Intraepithelial autoimmune bullous dermatoses disease activity assessment and therapy

Montagnon, Carmen M.; Lehman, Julia S.; Murrell, Dédée F.; Camilleri, Michael; Tolkachjov, Stanislav N.

doi:10.1016/j.jaad.2021.02.073

Cited by 13 publications

(17 citation statements)

References 141 publications

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“…Pemphigus often requires inductive therapy with corticosteroids or rituximab, followed by long-term immunosuppressants such as cytotoxic immunomodulators (mycophenolate mofetil, azathioprine, methotrexate, cyclosporine) and antimalarial agents. 6 We encourage clinicians to conduct appropriate cancer screening in patients with pemphigus before initiating rituximab and long-term immunosuppressive therapies. Dapsone and tetracyclines have both shown efficacy in pemphigus and may be the preferred maintenance drugs in patients with uncertain cancer status.…”

Section: Discussionmentioning

confidence: 99%

“…Dapsone and tetracyclines have both shown efficacy in pemphigus and may be the preferred maintenance drugs in patients with uncertain cancer status. 6 For more severe cases, intravenous immunoglobulin is a nonimmunosuppressive and efficacious option. 6 A consensus on future guidelines on cancer screening in patients with pemphigus and treatment algorithms for those with concomitant malignancies is needed for safe and effective management of pemphigus.…”

Section: Discussionmentioning

confidence: 99%

“…6 For more severe cases, intravenous immunoglobulin is a nonimmunosuppressive and efficacious option. 6 A consensus on future guidelines on cancer screening in patients with pemphigus and treatment algorithms for those with concomitant malignancies is needed for safe and effective management of pemphigus.…”

Section: Discussionmentioning

confidence: 99%

“…Collectively, these findings confirmed a diagnosis of PF, with a pemphigus disease activity index score of 35. 6 The patient was started on a prednisone taper course, with rapid improvement, and was transitioned to mycophenolate mofetil. Meanwhile, she underwent laboratory workup and age-appropriate cancer screening to rule out underlying autoimmune and paraneoplastic etiologies.…”

Section: Case Reportmentioning

confidence: 99%

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Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

et al. 2021

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

See 2 more Smart Citations

Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

et al. 2021

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“…The mainstay of treatment is currently systemic glucocorticoids. In moderate‐to‐severe PV and PF, clinicians commonly use systemic immunosuppressive agents, such as rituximab, azathioprine, or mycophenolate mofetil, while tapering glucocorticoids 3 …”

Section: Introductionmentioning

confidence: 99%

Assessing the use of methotrexate as an alternate therapy for pemphigus vulgaris and pemphigus foliaceus

Kolla

Shah

Cymerman

et al. 2022

Dermatologic Therapy

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Methotrexate is historically recognized as an effective treatment of pemphigus but its utility as a single or alternate steroid‐sparing agent was not recognized in recent consensus recommendations in pemphigus management. We aimed to evaluate the efficacy and safety of a treatment course for pemphigus that involves methotrexate as a single or steroid‐sparing agent. In a retrospective cohort study, we examined patients with pemphigus vulgaris or pemphigus foliaceus who were on ≥3 months of methotrexate therapy. Efficacy and safety were evaluated by established pemphigus disease endpoints. Of the 34 patients who met inclusion criteria, 25 (73.5%) were on glucocorticoids at time of methotrexate initiation (median follow‐up: 5.4 years; median time on methotrexate: 3.7 years). An appreciable proportion achieved disease control (91.2%), with some achieving clinical remission off all systemic therapies (23.5%). For patients on glucocorticoids, median time to control was 42 days, median time to minimal steroid dose tapering (5 mg prednisone) was 161 days, and median time to complete steroid tapering was 308 days. For patients on methotrexate as a single agent, median time to control was 119 days. Among all patients, relapse commonly occurred (88.2%). At last follow‐up, 26.5% were managed on topical therapies alone and 11.8% required systemic steroid therapy. Methotrexate was largely tolerated with a low incidence of adverse events leading to treatment discontinuation (2.9%). Methotrexate has the potential to be an effective and well‐tolerated option for patients and may be considered for use as an alternate single or steroid‐sparing agent for pemphigus.

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Immunobullous Diseases

Schmidt,

Groves

2024

Rook's Textbook of Dermatology

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The immunobullous disorders represent a group of conditions characterised by antibody‐mediated autoimmune responses against structural elements of the skin resulting in blistering of the skin and/or mucosae. Antibody targets include proteins in the hemidesmosomes basement membrane zone and superficial dermis (pemphigoid group), desmosomes (pemphigus group) and epidermal and tissue‐type transglutaminase (dermatitis herpetiformis). If left untreated, immunobullous disorders are associated with significant morbidity and mortality and thus prompt, accurate diagnosis and treatment are mandatory. Therapeutic options include corticosteroids, steroid‐sparing immunosuppressants such as azathioprine, mycophenolate mofetil and anti‐inflammatory agents such as dapsone and tetracyclines. In severe and/or refractory patients, intravenous immunoglobulin, immunoadsorption and anti‐B‐cell agents such as rituximab may be of benefit. Indeed, recently international and European guidelines have recommended the use of rituximab as first line treatment in moderate and severe pemphigus vulgaris and foliaceous.

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Intraepithelial autoimmune bullous dermatoses disease activity assessment and therapy

Cited by 13 publications

References 141 publications

Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

Invasive breast cancer found in a patient with new-onset pemphigus foliaceus

Assessing the use of methotrexate as an alternate therapy for pemphigus vulgaris and pemphigus foliaceus

Immunobullous Diseases

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