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Dermatological manifestations of hematologic neoplasms. Part II: nonspecific skin lesions/paraneoplastic diseases.

Cutaneous manifestations occur in the course of hematologic malignancies and precede, accompany or occur late in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, immunosuppression resulting from the hematologic disease itself or its treatment. The dermatologist must be aware of these conditions that may be helpful both in the diagnosis of the underlying disease and in reducing patient morbidity. This review (part II) addresses the paraneoplastic dermatological changes associated with systemic hematologic malignancies.

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… In many stomatitis lesions, only inflammation, necrosis and suprabasal acantholysis are found in the perilesional mucosa. Intact bullae show suprabasal acantholysis; however, unlike other types of pemphigus, there is significant inflammatory infiltrate even in early lesions. … membrane. 66 Anhalt proposed a redefinition of the original diagnostic criteria in 2004, where he considers as the minimum criteria for PNP diagnoses with a high degree of confidence: (1) Erosive and progressive stomatitis, with preferential involvement of the tongue; (2) Histopathological findings of acantholysis …

Galli-Galli Disease Presenting as a Lentigo-like Eruption: A Further Clinical Feature in the Wide Spectrum of Reticulate Pigment Disorders.

Dear Editor, Reticulate pigmentary disorders (RPD) is a term used to classify a spectrum of several acquired and congenital disorders. Diffe … performed. Histologic examination showed a digitate elongations of rete ridges, with small foci of acantholysis (Figure 2, a). The epidermis showed a finger-like projections extending into the papillary dermis with increased melanin pigment. The epidermis was atrophic above the digitate proliferations and above the acantholytic foci, where necrotic and dysker …

SARS-CoV-2 Infection in Pemphigus Vulgaris Two Weeks after Rituximab Therapy with Total Recovery: A Case Report.

The mortality risk factors for Corona Virus Disease-19 (COVID-19) infection (caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2)) include advanced age, male sex, certain comorbidities, and immunosuppression (1). Pemphigus vulgaris is a rare mucocutaneous autoimmune disease with autoantibodies against desmosomal desmoglein-1 and desmoglein-3, resulting in acantholysis and blister formation. This epithelial barrier defect increases susceptibility to infections, which may lead to relapses (2). Additionally, therapy-associated immunosuppression can lead to severe infections.…

Dowling-Degos Disease in the Anogenital Region.

Dowling-Degos disease (DDD) is a benign, rare genodermatosis (reticulate pigmented anomaly) of flexure sites with autosomal dominant inherit … entical clinical presentation, the only difference between those two entities being the presence of acantholysis on biopsy in GGD (9). RAPK presents with hyperpigmentation on the dorsum on the hands and feet, and that pattern has been observed in some patients with DDD as well as GGD (6,7,10-12). However, it differs from DDD in the presence of palmar and plant …

Integumentary System

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… Acantholysis is the hallmark of the pemphigus complex. It can also result from proteolytic enzymes released by neutrophils or eosinophils in an inflammatory process. In domestic animals, acantholysis is most commonly manifested within pustules and crusts of pemphigus foliaceus. … Figure 6-1 Acantholysis in pemphigus foliaceus. Loss of cohesion between keratinocytes leads to individualization of cells. Note that the acantholytic cells have normal nuclear morphology. Figure 6-1 Acantholysis in pemphigus foliaceus. …

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review.

Haematological malignancies induce important alterations of the immune system, which account for the high frequency of autoimmune complications observed in patients. Cutaneous immune-mediated diseases associated with haematological malignancies encompass a heterogeneous group of dermatoses, including, among others, neutrophilic and eosinophilic dermatoses, autoantibody-mediated skin diseases, vasculitis and granulomatous dermatoses. Some of these diseases, such as paraneoplastic pemphigus, are associated with an increased risk of death; others, such as eosinophilic dermatoses of haematological

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… Besides acantholysis, PNP shows a lichenoid lymphocytic infiltrate along the dermal–epidermal junction by histopathology [ 136 , 137 ]. … Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. J Clin Invest. 1998;102:775–782. doi: 10.1172/JCI3647. 143. …

Clinical Characteristics, Comorbidities, and Treatment in Patients with Pemphigus-A Single-Center Retrospective Study.

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. We aimed to assess clinical characteristics, comorbidities, medication, as well as the treatment of various types of pemphigus in pemphigus patients. METHODS: We gathered data from 69 patients treated in the Department of Dermatology in the years 2016-2023. The investigation included sex,

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… In these areas, acantholysis leads to the formation of blisters located above the basal layer, typically filled with clear fluid. These blisters rupture easily, resulting in erosions that usually heal without scarring. …

Severe Relapsing Hailey-Hailey Disease Displaying a Durable Complete Response to Hydroxyurea.

Familial benign chronic pemphigus, also known as Hailey-Hailey disease, was first described by the Hailey brothers in 1939 (1). It represent … ecessary protein to be synthesized. Consequently, impaired keratinocyte adhesion ensues, leading to acantholysis, blisters, and rash (2). The initial lesion may be an erythematous area or a fluid-filled blister which subsequently ruptures, leading to a macerated or crusted lesion. The lesions more commonly affect the sides of the neck, armpits, forearms, butto …

Darier Disease Presenting with Recurrent Kaposi Varicelliform Eruption in a 10-year-old Boy with Seborrheic Dermatitis.

We present a case of a 10-year-old boy with a longstanding history of seborrheic dermatitis (SD) referred to the Allergy and Immunology Depa … confirmed based on medical history, clinical findings and histological finding of focal suprabasal acantholysis and dyskeratosis (Figure 3). Low-dose oral retinoid therapy was initiated with modest clinical response after 6 months of therapy. In the light of recent publication (1), we initiated intravenous immunoglobulin (IVIG) substitution (400 mg/kg every m …

Severe cutaneous adverse reactions induced by targeted anticancer therapies and immunotherapies.

With the increasing use of targeted anticancer drugs and immunotherapies, there have been a substantial number of reports concerning life-threatening severe cutaneous adverse reactions (SCARs), including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), drug rash with eosinophilia and systemic symptoms, drug-induced hypersensitivity syndrome, and acute generalized exanthematous pustulosis. Although the potential risks and characteristics for targeted anticancer agent- and immunotherapy-induced SCAR were not well understood, these serious adverse reactions usually result in morb

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… Typical histopathological features with suprabasal acantholysis, lichenoid interface dermatitis, positive DIF/IIF, and immunoblotting recognition of envoplakin and/or periplakin can support a diagnosis of PNP. 61 , 62 In cases of suspicious rituximab SJS/TEN, DIF or IIF has been suggested to exclude …

Diabetes mellitus and the skin.

Several dermatoses are routinely associated with diabetes mellitus, especially in patients with chronic disease. This relationship can be easily proven in some skin disorders, but it is not so clear in others. Dermatoses such necrobiosis lipoidica, granuloma annulare, acanthosis nigricans and others are discussed in this text, with an emphasis on proven link with the diabetes or not, disease identification and treatment strategy used to control those dermatoses and diabetes.

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… The most common type shows an subepidermal cleavage at the level of the lamina lucida without acantholysis, which appear and disappear spontaneously, without scarring. 8 , 14 Blisters present hyaline content and are located at the tips of the toes and less frequently in the dorsal surfaces of the feet …

Neutrophilic and eosinophilic dermatoses associated with hematological malignancy.

Cutaneous manifestations of hematologic malignancy represent both a clinical challenge for the treating physician and a pathophysiological model for advancing the knowledge on individual neoplasms. Indeed, a growing body of evidence supports the concept of recurrent molecular defects associating with specific clinical features, as best exemplified by VEXAS. Herein neutrophilic and eosinophilic dermatoses of potential interest for both hematologists and dermatologists will be reviewed, including subcorneal pustular dermatosis-type IgA pemphigus, neutrophilic eccrine hidradenitis, Sweet's syndro

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… Histologically, it shows subcorneal acantholysis, pustules with intercellular IgA deposits in the upper epidermis and predominant neutrophil infiltration ( 9 ). True SPD differs from it for the negativity of immunofluorescence studies ( 9 ). …

Oral and Maxillo-Facial Manifestations of Systemic Diseases: An Overview.

Many systemic (infective, genetic, autoimmune, neoplastic) diseases may involve the oral cavity and, more generally, the soft and hard tissues of the head and neck as primary or secondary localization. Primary onset in the oral cavity of both pediatric and adult diseases usually represents a true challenge for clinicians; their precocious detection is often difficult and requires a wide knowledge but surely results in the early diagnosis and therapy onset with an overall better prognosis and clinical outcomes. In the current paper, as for the topic of the current Special Issue, the authors pre

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… autoimmune diseases caused by autoantibodies targeting specific desmosomal proteins on the surface of epithelial cells (in pemphigus vulgaris are typically linked to desmoglein-3 and rarely, in 50% of cases, also to desmoglein-1, the latter an exclusive target antigen in pemphigus foliaceus) with subsequent acantholysis …

Bruton's tyrosine kinase inhibition-An emerging therapeutic strategy in immune-mediated dermatological conditions.

Bruton's tyrosine kinase (BTK), a member of the Tec kinase family, is critically involved in a range of immunological pathways. The clinical application of BTK inhibitors for B-cell malignancies has proven successful, and there is strong rationale for the potential benefits of BTK inhibitors in some autoimmune and allergic conditions, including immune-mediated dermatological diseases. However, the established risk-to-benefit profile of "first-generation" BTK inhibitors cannot be extrapolated to these emerging, non-oncological, indications. "Next-generation" BTK inhibitors such as remibrutinib

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… In particular, they may help to address the suboptimal levels of response to currently available treatments in type IIb CSU. 52 , 53 , 55 Pemphigus Pemphigus is an autoantibody‐driven skin disease characterized by a loss of cell adhesion (acantholysis), leading to blisters or erosions of the skin and …

Chronic pruritus: a narrative review.

Chronic pruritus encompasses a manifestation of several cutaneous, allergic, infectious, neurological, psychological, and systemic conditions, whose etiological investigation and therapeutic strategy can be challenging. This comprehensive review aims to enhance the understanding of pruritus by highlighting important elements in its pathogenesis, including keratinocytes, Merkel cells and mast cells, nerve fibers, histaminergic and nonhistaminergic pathways, and the interaction of itch signals with the central nervous system. Diagnostic evaluation of chronic pruritus may require a meticulous app

Purinergic Signalling: Therapeutic Developments.

Purinergic signalling, i.e., the role of nucleotides as extracellular signalling molecules, was proposed in 1972. However, this concept was not well accepted until the early 1990's when receptor subtypes for purines and pyrimidines were cloned and characterised, which includes four subtypes of the P1 (adenosine) receptor, seven subtypes of P2X ion channel receptors and 8 subtypes of the P2Y G protein-coupled receptor. Early studies were largely concerned with the physiology, pharmacology and biochemistry of purinergic signalling. More recently, the focus has been on the pathophysiology and the

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Disorders of the Gastrointestinal System

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Резюме: 32 Второй Конгресс ЭСП и Международный конгресс XXXIII МПД.

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33rd Европейский конгресс патологии - резюме.

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