Subcorneal Pustular Dermatosis Research Articles

Case report: segmental form of Hailey-Hailey disease

The article presents a clinical case of a rare segmental form of familial benign chronic pemphigus with a positive family history. Patient S., born in 2014, complained of unilateral eruptions in the area of the left retroauricular fold, left axilla, inguinal fold and labia majora on the left, plantar surface of the left foot. Eruptions were represented by erythematous foci, single blisters with serous contents, erosions, crusts. For the entire period, the patient was observed with the following diagnoses: microbial eczema, Sneddon-Wilkinson Disease, Inflammatory Linear Verrucose Epidermal Nevus. Due to the long-term relapsing course and ineffective therapy, additional examination methods were carried out – histological and genetic studies. Only after genetic testing, in which a heterozygous mutation of the ATP2C1 gene was revealed, and geneticist’s consultation, the final diagnosis was established – Hailey-Hailey Disease.It is known, that the segmental form is formed as a result of mosaicism, which can be of two types. The term "mosaicism" refers to the presence in an organism of two or more genetically heterogeneous cell populations formed from a genetically homogeneous zygote. The formation of type 1 mosaicism is based on a de novo postzygotic mutation at an early stage of embryogenesis. In turn, type 2 mosaicism develops as a result of a postzygotic mutation against the background of an already existing prezygotic mutation. Determining the type of mosaicism by genetic testing will make it possible to assess the prognosis of the disease and the chance of transmitting this disease to offspring.

Genetic analysis of different subtypes of aseptic pustulosis in the Chinese population.

Aseptic pustulosis involves inflammatory skin conditions with nonbacterial pustules on erythema, accompanied by neutrophil and eosinophil infiltration in the epidermis. Dysregulation of the interleukin (IL)-36 pathway leads to neutrophil aggregation and pustule formation. Variants in IL36RN, CARD14, AP1S3, MPO, SERPINA3 and BTN3A3 have been identified in generalized pustular psoriasis (GPP) in the past. Some patients with acrodermatitis continua of Hallopeau (ACH), palmoplantar pustulosis and acute generalized exanthematous pustulosis (AGEP) also exhibit mutations in IL36RN, CARD14 and AP1S3, albeit with regional and population-specific variations. This study aims to explore a shared genetic foundation among those with aseptic pustulosis. We performed Sanger sequencing on six genes in 126 patients with aseptic pustulosis. Genetic analysis identified IL36RN variants strongly associated with ACH, AGEP and subcorneal pustular dermatosis (SPD). Immunohistochemistry revealed elevated inflammatory cytokines in all subtypes. This study establishes a significant association between IL36RN variants and ACH, AGEP and SPD, emphasizing the IL-1/IL-36-chemokine-neutrophil axis as a common pathogenic mechanism. Targeting this axis holds promise for therapeutic interventions for aseptic pustulosis.

One patient, two dermatosis

The coexistence of psoriasis and bullous pemphigoid is well-documented in the literature. However, the association of psoriasis with IgA pemphigus, is rarely reported in the literature. To the best of our knowledge this is the second case describing the association of pustular psoriasis and IgA pemphigus.

Diagnostic Utility of Direct Immunofluorescence on Paraffin-Embedded Skin Biopsy Samples for the Diagnosis of Autoimmune Vesiculobullous Lesions.

Background Autoimmune vesiculobullous diseases (AIBDs) are a group of diseases characterized by blisters of the skin/mucosa due to the presence of circulating autoantibodies against antigens in the epidermis or the dermo-epidermal junction. Direct immunofluorescence (DIF) for immunoglobulin (Ig)G, IgC3, and IgA on fresh-frozen tissue is the gold standard diagnostic test for AIBDs. However, DIF in the absence of frozen tissue is challenging for the diagnosis of AIBDs. This study aimed to analyze the practical utility of DIF using paraffin-embedded skin biopsy rather than fresh frozen tissue for the diagnosis of AIBDs. Methodology This cross-sectional comparative study included 30 cases of AIBDs. DIF for IgG and IgA was performed on paraffin-embedded tissue (PE-DIF) after proteinase digestion on histopathologically confirmed 15 pemphigus vulgaris (PV), three pemphigus foliaceous (PF), four bullous pemphigoid (BP), three dermatitis herpetiformis (DH), three subcorneal pustular dermatosis (SCPD), and one case each of linear IgA disease and pemphigoid gestationis (PG). PE-DIF staining pattern was compared with the DIF on fresh frozen tissue (FF-DIF). Results All cases of PV and PF showed an intercellular IgG chicken wire staining pattern similar to FF-DIF. However, background staining was more intense in PV cases while less intense in PF cases. Three BP cases showed linear IgG staining in PE-DIF. DH, SCPD, linear IgA disease, and PG cases did not show IgG positivity. Out of three DH cases, two cases showed granular IgA positivity while linear IgA positivity along the basement membrane was seen in a single case of linear IgA disease. Negative IgG staining was observed in SCPD. Immunofluorescence in PE-DIF was rapidly deteriorating than in FF-DIF. Conclusions DIF done on paraffin-embedded tissue can be used as a supplement and salvage technique with histopathology for the diagnosis of AIBDs,particularly when a cryostat facility for frozen tissue is not available and the patient is unable to undergo a second biopsy procedure.

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 syndrome as well as myelodysplasia cutis and VEXAS, pyoderma gangrenosum, eosinophilic annular erythema, eosinophilic dermatosis of hematological malignancy, Wells syndrome and cutaneous involvement in hypereosinophilic syndromes. Possible management approaches are discussed for each, emphasizing scenarios that require treatment of the underlying condition to achieve remission at the skin level.

Subcorneal pustular dermatosis: Clinical characteristics and long-term follow-up of seventeen patients

Background/Aim. Subcorneal pustular dermatosis (SPD) is a rare, relapsing vesiculopustular disease predominantly presenting on the flexor surfaces. Since data about the course and duration of the disease is limited, the aim of the study was to analyze the clinical features and long-term follow-up data on patients with SPD. Methods. A hospital database was searched for medical records of patients with SPD hospitalized at the institution between 1985 and 2014. The focus was on clinical characteristics, associated diseases, disease courses, and outcomes. Results. Seventeen patients with clinical features of SPD were analyzed ? nine females and eight males with a median age at presentation of 45 years (range 18?90 years). Follow-up data were available for 12 patients. The median follow-up time was 9.5 years (1?28 years). In order to establish a histological diagnosis, repeated biopsies were required (on average 1.7 biopsies per patient). In one male patient, IgA pemphigus was diagnosed by direct immunofluorescence. In most patients, skin eruption was widespread, while in a smaller number of patients, the changes were present only on the flexor surfaces. Dysproteinemia was evident in three patients. The disease was self-limiting in three patients; five patients had mild flare-ups occurring 1?3 times a year without the need for treatment; four patients had continuous flare-ups requiring treatment. Most patients with SPD responded positively to dapsone. Conclusion. SPD is a rare disease, and it usually occurs in a person?s fifth decade. While it is self-limiting in some patients, approximately one-third of patients require continuous therapy for continuous flare-ups.

Subcorneal pustular dermatosis successfully treated with adalimumab monotherapy: A case report

Subcorneal pustular dermatosis successfully treated with adalimumab monotherapy: A case report

Quantifying the clinical and economic burden of desquamating dermatological conditions: Implications for a supraregional burns centre

IntroductionThis study sought to identify the spectrum of desquamating skin diseases referred for tertiary burns care and quantify the care requirements and expense associated with caring for these patients within the burns service. MethodsPatient records were identified with non-burn induced skin loss between 2016 and 2022. Data was extracted from inpatient records, operative notes, dressing clinic records. A cost analysis was conducted using figures from the National Schedule of National Health Service Costs and our own unit-specific costs. Results20 patients were identified, with a median age of 46.5 and median TBSA of 30%. The mean length of stay was 21.2 days with 8/20 patients requiring intensive care. Overall mortality was 30%, rising to 50% if patients required ITU admission. Patients had a mean of 1.5 procedures under general anaesthesia and a mean operative time of 169minutes per patient. Post-operatively, a mean of 8.3 dressing changes were required per patient (range 1-21). 75% of patients were referred as suspected toxic epidermal necrolysis syndrome (TENS) yet, only 32% of patients histologically had TENS (32%), with linear IgA disease, pemphigus vulgaris, and bullous lupus comprising the other diagnoses. Cost analysis predicted a total cost to the unit of £1,422,106. ConclusionDesquamating dermatological diseases are life-threatening conditions with exhaustive care requirements. Our experiences highlight the importance of awareness of the range of desquamating skin conditions beyond TENS to enable optimum management, and the need to ensure adequate financial provisions to accommodate the care requirements mandated by these patients.

Three cases of subcorneal pustular dermatosis with immunohistochemical examinations.

Subcorneal pustular dermatosis, a rare, benign skin disease, is a type of neutrophilic dermatosis. The authors reported three cases of subcorneal pustular dermatosis. In case 1, a 9-year-old girl developed a skin rash with blisters following a mycoplasma infection and had a flare-up due to a common cold. She was successfully treated with a topical corticosteroid. In case 2, a 70-year-old woman who had been treated for rheumatoid arthritis with adalimumab, salazosulfapyridine, and leflunomide developed 3- to 5-mm pustules on her trunk and thighs 4 days after flu vaccination. The rash disappeared with drug withdrawal and treatment with diaminodiphenyl sulfone. In case 3, an 81-year-old man, who was diagnosed with pyoderma gangrenosum at 61 years old, developed multiple small flaccid pustules on his trunk and extremities due to an infection in the arteriovenous shunt area on the forearm. The pustule disappeared with intravenous antibiotic therapy; however, the pustules subsequently flared up along with ulcers typical of pyoderma gangrenosum. He was given oral prednisolone therapy, which was effective for the small pustules and some ulcers. Immunohistochemical examination of the three cases revealed neutrophilic infiltration in the subcorneal layer of the epidermis. The pustules contained neutrophils as well as some CD68+ and a few CD1a+ cells. The epidermis and dermis were more predominantly infiltrated by CD4+ cells than by CD8+ cells. Positive stainings for interleukin 8, interleukin 36γ, and phospho-extracellular signal-regulated kinases 1 and 2 were observed in the upper layers of the epidermis below the pustules. Although the pathogenesis of subcorneal pustular dermatosis has not been clarified, the current results suggest that a variety of inflammatory cells, including those responsible for both innate and acquired immunity, are involved in the accumulation of neutrophils in subcorneal pustular dermatosis.

Subcorneal pustular dermatosis treated successfully with apremilast.

Australasian Journal of DermatologyEarly View LETTER Subcorneal pustular dermatosis treated successfully with apremilast Lori Zhang MD, Corresponding Author Lori Zhang MD [email protected] orcid.org/0009-0008-9403-0812 Royal Perth Hospital, Perth, Western Australia, Australia Correspondence Lori Zhang, Royal Perth Hospital, Perth, WA, Australia. Email: [email protected]Search for more papers by this authorKurt Gebauer MBBS, FACD, FACP, Kurt Gebauer MBBS, FACD, FACP University of Western Australia, Perth, Western Australia, Australia Probity Health Care, Waterloo, Ontario, Canada Fremantle Dermatology, Fremantle, Western Australia, AustraliaSearch for more papers by this author Lori Zhang MD, Corresponding Author Lori Zhang MD [email protected] orcid.org/0009-0008-9403-0812 Royal Perth Hospital, Perth, Western Australia, Australia Correspondence Lori Zhang, Royal Perth Hospital, Perth, WA, Australia. Email: lori.zhan[email protected]Search for more papers by this authorKurt Gebauer MBBS, FACD, FACP, Kurt Gebauer MBBS, FACD, FACP University of Western Australia, Perth, Western Australia, Australia Probity Health Care, Waterloo, Ontario, Canada Fremantle Dermatology, Fremantle, Western Australia, AustraliaSearch for more papers by this author First published: 27 May 2023 https://doi.org/10.1111/ajd.14073 The photographs in this letter were obtained with the informed consent of the patient, and there are no identifying markings or words in the images. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation

TZANCK SMEAR: A USEFUL DIAGNOSTIC TOOL IN SPECTRUM OF VESICULOBULLOUS DISEASES OF SKIN.

Introduction: The pemphigus group of autoimmune bullous disorders and mucocutaneous herpesvirus infections are generally diagnosed with the Tzanck smear. Only a few research have looked into its diagnostic value in the literature. To evaluate tzanck smear ndings in patients Objectives: with vesiculobullous skin lesions and histopathological correlation. All recei Material and Methods: ved Samples received between 01/08/2021 to 31/01/2022 were stained with May-Grunwald-Giemsa. In some patients, methylene blue staining and ZN staining were done. Tzanck Results: smear was performed on a total of 25 patients with skin lesions. The sensitivities of multinucleated giant cells and acantholytic cells in herpetic infections, dyskeratotic acantholytic cells,inammatory cells and cocci in bullous impetigo, acantholytic cells with neutrophils and lymphocytes in pemphigus foliaceous, acantholytic cells and plenty of neutrophils in drug-induced pemphigus, no acantholytic cells with neutrophils and lymphocytes in IgA pemphigus, cluster of acantholytic cells in pemphigus vulgaris, only lymphocytes with no acantholytic cells in bullous pemphigoid and dyskeratotic acantholytic cells without inammation in staphylococcal scalded skin syndrome, intracytoplasmic HendersonPatterson bodies and anucleated squamous cells in molluscum contagiosum and broblast, leucocytes with necrotic basal cells in Toxic epidermal necrolysis. The tzanck smear test is an inexpensive, useful, quick, reliable Conclusion: and an easy diagnostic tool for certain vesiculobullous skin diseases.

Pruritic crusted lesions in an 89‐year‐old woman

Pruritic crusted lesions in an 89‐year‐old woman

Subcorneal pustular dermatosis (Sneddon-Wilkinson disease): a case report

This article contains modern data on the etiology, clinical presentation and treatment as well as the differential diagnosis of a rare dermatosis, Sneddon-Wilkinson disease. Clinical observation of subcorneal pustular dermatosis in a 68-year-old woman is reported. Histopathological examination played a key role in making the correct diagnosis. Treatment consisted of a physiotherapeutic approach (311 nm UVB phototherapy) in combination with systemic glucocorticosteroids (dexamethasone). This therapeutic approach (instead of dapsone therapy) can be used due to its relatively good safety profile.

A pen sketch for oral pemphigus vulgaris: A review

Pemphigus is a rare chronic mucocutaneous autoimmune bullous dermatosis. Based on clinical features and pathophysiology the various subtypes include pemphigus Vulgaris (PV), pemphigus foliaceus (PF), IgA pemphigus, and paraneoplastic pemphigus (PNP). Autoantibodies against desmogleins 1 and 3 cause pemphigus Vulgaris which results in acantholysis, or the loss of cell-to-cell adhesion ultimately causing potentially lethal bullae and erosion formation. 80 to 90% of patients develop oral lesions that are manifested before mucocutaneous lesions in more than half of patients.Dental professionals are pivotal and can thus diagnose the disease and prevent skin involvement through proper therapy. Treatment should include systemic corticosteroids and immunosuppressive drugs. Intravenous pulse therapy is instituted in severe cases of pemphigus. This article is an attempt to present clinical manifestations, pathophysiology, and newer medical treatment modalities of pemphigus.

Dermoscopic approach for differential diagnosing of autoimmune bullous disease: pemphigus vulgaris, pemphigus foliaceus, and IgA pemphigus.

Dermoscopy is a noninvasive technique for the evaluation of different pigments and microstructures of the epidermis, dermoepidermal junction, and papillary dermis that are not apparent to the naked eye, which therefore improves diagnostic accuracy. This study aims to describe the characteristic dermoscopic features of bullous diseases and analyze the characteristic dermoscopic features of bullous diseases of the skin and hair. A descriptive study was conducted to describe and analyze the characteristic dermoscopic features of bullous diseases in the Zagazig University Hospitals. This study enrolled 22 patients. Dermoscopy revealed yellow hemorrhagic crusts in all patients and white yellow structure with red halo in 90.9% of patients. Pemphigus vulgaris patients were identified by the presence of dermoscopic clues such as bluish deep discoloration, tubular scaling, black dots, hair casts, hair tufts, yellow dots with whitish halos (fried egg sign) and yellow follicular pustules that are not seen in pemphigus foliaceus and IgA pemphigus. Dermoscopy is an important tool that serves as a link between clinical and histopathological diagnoses, and it can easily be used in daily practice. Several suggestive dermoscopic features can help in the differential diagnosis of autoimmune bullous disease but only after making a provisional clinical diagnosis. Dermoscopy is a very useful tool in the differentiation of pemphigus subtypes.

Mukozayı da etkileyen bir generalize subkorneal püstüler dermatozun siklosporin ile başarılı tedavisi: Bir olgu sunumu

Subcorneal pustular dermatosis (SPD), is rare, chronic, and relapsing pustuler eruption. Mostly, it affects middle-aged and elderly women. Mucosal involvement is extremely rare. Here we report a 31-year-old woman with generalized SPD with lip involvement. The patient had annular or serpiginous patterned well-demarcated plaques on the trunk, intertriginous areas and flexor aspects of the limbs, palmoplantar areas, face, and lip mucosa. The bullous lesions exhibited the typical half and half sign. The patient was treated successfully with systemic steroids and cyclosporine.

Subcorneal Pustular Dermatosis Occuring in Association with Pyoderma Gangrenosum and Rheumatoid Arthritis: A Triple Whammy!

Neutrophilic dermatoses are a wide group of disorders encompassing indolent to severely disabling conditions. A co-existence of two such conditions, pyoderma gangrenosum (PG) and subcorneal pustular dermatosis, necessitates a thorough investigation for IgA dysglobulinemia. We report a middle-aged woman who developed PG following 18 years of (undiagnosed) subcorneal pustular dermatosis, along with rheumatoid arthritis, a known association of PG.

Significance of anti-desmocollin autoantibodies in pemphigus.

The major autoantigens for pemphigus are desmogleins (Dsgs), cell-cell adhesive structure proteins, one of the desmosomal cadherins. Recent progress in molecular biology has revealed that IgG autoantibodies of classical pemphigus react with Dsg1 or Dsg3. Desmocollins (Dscs) also belong to the cadherin supergene family that provides structure to the desmosomes and play an important role in cell-to-cell adhesion. In addition to the presence of four desmosomal Dsg isoforms, i.e. Dsg1-4, Dsc1, 2 and 3, all of which are derived from different genes, Dsc1 has been previously identified as the target antigen of IgA autoantibodies in the subcorneal pustular dermatosis (SPD)-type of intercellular IgA dermatosis. In addition to the IgA anti-Dsc1 autoantiboides, the presence of IgG anti-Dsc autoantibodies is described in patients of some autoimmune bullous diseases. In particular, the current pemphigus detecting autoantibodies to Dscs has shown a tendency in atypical variants of pemphigus. Therefore, autoantibodies against Dscs alone may cause detachment of cell-cell adhesion in the epidermis in some pemphigus. However, except for the findings of a few in vitro and in vivo studies, there is currently no clear evidence for the pathogenicity of anti-Dsc autoantibodies in pemphigus, whereas significance of anti-Dsg autoantibodies is well established. This article describes the structure and function of the Dscs, and explores the evidence regarding the pathogenic role of anti-Dsc autoantibodies in pemphigus.

Mycoplasma Pneumoniae-associated Subcorneal Pustular Dermatosis: Not as Rare as We Think?

Mycoplasma Pneumoniae-associated Subcorneal Pustular Dermatosis: Not as Rare as We Think?

IgA pemphigus and Sneddon Wilkinson disease: a spectrum of diseases?

IgA pemphigus and Sneddon Wilkinson disease: a spectrum of diseases?