Sacral Dimple, Conjunctiva, and Nipple as Less Obvious Pemphigus Vulgaris Locations around Natural Body Orifices: A Report of Three Cases

Jałowska, Magdalena; Gornowicz‐Porowska, Justyna; Bowszyc‐Dmochowska, Monika; Dmochowski, Marian

doi:10.3390/jcm11102855

Cited by 8 publications

(12 citation statements)

References 47 publications

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“…Children above 1 year were covered in blisters mostly located on mucous membranes. In childhood pemphigoid lesions have a tendency to localize around natural body orifices similar to pemphigus [29].…”

Section: Discussionmentioning

confidence: 99%

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Pemphigoid diseases in infancy and childhood. A review of the literature

Jałowska,

Dmochowski

2023

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Bullous pemphigoid is an autoimmune blistering disease that very rarely affects children. Clinical differences in locations of skin lesions led to the distinction of infantile versus childhood pemphigoid. Mucous membranes can be affected. The first-line therapy usually consists of topical or/and systemic glucocorticosteroids. Intravenous immunoglobulin infusions can be a valuable treatment option. Prognosis of infantile pemphigoid and childhood pemphigoid is good and the duration is often limited to one year with adequate treatment. The differential diagnosis of blisters should include: linear immunoglobulin A bullous disease of childhood, epidermolysis bullosa acquisita, bullous systemic lupus erythematosus, bullous scabies and childhood infectious diseases with blisters.

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Section: Discussionmentioning

confidence: 99%

“…U dzieci powyżej 1. roku życia obecne były pęcherze zlokalizowane głównie na błonie śluzowej. W dzieciństwie zmiany pemfigoidowe mają tendencję do lokalizowania się wokół naturalnych otworów ciała, podobnie jak w pęcherzycy [29].…”

Section: Conflict Of Interestunclassified

Pemphigoid diseases in infancy and childhood. A review of the literature

Jałowska,

Dmochowski

2023

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“…The hallmark feature of pemphigus is the immune system's predominant attack on desmoglein (DSG) proteins, primarily DSG-3, DSG-1, or both DSG-3/DSG-1. However, it is important to note that other antibodies may also play a role in immune response [2,3].…”

Section: Introductionmentioning

confidence: 99%

Rituximab in the Management of Autoimmune Bullous Diseases: A Treatment-Resistant Case Series from a Single Central European Referral Center

Spałek,

Jałowska,

Bowszyc-Dmochowska

et al. 2024

Medicina

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Background and Objectives: Rituximab (RTX) has been the predominant treatment for autoimmune bullous diseases (AIBDs). The objective of this research was to assess the advantages and safety characteristics of RTX treatment in individuals with AIBD. This assessment focused on clinical remission and a reduction in glucocorticosteroid usage, its effect on the titers of autoantibodies targeting desmoglein-1 (DSG-1) and desmoglein-3 (DSG-3), and adverse occurrences during a 12-month follow-up period in a dermatology department within a Central European university context. Materials and Methods: Our case series involved eleven patients, including eight patients with pemphigus vulgaris, two with pemphigus foliaceus, and one with epidermolysis bullosa acquisita. They received a 1 g dose of rituximab, repeated over a two-week interval. Results: The reduction in a prednisone-equivalent dosage after 2, 6, and 12 months following the second RTX infusion was 65.05%, 73.99%, and 76.93%, in that order. The titers of antibodies against DSG-1 exhibited reductions of 43.29%, 75.86%, and 54.02% at 2, 6, and 12 months, respectively. By contrast, the antibody concentrations targeting DSG-3 displayed a decrease of 27.88%, 14.48%, and 5.09% at the corresponding time points. Over the course of the 12-month monitoring period, 18.18% of patients experienced disease relapse, while the remaining individuals achieved either complete or partial remission with minimal or no therapy. Adverse effects were noted in 36.36% of the patient population; they were mild, and no serious adverse effects were reported. Conclusions: RTX represents an efficacious and well-tolerated therapeutic option for the management of AIBD and merits consideration in cases of refractory AIBD. However, further research is imperative to delineate the most optimal dosage, dosing frequency, and total quantity of maintenance infusions required. Additionally, there is a compelling need for studies that explore the impact of RTX on individuals with AIBD who do not exhibit a significant reduction in anti-desmoglein autoantibody levels.

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“…Pemphigus is a heterogeneous group of autoimmune acantholytic illnesses expressing possibly misleading clinical peculiarities and running a potentially lethal course [ 1 ]. In humans there are seven desmosomal cadherins, three desmocollins (DSC1–3) and four desmogleins (DSG1–4).…”

Section: Introductionmentioning

confidence: 99%

Treatment-naïve pemphigus patients showing IgG tissue deposits have serum IgG antibodies against desmoglein 1 detected more often than those against desmoglein 3: a monocentre laboratory experience

Jałowska¹,

Dmochowski²,

Gornowicz‐Porowska³

et al. 2023

pdia

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Introduction Pemphigus is a heterogeneous group of autoimmune acantholytic diseases. Aim To check whether there is a relationship between detecting IgG deposits in the direct immunofluorescence (DIF) and finding IgG antibodies against particular desmoglein (DSG) isoforms in ELISA techniques in patients with pemphigus. Material and methods Single-step DIF for revealing the deposits of IgA, IgM, IgG, IgG1, IgG4 and C3, and monoanalyte ELISAs or the multiplex ELISA were used for diagnosis. The Z test for two independent proportions was used for the statistical analysis. Results We evaluated 19 consecutive treatment-naïve pemphigus patients, who exhibited IgG deposits, accompanied by other types of immunoreactants in various combinations, in DIF. Serum IgG antibodies against DSG1 were detected in 18 patients, whereas serum IgG antibodies against DSG3 were found in 10 patients. The statistical analysis showed that the proportion of anti-DSG1 antibody-positive individuals (18 of 19, 94.74%) was statistically significantly higher than the proportion of anti-DSG3 antibody-positive ones (10 of 19, 52.63%) ( p = 0.0099). Conclusions IgG deposition in the pemphigus pattern seems to be related to the presence of serum IgG antibodies against DSG1 rather than against DSG3. DSG1 may bind IgG more efficiently than DSG3 since DSG1 has a longer cytoplasmic region compared to that of DSG3.

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Sacral Dimple, Conjunctiva, and Nipple as Less Obvious Pemphigus Vulgaris Locations around Natural Body Orifices: A Report of Three Cases

Cited by 8 publications

References 47 publications

Pemphigoid diseases in infancy and childhood. A review of the literature

Pemphigoid diseases in infancy and childhood. A review of the literature

Rituximab in the Management of Autoimmune Bullous Diseases: A Treatment-Resistant Case Series from a Single Central European Referral Center

Treatment-naïve pemphigus patients showing IgG tissue deposits have serum IgG antibodies against desmoglein 1 detected more often than those against desmoglein 3: a monocentre laboratory experience

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