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Indian Journal of Dermatology 2024
PubMed: 38841239
DOI: 10.4103/ijd.ijd_1101_23 -
Veterinary Dermatology Jun 2024Cytological detection of acantholytic keratinocytes (acantholytic cells [AC]) helps to identify canine pemphigus foliaceus (cPF) yet AC also occurs in superficial...
BACKGROUND
Cytological detection of acantholytic keratinocytes (acantholytic cells [AC]) helps to identify canine pemphigus foliaceus (cPF) yet AC also occurs in superficial pyoderma (SP), the main differential diagnosis.
HYPOTHESIS/OBJECTIVES
To compare selected cytomorphological features of cPF and SP and to establish cytological diagnostic criteria that could differentiate cPF from SP.
ANIMALS
40 and 51 client-owned dogs with PF and SP, respectively.
MATERIALS AND METHODS
Impression smears from cPF (64), impetigo (40) and exfoliative superficial pyoderma (ESP) (17) samples were stained with Romanowsky stain, randomised, blinded and evaluated by two investigators independently. The entire sample was screened (×500 or ×1000 magnification) for round (AC1), boat (AC2) and raft AC, eosinophils and bacteria. Interobserver agreements were calculated.
RESULTS
The average number of the 10 highest ×500 fields for AC1 and AC2 was significantly higher in PF than SP (p < 0.0001; Kruskal-Wallis test). Rafts and eosinophils were more common in PF than SP (p < 0.0001; chi-square test), while bacteria were rare in PF (5%; p < 0.0001; chi-square test). Observations between the experienced and novice investigators were highly correlated. An ROC analysis identified five AC1/×500-magnification field as a suitable cut-off value for predicting PF diagnosis. This cut-off value was tested by two additional investigators, who identified sensitivity of 84%-100%, specificity of 95%-97% and accuracy of 95%-96% for the diagnosis of cPF.
CONCLUSIONS AND CLINICAL RELEVANCE
Criterion-based impression smear cytological evaluation can provide strong evidence to support the clinical diagnosis. Acantholytic cell morphology varies in cPF and SP, and experience can improve accuracy in cytological differentiation.
PubMed: 38840462
DOI: 10.1111/vde.13267 -
BMJ Case Reports Jun 2024Paraneoplastic pemphigus (PNP) is a rare disease with an unclear mechanism of pathogenesis. We present a case of a male patient who presented with wound management after...
Paraneoplastic pemphigus (PNP) is a rare disease with an unclear mechanism of pathogenesis. We present a case of a male patient who presented with wound management after being diagnosed with Castleman disease-associated paraneoplastic pemphigus (PNP). The patient's condition was not improving; as a result, extensive workup was repeated, which confirmed the diagnosis of aggressive T cell lymphoblastic lymphoma. Our case signifies the importance of keeping a high index of suspicion for PNP-associated malignancies. This case report also adds emphasis to the diagnostic challenges faced by clinicians, making clinical correlation with multidisciplinary approach essential. Therefore, if clinically indicated, we need to revisit the diagnosis and seek alternative explanations to prevent delays in management.
Topics: Humans; Pemphigus; Male; Paraneoplastic Syndromes; Castleman Disease; Diagnosis, Differential; Middle Aged
PubMed: 38839409
DOI: 10.1136/bcr-2023-258580 -
Journal of the European Academy of... Jun 2024Autoimmune blistering diseases (AIBDs) are severe dermatologic disorders known for their debilitating physical impact. Recent research has reported that AIBDs lead to...
BACKGROUND
Autoimmune blistering diseases (AIBDs) are severe dermatologic disorders known for their debilitating physical impact. Recent research has reported that AIBDs lead to psychosocial impairment, including depression and anxiety. Missing from the extant literature is an examination of the impact of AIBDs on body image and related psychological constructs.
OBJECTIVES
The current study seeks to characterize the psychological and social consequences of AIBD diagnosis, with particular attention to body image dissatisfaction.
METHODS
We conducted a survey study of adults with AIBDs. The survey was open from February 2023 to March 2023. Validated self-report questionnaires assessed depressive symptomatology, body image disturbance and quality of life. Demographic information and self-reported psychiatric history before and after AIBD diagnosis were collected via self-report. Participants were 451 adults with AIBDs, recruited through the International Pemphigus and Pemphigoid Foundation newsletters, email distribution lists and social media.
RESULTS
Participants reported increased incidence of psychiatric disorders following AIBD diagnosis. Participants reported high levels of depressive symptomatology and impairments to quality of life compared to other patient groups. The sample reported extremely high levels of body image disturbance, more so than other patients with disfiguring diseases or injury. Correlation analyses revealed significant relationships between body image variables and quality of life, even after controlling for depression.
CONCLUSIONS
Current treatment guidelines for AIBDs focus primarily on the management of disease flares and the consequences of immunosuppression, without consideration of the psychosocial consequences of the disease. The current study underscores the need for mental health support for patients with AIBDs.
PubMed: 38837452
DOI: 10.1111/jdv.20156 -
Anais Brasileiros de Dermatologia Jun 2024
PubMed: 38834398
DOI: 10.1016/j.abd.2023.04.012 -
Acta Cytologica Jun 2024Pitfalls in Pap test could be defined as false positive, false negative or underdiagnosed results which can lead to unnecessary diagnostic procedures or delayed and... (Review)
Review
BACKGROUND
Pitfalls in Pap test could be defined as false positive, false negative or underdiagnosed results which can lead to unnecessary diagnostic procedures or delayed and inadequate treatment. It can be a consequence of misinterpretation of certain morphological entities which are described in this paper.
SUMMARY
the paper presents the overview of the morphological features and look-alikes of the common sources of pitfalls such as atrophy, repair, IUD change, tubal metaplasia, hyperchromatic crowded groups, and radiation changes. Rare causes of pitfalls such as Arias-Stella changes, pemphigus, tumor diathesis per se, rare types of cervical cancer, including verrucous and papillary squamous cell cancer, gastric type and endometrioid adenocarcinoma are also described.
KEY MESSAGES
The awareness of pitfalls in cervical cytology is important for cytopathologists and clinicians to avoid future errors. Review of Pap tests with erroneous diagnosis is important for quality control in cytology laboratory, and it must be considered as educational and experience building procedure. Cytopathologist should not pull back in significant diagnoses, especially in HPV negative cases.
PubMed: 38834045
DOI: 10.1159/000539637 -
The Veterinary Clinics of North... May 2024Autoimmune dermatopathies are not common in horses. These autoimmune diseases can be idiopathic or triggered by an antigen such as drugs, vaccines, or neoplasia. The... (Review)
Review
Autoimmune dermatopathies are not common in horses. These autoimmune diseases can be idiopathic or triggered by an antigen such as drugs, vaccines, or neoplasia. The most common one is pemphigus foliaceus, which manifests as a pustular, crusting eruption. Other more common pustular diseases should be ruled out before considering pemphigus. Vasculitis is relatively common in horses and can be triggered by a variety of antigenic stimulations. Systemic lupus and true idiopathic autoimmune vasculitis are very rare in horses. Every effort should be made to reach a final diagnosis, as the prognosis for true idiopathic autoimmune skin diseases is poor.
PubMed: 38821844
DOI: 10.1016/j.cveq.2024.04.001 -
Re-evaluating the prevalence of anti-desmocollin-1 IgA autoantibodies in canine pemphigus foliaceus.Veterinary Immunology and... Jul 2024Pemphigus foliaceus (PF) is an autoimmune skin disease of dogs characterized by intraepidermal pustules containing neutrophils and dissociated keratinocytes that develop...
Pemphigus foliaceus (PF) is an autoimmune skin disease of dogs characterized by intraepidermal pustules containing neutrophils and dissociated keratinocytes that develop in association with circulating and tissue-bound IgG autoantibodies. A subset of IgG autoantibodies in canine PF target desmocollin-1 (DSC1), a component of intercellular adhesion complexes within the epidermis. Passive transfer of IgG autoantibodies from canine PF sera to mice was previously shown to induce skin disease in the absence of infiltrating neutrophils. In attempts to identify a mechanism responsible for neutrophil recruitment, past studies evaluated the prevalence of IgA autoantibodies in canine PF sera where they were found in <20% of affected dogs. We re-evaluated the prevalence of anti-DSC1 IgA in canine PF due to concerns regarding the sensitivity of previously used methods. We hypothesized that anti-DSC1 IgA are present in most dogs with PF but have been under-detected due to competition with concurrent anti-DSC1 IgG for binding to their mutual antigenic target. Despite removing approximately 80% of IgG from patient sera using affinity chromatography, we did not detect an increase in anti-DSC1 IgA by performing indirect immunofluorescence on canine DSC1-transfected HEK293T cells. Taken together, our results do not support a role for pathogenic IgA in canine PF.
Topics: Dogs; Animals; Pemphigus; Desmocollins; Dog Diseases; Immunoglobulin A; Autoantibodies; Humans; HEK293 Cells; Immunoglobulin G; Fluorescent Antibody Technique, Indirect
PubMed: 38820947
DOI: 10.1016/j.vetimm.2024.110773 -
International Journal of Dermatology May 2024Pediatric pemphigus is a rare bullous disease that represents a diagnostic and therapeutic challenge; evidence on patients' response to various treatments and...
BACKGROUND
Pediatric pemphigus is a rare bullous disease that represents a diagnostic and therapeutic challenge; evidence on patients' response to various treatments and long-term surveillance data are lacking. We aimed to investigate pediatric pemphigus patients' characteristics, diagnosis, therapeutics, response, and long-term follow-up.
METHODS
This is a retrospective study of all pemphigus patients aged <18 years, diagnosed between 2000 and 2023, from three tertiary medical centers in Israel. The diagnosis was confirmed by positive immunofluorescence.
RESULTS
Twelve pediatric pemphigus patients were included (mean age 10.7 ± 4.3 years, male:female ratio 1:1). Mean diagnostic delay was 11.1 ± 12.6 months (range 1.8-36 months). Most patients had pemphigus vulgaris with mucosal involvement (58.3%). First-line treatment for all patients included systemic corticosteroids (sCS), with a treatment duration (including tapering down) of 28 ± 18.4 months. Hospitalization did not yield better outcomes. Only three patients achieved sustained complete response with sCS treatment (25.0%), and the rest required additional therapeutics, most commonly rituximab. Rituximab showed a good safety profile and therapeutic response. Follow-up was recorded up to 18.1 years after diagnosis (mean: 5.6 years). Three of five patients with information available more than 5 years after the pemphigus diagnosis still exhibited disease symptoms.
CONCLUSIONS
Pediatric pemphigus is associated with a significant diagnostic delay. While sCS can induce remission in most patients as a first-line treatment, long-term disease control requires additional immunomodulators. Long-term follow-up reveals a chronic yet mostly benign disease course in this population and advocates for the use of rituximab in pediatric pemphigus patients.
PubMed: 38819034
DOI: 10.1111/ijd.17251 -
Cureus Apr 2024Seborrheic pemphigus (SP) represents a localized and superficial form of pemphigus foliaceus (PF) often mistaken for other dermatological conditions such as seborrheic...
Seborrheic pemphigus (SP) represents a localized and superficial form of pemphigus foliaceus (PF) often mistaken for other dermatological conditions such as seborrheic dermatitis (SD) due to clinical similarities. Additionally, SP may be conceptually confused with pemphigus erythematosus (PE) due to historical terminology and overlapping clinical features. We present a case study of a 38-year-old female initially diagnosed with SD but later identified as SP through detailed clinical and histopathological analysis. We discuss the challenges in accurately diagnosing SP, emphasizing the importance of distinguishing it from PE and other acantholytic dermatoses. Furthermore, we highlight the effectiveness of topical treatment in managing SP, contrary to the systemic therapy often required for PE. Our findings underscore the necessity for further research to optimize management strategies for SP and emphasize the significance of precise terminology in clinical practice and research.
PubMed: 38817480
DOI: 10.7759/cureus.59389