A case of anti-laminin γ1 (p200) pemphigoid with psoriasis vulgaris successfully treated with apremilast

Waki, Yuma; Komine, Mayumi; Maekawa, Takeo; Murata, Satoru; Ishii, Norito; Hashimoto, Takashi; Ohtsuki, Mamitaro

doi:10.1684/ejd.2018.3280

Cited by 12 publications

(7 citation statements)

References 8 publications

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“…A mixed infiltrate of neutrophils and eosinophils was seen in 40 (40.0%) of patients. In two patients, an exclusively lymphocytic infiltrate was observed (24, 25); papillary microabscesses were observed in 11 (11.0%) patients, a predominantly neutrophilic infiltrate in 10 patients (2, 14, 18–20, 26–29) and a mixed infiltrate in one patient (3). Eosinophilic spongiosis was observed in two patients (3, 27) and ruptured secondary milia with inflammatory granulomatous in one patient (16).…”

Section: Resultsmentioning

confidence: 99%

Anti-p200 Pemphigoid: A Systematic Review

Kridin

Ahmed

2019

Front. Immunol.

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The many clinical aspects of anti-p200 pemphigoid are not well-characterized. We aimed to analyze and correlate known existing data on the epidemiological, clinical, histological, and immunological features of anti-p200 pemphigoid. We performed a review using Medline, Embase, and Web of Science databases (1900–2018). Case reports and series of patients were included. A total of 68 eligible studies that comprised 113 anti-p200 pemphigoid patients were included in the qualitative analysis, where there was a mean age of onset of 65.5 years. All patients presented with bullae/vesicles, and 54.3% had urticarial plaques. A similarity to bullous pemphigoid was reported in 66.1% of cases, but palmoplantar (51.4%), cephalic (40.3%), and mucosal (38.5%) involvement, besides frequent development of scars/milia (15.7%), were reported. Autoantibodies against recombinant laminin γ1 were detected in the sera of 73.1% of patients. Psoriasis was present in 28.3% of anti-p200 pemphigoid patients, particularly among Japanese patients (56.4%). The incidence of pustular psoriasis in this subgroup, was significantly greater than in the normal population. In conclusion, the diagnosis of anti-p200 pemphigoid may be suspected when a subepidermal autoimmune blistering disease develops in a younger age group, along with significant acral and cephalic distribution and mucosal involvement.

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Section: Resultsmentioning

confidence: 99%

Anti-p200 Pemphigoid: A Systematic Review

Kridin

Ahmed

2019

Front. Immunol.

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“…Though approved only for plaque psoriasis, psoriatic arthritis and Behcet's disease, off-label reports with limited patient numbers suggest efficacy in discoid lupus erythematosus, atopic dermatitis, pytiriasis rubra pilaris, and generalized granuloma anulare. PDE4i seems to be effective in the management of laminin g-1 pemphigoid as reported in an individual with concomitant psoriasis and in a preclinical model of epidermolysis bullosa acquisita (8,12). Both are blistering disorders with skin fragility, driven by autoantibodies against laminin g-1 and collagen VII, respectively.…”

Section: Discussionmentioning

confidence: 92%

Case Report: Apremilast for Therapy-Resistant Pemphigus Vulgaris

et al. 2020

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Background: In pemphigus, elucidating the disease-causing immune mechanism and developing new therapeutic strategies are needed. In this context, the second messenger 3′,5′-cyclic adenosine monophosphate (cAMP) is gaining attention. cAMP is important in hematological and auto-inflammatory disorders. A class of enzymes called phosphodiesterases (PDEs) control intracellular cAMP levels. In pemphigus, cAMP levels increase following IgG binding to Dsg3. This appears to be a mechanism to preserve epithelial integrity. Objectives: To determine whether apremilast, an inhibitor of the PDE4 normally used in psoriasis, may be of benefit in the blistering skin disorder pemphigus. Methods: Here we report of a 62 years old patient with chronic debilitating and recalcitrant pemphigus not responding to several previous treatments, who received treatment with apremilast over a period of 32 weeks. Desmoglein autoantibody levels were assessed by Enzyme-linked Immunosorbent Assay (ELISA), whereas disease severity and quality of life were assessed by the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). In an attempt to explain the effects of apremilast in pemphigus, peripheral blood mononuclear cells (PBMCs) were analyzed for the duration of treatment by flow cytometry for the distribution of specialized T cell subsets. The frequencies of circulating T helper (Th) 1, Th2, Th17, Th17.1 and T follicular helper (Tfh) 1, Tfh2, Tfh17, and Tfh17.1 were analyzed by CCR6, CXCR3, and CXCR5 expression of CD4 + T cells. Further, based on the different expressions of CXCR5, CD127, and CD25, we analyzed the T regulatory (Treg) and T follicular regulatory (Tfreg) compartment. Results: In response to apremilast treatment, Dsg-specific autoantibody titers decreased, blistering ceased and lesions healed, showing a long-lasting effect. While the frequencies of most of the Th and Tfh cell subsets remained unchanged, we observed a continuous increase in Treg and Tfreg cell levels. Conclusion: Our findings are encouraging and warrant extension of the beneficial effect of PDE4 inhibition on a larger cohort of pemphigus patients.

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“…Six patients had a history of UV therapy, but one patient was not on the treatment session at the onset of pemphigoid ( 33 ) ( Table 5 ) . Among 19 patients who reported therapy strategies leading to first complete remission, six patients were treated with prednisolone ( 20 , 26 , 31 , 33 , 37 , 38 ), three patients cyclosporine ( 1 , 25 , 30 ) and the rest minocycline ( 30 ), topical corticosteroids ( 23 ), and dapsone ( 34 ), respectively, as effective monotherapy. The remaining seven (36.8%) patients were administrated with corticosteroid (orally or topically) and other adjuvant therapies [cyclosporine ( 28 ), mycophenolate mofetil ( 39 ), plasmapheresis ( 32 , 40 ), apremilast ( 35 ), and intravenous immunoglobulin (IVIG) ( 36 )].…”

Section: Resultsmentioning

confidence: 99%

Coexistence of Anti-p200 Pemphigoid and Psoriasis: A Systematic Review

Xie

Wang

et al. 2022

Front. Immunol.

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BackgroundA close association between psoriasis and anti-p200 pemphigoid has been demonstrated by numerous studies. However, the clinical characteristics of patients suffering from these two entities have not yet been well-elucidated.ObjectiveThis study aimed to review the case reports and case series, summarizing clinical features and therapeutic strategies in patients suffering from anti-p200 pemphigoid and psoriasis.MethodsA systematic review was conducted by searching PubMed, EMBASE, and Web of Science databases for studies published in English involving patients with psoriasis and anti-p200 pemphigoid on 6 September 2021. All case reports and case series reporting patients diagnosed with anti-p200 pemphigoid and psoriasis were included in this systematic review.ResultsA total of 21 eligible studies comprising 26 anti-p200 pemphigoid patients with preceding psoriasis were included in the qualitative synthesis. The average age at blisters eruption was 62.5 years, and the mean duration between the two entities was 15.6 years. Twenty-four percent of patients developed bullous lesions during UV therapy. Clinical manifestation of bullae and/or vesicles was recorded in all patients, and the trunk (94.7%) was most frequently involved, with only 15.8% reporting mucosal involvement. Epitope spreading was detected by immunoblotting in 33.3% of patients. All the patients reached completed remission during the course of disease, with 36.8% experiencing at least one relapse. Monotherapy of prednisolone was the leading therapeutic approach (n=6, 31.6%) required for disease control, but 5 (83.3%) of them suffered from blister recurrence after tapering or ceasing corticosteroid.ConclusionMost of the clinical aspects of patients with anti-p200 pemphigoid and psoriasis were similar to what was demonstrated in previous articles on anti-p200 pemphigoid. Nevertheless, compared with other anti-p200 pemphigoid cases without psoriasis, a clinical manifestation pattern with more frequent involvement of the trunk and less mucosal involvement was illustrated in those with psoriasis. Generally, monotherapy is sufficient for a complete remission for such patients. However, one or more relapses have been recorded in a considerable portion of patients, especially those prescribed with prednisolone. It reminded us to be more cautious during a tapering of medication.

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A case of anti-laminin γ1 (p200) pemphigoid with psoriasis vulgaris successfully treated with apremilast

Cited by 12 publications

References 8 publications

Anti-p200 Pemphigoid: A Systematic Review

Anti-p200 Pemphigoid: A Systematic Review

Case Report: Apremilast for Therapy-Resistant Pemphigus Vulgaris

Coexistence of Anti-p200 Pemphigoid and Psoriasis: A Systematic Review

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