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The American Journal of Dermatopathology Jul 2021Cutaneous reactions surrounding abdominal stoma sites are typically irritant, allergic, infectious, traumatic or pathergic in etiology. Pemphigus, which encompasses a... (Review)
Review
Cutaneous reactions surrounding abdominal stoma sites are typically irritant, allergic, infectious, traumatic or pathergic in etiology. Pemphigus, which encompasses a group of vesiculobullous autoimmune skin disorders, is seldom encountered as a peristomal dermatosis. Direct immunofluorescence (DIF) studies of pemphigus generally show continuous intercellular net-like depositions of IgG. However, punctate or dot-like intercellular deposition of IgG can also be seen in cases of pemphigus. The punctuate pattern is underreported in the literature and little is known about its implication. We describe a case of a 58-year-old Caucasian man with a history of bowel obstruction, status postcolostomy, who presented with a sharply demarcated, erythematous, crusted plaque surrounding his abdominal stoma. The patient endorsed persistent pruritus. A punch biopsy of the lesion was performed for clinical suspicion of fungal infection versus irritant dermatitis. Histopathology revealed a predominantly subcorneal acantholytic dermatitis. Periodic acid-Schiff with diastase and Grocott methenamine silver histochemical stains were negative for fungi. DIF was positive for IgG and C3 detected in a punctate intercellular pattern. In conjunction with the patient's clinical presentation and DIF, a diagnosis of peristomal pemphigus foliaceous was rendered. Herein, we describe a case of punctate pemphigus presenting as a peristomal dermatosis and include a review of the literature to raise awareness of this phenomenon.
Topics: Acantholysis; Aged; Biopsy; Colostomy; Fluorescent Antibody Technique, Direct; Humans; Male; Pemphigus; Skin
PubMed: 33156025
DOI: 10.1097/DAD.0000000000001845 -
The Journal of Dermatology Mar 2017Acantholysis is a commonly encountered histological pattern which typically generates a differential of the pemphigus variants, Hailey-Hailey, Darier's and Grover's... (Review)
Review
Acantholysis is a commonly encountered histological pattern which typically generates a differential of the pemphigus variants, Hailey-Hailey, Darier's and Grover's diseases. In addition to these diseases, the dermatologist and dermatopathologist must be aware of entities that mimic classic acantholytic dermatoses and of rare disease variants, which are characterized by acantholysis.
Topics: Acantholysis; Diagnosis, Differential; Humans
PubMed: 28256765
DOI: 10.1111/1346-8138.13769 -
Current Problems in Dermatology 2016Paraneoplastic itch occurs as the result of a systemic reaction to an underlying malignancy. Paraneoplastic itch is most commonly associated with lymphoproliferative... (Review)
Review
Paraneoplastic itch occurs as the result of a systemic reaction to an underlying malignancy. Paraneoplastic itch is most commonly associated with lymphoproliferative malignancies and solid tumors that result in cholestasis. Paraneoplastic itch may occur in the absence of a primary rash or in association with dermatologic conditions such as erythroderma, acanthosis nigricans, dermatomyositis, Grover's disease, and eruptive seborrheic keratosis. Treatment of paraneoplastic itch is centered on targeting the underlying malignancy responsible for the systemic reaction. In cases of malignancy that are refractive to treatment, other therapies have been found to be effective for paraneoplastic itch, including selective serotonin reuptake inhibitors, mirtazapine, gabapentin, thalidomide, opioids, aprepitant, and histone deacetylase inhibitors.
Topics: Acantholysis; Acanthosis Nigricans; Analgesics, Opioid; Anticonvulsants; Antidepressive Agents; Aprepitant; Dermatitis, Exfoliative; Dermatomyositis; Histone Deacetylase Inhibitors; Humans; Ichthyosis; Immunosuppressive Agents; Keratosis, Seborrheic; Mianserin; Mirtazapine; Morpholines; Neoplasms; Neurokinin-1 Receptor Antagonists; Paraneoplastic Syndromes; Pruritus; Selective Serotonin Reuptake Inhibitors; Thalidomide
PubMed: 27578084
DOI: 10.1159/000446060 -
Clinical and Experimental Dermatology May 2022We report the case of a 61-year-old woman presenting with a subacute eruption of a florid, circinate eruption with peripheral pustules. Histological findings included a...
We report the case of a 61-year-old woman presenting with a subacute eruption of a florid, circinate eruption with peripheral pustules. Histological findings included a neutrophilic intraepidermal pustule with associated acantholysis, and a mixed inflammatory cell infiltrate within the dermis.
Topics: Diagnosis, Differential; Exanthema; Female; Humans; Middle Aged
PubMed: 35106790
DOI: 10.1111/ced.15077 -
Eye & Contact Lens Mar 2016To describe the epidemiology, etiology, pathogenesis, clinical characteristics, and management of pemphigus, with an emphasis on ocular involvement. (Review)
Review
OBJECTIVE
To describe the epidemiology, etiology, pathogenesis, clinical characteristics, and management of pemphigus, with an emphasis on ocular involvement.
METHODS
Literature review.
RESULTS
Pemphigus is an autoimmune epithelial blistering disease of the skin and mucous membranes. The typical pathological finding is acantholysis of the epidermis that leads to blister formation. Immunofluorescence techniques show autoantibody deposition on the epidermal intercellular substance. Although a genetic background is necessary, environmental factors are crucial for the onset and perpetuation of the disease. Exposure to some drugs, toxic agents, and foods and associations with other autoimmune diseases and lymphoproliferative conditions should be assessed. Generally, the skin is the most commonly affected tissue. Ocular involvement might be present and exhibit a clinical course that is independent of skin compromise. Visual function may be affected depending on the severity of the presentation. In untreated cases, mortality is high because of bacterial sepsis and hydroelectrolyte imbalance. A multidisciplinary approach should be used involving a dermatologist, ophthalmologist, and immunologist. Immunosuppressive agents are the mainstay of treatment; corticosteroids typically with azathioprine or mycophenolate mofetil are the drugs of choice. Surgical treatment of trichiasis and malposition of the eyelids and tectonic procedures for corneal perforation are sometimes required in very severe and recalcitrant cases.
CONCLUSIONS
Pemphigus is a potential life- and sight-threatening disease. Understanding the disease facilitates the adequate assessment of the modifiable factors and the prompt initiation of immunotherapy. Ocular involvement can develop in patients with pemphigus. Adequate ophthalmological care is needed, in particular, prevention of infections, scarring, and corneal perforation.
Topics: Adrenal Cortex Hormones; Conjunctival Diseases; Eyelid Diseases; Humans; Immunosuppressive Agents; Pemphigus; Risk Factors; Vision Disorders
PubMed: 26657660
DOI: 10.1097/ICL.0000000000000155 -
Frontiers in Immunology 2019Pemphigus encompasses a heterogeneous group of autoimmune blistering diseases, which affect both mucous membranes and the skin. The disease usually runs a... (Review)
Review
Pemphigus encompasses a heterogeneous group of autoimmune blistering diseases, which affect both mucous membranes and the skin. The disease usually runs a chronic-relapsing course, with a potentially devastating impact on the patients' quality of life. Pemphigus pathogenesis is related to IgG autoantibodies targeting various adhesion molecules in the epidermis, including desmoglein (Dsg) 1 and 3, major components of desmosomes. The pathogenic relevance of such autoantibodies has been largely demonstrated experimentally. IgG autoantibody binding to Dsg results in loss of epidermal keratinocyte adhesion, a phenomenon referred to as acantholysis. This in turn causes intra-epidermal blistering and the clinical appearance of flaccid blisters and erosions at involved sites. Since the advent of glucocorticoids, the overall prognosis of pemphigus has largely improved. However, mortality persists elevated, since long-term use of high dose corticosteroids and adjuvant steroid-sparing immunosuppressants portend a high risk of serious adverse events, especially infections. Recently, rituximab, a chimeric anti CD20 monoclonal antibody which induces B-cell depletion, has been shown to improve patients' survival, as early rituximab use results in higher disease remission rates, long term clinical response and faster prednisone tapering compared to conventional immunosuppressive therapies, leading to its approval as a first line therapy in pemphigus. Other anti B-cell therapies targeting B-cell receptor or downstream molecules are currently tried in clinical studies. More intriguingly, a preliminary study in a preclinical mouse model of pemphigus has shown promise regarding future therapeutic application of Chimeric Autoantibody Receptor T-cells engineered using Dsg domains to selectively target autoreactive B-cells. Conversely, previous studies from our group have demonstrated that B-cell depletion in pemphigus resulted in secondary impairment of T-cell function; this may account for the observed long-term remission following B-cell recovery in rituximab treated patients. Likewise, our data support the critical role of Dsg-specific T-cell clones in orchestrating the inflammatory response and B-cell activation in pemphigus. Monitoring autoreactive T-cells in patients may indeed provide further information on the role of these cells, and would be the starting point for designating therapies aimed at restoring the lost immune tolerance against Dsg. The present review focuses on current advances, unmet challenges and future perspectives of pemphigus management.
Topics: Adoptive Transfer; Adrenal Cortex Hormones; Animals; Autoantibodies; Cell Adhesion; Desmoglein 1; Desmoglein 3; Disease Models, Animal; Epidermis; Humans; Immunoglobulin G; Keratinocytes; Mice; Pemphigus; Rituximab
PubMed: 31293582
DOI: 10.3389/fimmu.2019.01418 -
Journal of the European Academy of... Sep 2019Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub-epidermal blisters (SEB) with circulating autoantibodies against components of the basement... (Review)
Review
Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub-epidermal blisters (SEB) with circulating autoantibodies against components of the basement membrane zone (BMZ). The main disorders to date include bullous pemphigoid (BP), pemphigoid gestationis, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LABD), dermatitis herpetiformis (DH), lichen planus pemphigoides and bullous lupus. This is in contrast to pemphigus and related disorders, which demonstrate intraepidermal acantholysis and a positive Nikolsky sign. The classification and management is based on clinical, histological and direct and indirect immunofluorescence findings. There are, however, overlapping clinical and histological features between the conditions and clinical heterogeneity within each disease.
Topics: Autoimmune Diseases; Humans; Skin Diseases, Vesiculobullous
PubMed: 31087464
DOI: 10.1111/jdv.15679 -
Human Pathology Oct 2022Autoimmune bullous dermatoses are characterized by the presence of tissue-bound and often circulating pathogenic autoantibodies targeting structural components of the... (Review)
Review
Autoimmune bullous dermatoses are characterized by the presence of tissue-bound and often circulating pathogenic autoantibodies targeting structural components of the skin and/or mucous membranes. The diagnostic workup for this heterogeneous group of disorders consists of a multi-step process, of which the light microscopic examination is a crucial component. This review is organized following a classification scheme that is based on two main histopathologic features, namely level of intraepithelial split and composition of the inflammatory infiltrate. Overall, we aim to place emphasis on the histopathologic clues that can assist pathologists in differential diagnosis and review the updates in the literature.
Topics: Autoantibodies; Autoimmune Diseases; Diagnosis, Differential; Humans; Skin; Skin Diseases, Vesiculobullous
PubMed: 35764145
DOI: 10.1016/j.humpath.2022.06.021 -
Indian Journal of Dermatology,... 2022
Review
Topics: Acantholysis; Diagnosis, Differential; Humans; Ichthyosis; Skin; Skin Diseases
PubMed: 34245536
DOI: 10.25259/IJDVL_1028_20 -
The Australasian Journal of Dermatology Feb 2021
Topics: Acantholysis; Betamethasone; Calcitriol; Female; Humans; Keratosis; Middle Aged; Skin Diseases, Papulosquamous; Treatment Outcome
PubMed: 32725622
DOI: 10.1111/ajd.13404