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Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation.
Dermatol Ther (Heidelb)
January 2025
Department of Dermatology, University Hospital Heidelberg, Im Neuenheimer Feld 440, 69120, Heidelberg, Germany.
Pemphigus vulgaris is a severe and often therapy-resistant bullous autoimmune disease. Standard therapy with steroids often administered together with another immunosuppressant does not respond in all patients or may not be a good therapeutic option in patients with severe underlying diseases. Intravenous immunoglobulins (IVIgs) represent a treatment alternative, often showing a rapid response which allows one to reduce concomitant immunosuppression.
View Article and Find Full Text PDFPrognostic Factors Predicting Remission Following Rituximab Therapy for Pemphigus Vulgaris.
Acta Derm Venereol
January 2025
Department of Dermatology, Sheba Medical Center, Tel HaShomer, Ramat Gan, Israel.
Pemphigus vulgaris is a chronic autoimmune blistering disease with significant morbidity. Rituximab, approved as its first-line treatment, effectively induces remission. However, few studies have analysed the prognostic factors for improved rituximab outcomes.
View Article and Find Full Text PDFPemphigus Foliaceus Mimicking Ichthyosis: A Rare Clinical Presentation.
Cureus
November 2024
Dermatology, Amiri Hospital, Ministry of Health, Kuwait City, KWT.
Pemphigus foliaceus (PF) is an autoimmune blistering disease characterized by the disruption of the epidermal cell adhesion protein desmoglein 1 (DsG1). PF classically presents with superficial erosions or blisters, but can rarely mimic other dermatological conditions, which makes diagnosis challenging. We report the case of a 57-year-old Sri Lankan man with a one-month history of widespread ichthyosis-like plaques and scales which started on his scalp and progressed in a cranio-caudal fashion and were associated with pruritus and few blisters.
View Article and Find Full Text PDFPotential Pitfalls of IgG4 Immunohistochemical Staining on Lesional Tissue in Cutaneous Acantholytic Disorders.
Dermatopathology (Basel)
December 2024
Department of Pathology, University of Virginia, Charlottesville, VA 22903, USA.
The diagnostic utility of immunohistochemistry on paraffin-embedded sections in bullous disorders is useful when frozen tissue is not available. In pemphigus vulgaris and pemphigus foliaceus, an intercellular lace-like staining pattern of IgG4 on lesional tissue by immunohistochemistry has been described, with a comparable sensitivity and specificity to direct immunofluorescence on perilesional tissue. This study aimed to evaluate the staining pattern of IgG4 in non-immunobullous disorders to highlight the potential pitfalls when using this stain.
View Article and Find Full Text PDFClinical Characteristics, Comorbidities, and Treatment in Patients with Pemphigus-A Single-Center Retrospective Study.
Antibodies (Basel)
December 2024
Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, 61701 Poznan, Poland.
Background/objectives: Pemphigus comprises a diverse group of disorders within the autoimmune bullous dermatoses (AIBDs) spectrum. Among these, pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the most commonly encountered variants. Despite its rarity, this condition can pose a life-threatening risk.
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