MB Abstract Background: Pemphigus are rare chronic autoimmune blistering disorders with challenging therapeutic management.Objective: To investigate the therapeutic outcomes of pemphigus patients admitted to a dermatology referral service in northern Brazil.Materials and methods: We conducted a retrospective analysis of 32 patients with histopathological diagnosis of pemphigus between 2010 and 2016. Clinical and epidemiological data were collected. Therapeutic outcome was evaluated according to the criteria proposed...
Source: Journal of Dermatological Treatment - Category: Dermatology Tags: J Dermatolog Treat Source Type: research
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