Pustular Dermatosis Research Articles

Pustular and crusted lesions in systemic lupus erythematosus: A case report

ABSTRACT Systemic lupus erythematosus (SLE) is the prototype of an autoimmune disease with various manifestations in the skin and several other organs. Subacute cutaneous lupus erythematosus may present with annular and psoriasiform lesions. There have been case reports of pustular lesions in SLE. Herein, we describe a known case of SLE, who developed pustular and crusted lesions which complicated the course of the disease. We considered Acute Generalised Exanthematous Pustulosis, Subcorneal Pustular Dermatosis, Pustular Psoriasis, Pustular Vasculitis, and Pustular Folliculitis as our initial differential diagnosis. A potassium hydroxide mount from crusted lesions showed scabies mites. With these findings, a diagnosis of SLE complicated with crusted scabies was made. She was managed with multiple systemic immunosuppressant and antiscabetic measures following which clinical improvement was seen with remission of her lesions.

Erosive pustular dermatosis of the scalp

This case of an elderly woman with a rapidly growing lesion of her scalp that developed several months after treatment of a squamous cell carcinoma with Mohs micrographic surgery illustrates a known precipitating trigger of erosive pustular dermatosis of the scalp. This report describes the clinical stages of the disease and their corresponding histologic findings. It reviews the epidemiology and proposed etiology of this erosive pustular dermatosis of the scalp. The differential diagnoses of scalp disease are extensive. An approach to evaluating scalp dermatoses through physical examination, laboratory testing and pathology findings will be discussed. Finally, treatment of this chronic condition, including both patient education and medical management, is detailed.

A Case of Erosive Pustular Dermatosis of the Scalp Due to Osimertinib

A Case of Erosive Pustular Dermatosis of the Scalp Due to Osimertinib

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.

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.

P29 Erosive and vesicular dermatitis

Abstract We present a case of a 3 year old girl, born at term via normal vaginal delivery, with significant erosive lesions present from birth. Finger and toenails were small and she was systemically well. At day three, there was already evidence of healing and by week two, the erosions had almost fully healed with residual scaling. Once fully resolved, areas of hypopigmented, reticulate scarring remained. Investigations included swabs for bacterial, fungal and viral infections were negative. There was no concern regarding perinatal infection and apart from oligohydramnios, there were no other issues in pregnancy. She was managed on the burns unit with nutritional support, antibiotics, emollients and dressings. She subsequently developed a left micro-ophthalmia and cataract, right severe myopia and a mild developmental delay with microcephaly. Older siblings aged 7 and 5 had a history of mild developmental delay and microcephaly. Parents had no relevant medical issues and were both of Jewish background. Genetic testing was carried out which did not identify a genetic cause with normal microarrays of her siblings. A diagnosis of vesicular and pustular dermatosis was made, a rare condition, first described in 1985 by Cohen et al. Lesions typically consist of erythema, erosions, crusting, vesicles and fissures which start to heal within 10 days with residual reticulate scarring. Most cases occur in premature infants, making our case less typical. Nail, ophthalmological abnormalities and developmental delay are other associations, seen in our case.

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

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

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.

Epidermal growth factor receptor inhibitor Geftinib-induced erosive pustular dermatosis of the scalp – A report with a brief review of the literature

Gefitinib is an epidermal growth factor receptor inhibitor used in the treatment of non-small cell carcinoma of the lungs, adenocarcinoma of the lungs, and solid malignancies of the head and neck region. A 65-year-old female patient with non-small cell carcinoma lung with bone metastasis was started on gefitinib monotherapy after which the patient noticed papulo-pustular lesions on the scalp, later leading to loss of hair. Dermatological examination revealed erythema, pustules with crusting, along with matting of the hair on the frontoparietal area with some areas of hair loss. Trichoscopic examination showed the absence of follicular openings indicating scarring alopecia. Skin biopsy showed perivascular mixed infiltrate of lymphocytes and neutrophils and absent hair follicles, consistent with gefitinib-induced erosive pustular dermatosis of the scalp, a rare entity.

Erosive Pustular Dermatosis of the Scalp After Excision and Skin Grafting of Scalp Squamous Cell Carcinoma: Erratum

Erosive Pustular Dermatosis of the Scalp After Excision and Skin Grafting of Scalp Squamous Cell Carcinoma: Erratum

Assessment of specificity of dermatopathologic criteria for IgG4-related skin disease.

IgG4-related disease (IgG4-RD) represents a recently characterized multisystemic fibroinflammatory condition that can manifest a spectrum of skin findings (IgG4-related skin disease; IgG4-RSD). Histopathologic and immunohistochemical criteria have been proposed; however, the specificity of these criteria merits scrutiny given the potential histopathologic overlap of IgG4-RSD and both neoplastic and inflammatory skin conditions featuring lymphoplasmacytic infiltrates (IgG4-RSD mimics). This study sought to assess the specificity of the criteria by quantifying the frequency by which an expanded spectrum of IgG4-RSD mimics meet proposed thresholds. Following IRB approval, a total of 69 cases of IgG4-RD mimics, representing 14 different diagnoses featuring plasma cells, were reviewed and analyzed for the following histopathologic and immunohistochemical features: (i) maximum IgG4+ count/high-powered field (hpf) >200; (ii) IgG4/IgG ratio >0.4 averaged over 3 hpfs; (iii) IgG4+ count >10 per hpf. Screening for IgG4-RSD by histopathologic criteria demonstrated the high frequency of lymphoplasmacytic infiltrates, contrasted with the rarity of storiform fibrosis (only one case of erythema elevatum diutinum [EED]) and obliterative phlebitis (0 cases). By immunohistochemical criteria, the analysis revealed that no cases exceeded 200 IgG4+ cells; 13% (9/69) cases demonstrated an IgG4/IgG ratio of >0.4 averaged over 3 hpfs; and 23% (16/69) cases demonstrated a mean IgG4+ count of >10 per hpf. Application of proposed IgG4-RSD histopathologic criteria to an expanded spectrum of potential IgG4-RSD mimics (to include cutaneous marginal zone lymphoma, syphilis, necrobiosis lipoidica, lichen sclerosus, ALHE, psoriasis, lymphoplasmacytic plaque, EED, and erosive pustular dermatosis), highlights the relative nonspecificity of lymphoplasmacytic infiltrates contrasted with the stringency of storiform fibrosis and obliterative fibrosis. Furthermore, an IgG4+ cell count of >10 per hpf and an IgG4/IgG ratio of >0.4 are not specific to IgG4-RSD alone. In the appropriate clinical context for IgG4-RSD, histopathologic features still represent the entry threshold for diagnosis consideration, which then allows for further screening by immunohistochemical criteria.

Inflammatory Tinea Capitis Masquerading as Erosive Pustular Dermatosis

Inflammatory Tinea Capitis Masquerading as Erosive Pustular Dermatosis

HLA-C*15:02 and epidermal growth factor receptor inhibitor-induced erosive pustular dermatosis of the scalp.

Epidermal growth factor receptor inhibitors (EGFRIs) are widely used to treat various types of malignancies. One of the common adverse reactions is cutaneous toxicity, mostly presenting as acneiform eruptions, paronychia and xerosis. Erosive pustular dermatosis of the scalp (EPDS) is a rare cutaneous adverse reaction that develops during treatment with EGFRIs. The pathogenesis of EGFRI-induced EPDS is poorly understood. Here we present three cases of EPDS induced by EGFRIs. The proteins LTA4H (leukotriene A-4 hydrolase), METAP1 (methionine aminopeptidase 1), BID (BH3-interacting domain death agonist), SMAD1 (mothers against decapentaplegic homologue), PRKRA (interferon-inducible double-stranded RNA-dependent protein kinase activator A), YES1 (tyrosine-protein kinase Yes) and EGFL7 (epidermal growth factor-like protein 7) were significantly upregulated in EGFRI-stimulated peripheral blood mononuclear cell cultures, and validated in the lesions. All of the proteins colocalized with CD4+ and CD8+ T-cell expression. Next-generation-based human leucocyte antigen (HLA) typing showed all patients carried HLA-C*15:02, and modelling studies showed that afatinib and erlotinib bound well within the E/F binding pockets of HLA-C*15:02. Moreover, T cells were preferentially activated by EGFRIs in individuals carrying HLA-C*15:02. The case series revealed that EGFRI-induced EPDS may be mediated by drug-specific T cells.

Erosive Pustular Dermatosis of the Scalp Treated with Serial Debridement: Case Report

An 82-year-old man with a past medical history of numerous non-melanoma skin cancers (NMSCs), including unresectable squamous cell carcinoma of the right temple treated with radiation therapy and cemiplimab, presented with worsening erosive pustular dermatosis of the scalp (EPDS). This had initially onset four years prior in the setting of 5-fluorouracil and photodynamic therapy for actinic keratoses. He had failed topical ultra-potent steroids, tacrolimus, and dapsone as well as oral dapsone, doxycycline, and acitretin. The condition’s chronicity and radiation to the area likely exacerbated his EPDS. We pursued serial debridement, followed by clobetasol propionate 0.05% ointment twice daily. The patient reported marked improvement with stable condition between treatments. Upon better control of his EPDS, a suspicious ulcerative lesion under a previously affected area was identified and biopsied, revealing atypical fibroxanthoma. EPDS is an inflammatory dermatosis characterized by pustules, erosions, and crusting, often caused by sun damage, local trauma, and topical medications. First-line treatment is topical corticosteroids. We show that serial debridement is effective for treating recalcitrant EPDS. We emphasize the importance of maintaining adequate control of EPDS to avoid masking suspicious lesions, as the patient demographic affected by EPDS is at high risk for NMSCs.

Efficacy and safety of biological agents to treat patients with palmoplantar pustulosis: A systematic scoping review.

Palmoplantar pustulosis (PPP), a chronic, recurrent pustular dermatosis associated with erythema, scales, and sterile pustules on the palms and soles, is commonly encountered in dermatology clinics. Whether PPP is a variant of psoriasis or a distinct condition is still debated. Although biological agents have been successfully used to treat moderate-to-severe psoriasis, existing literature on PPP is limited to case reports or small case series. The lack of well-documented clinical studies makes it difficult to select the ideal treatment for this condition. This review aims to discuss the efficacy and safety of biological agents in PPP treatment based on randomized controlled trials with the hope of inspiring dermatologist clinicians to propose new therapeutic approaches. This review aims to obtain high-level evidence to assess the efficacy and safety of biological agents in the treatment of patients with PPP. We searched the PubMed, Embase, and Cochrane databases up to May 18, 2023, for high-quality randomized controlled trials that reported at least one adverse event after PPP treatment with biological agents in patients>18years of age. RevMan 5.3 software was used for the meta-analysis. Nine trials involving 799 participants were included in the analysis. We used ppPASI 75 as the primary efficacy measure. Anti-IL-23 and anti-IL-17A agents afforded 4.14-fold and 1.95-fold better outcomes than placebo treatment at weeks 16 and 12, respectively (P-value=0.009, RR=4.14, 95% confidence interval [CI; 1.43-11.98]; P-value=0.02, RR=1.95, 95% CI [1.11-3.42]). Moreover, anti-IL-23 agents at a dose of 100mg were more effective than at 200mg, indicating that 100mg may be the best dose for anti-IL-23 agents. Next, we investigated the safety of biological agents for PPP treatment. The incidence of total adverse events (AEs) was 1.25 times higher for biological agents than for controls, indicating a good safety profile (RR=1.25, P-value ＜ 0.00001, 95% CI [1.13, 1.37]). Additionally, we divided the common AEs into 16 categories and found that anti-IL-23 agents were more likely to induce infections. In conclusion, we evaluated safety and efficacy in a comprehensive comparison and found that anti-IL-23 agents conferred good clinical efficacy with a low incidence of AEs and could be recommended with caution. Only a few relevant, high-quality, randomized controlled trials were included in the study. This study showed that biological agents can be used to treat patients with PPP with good efficacy; however, AEs cannot be ignored. Multi-center, high-quality clinical studies with large sample sizes are needed to further evaluate the effects and safety of biological agents in PPP treatment.

ORF Nodule Complicated by Erythema Multiforme: About 2 Cases

ORF nodule is a disease caused by a parapoxvirus ovis with epithelial tropism which is transmitted to humans from sick animals, sheep and goats, suffering from contagious ovine pustular dermatosis ecthyma [1]. Common complications of ORF infection have been reported, including secondary bacterial infection, lymphadenopathy, lymphangitis, more rarely erythema multiforme and bullous pemphigoid [2]. We describe here two cases of ORF nodule with secondary erythema multiforme.

H07 Papyri and sarcophagi: a tour of dermatology in Ancient Egypt

Abstract Long before medicine was demystified by the Renaissance, Enlightenment and modern thinkers with whom we are most familiar, the skin and its diseases were pondered on by the Ancient Egyptians. Through their writings and remains, modern scholars are able to assemble a picture of what dermatological conditions ailed the Egyptians and how they were contemporaneously perceived. The Ebers Papyrus (c. 1550 Bc), the largest record of Ancient Egyptian medicine, is noted for its vivid descriptions of lesions and grisly details of treatments. Some of the conditions mentioned therein are straightforwardly identified from their descriptions, whereas others are open to interpretation. Recent DNA evidence has also shed light on this topic, causing scholars to re-evaluate the presence of various conditions, such as leprosy and tuberculosis, in different parts of the ancient world. The Egyptians are perhaps most famed for the preservation of bodies through the process of mummification, which provides us with a unique glimpse into the nature of skin disease in the ancient world. Mummified skin tends to be shrunken, deeply pigmented and brittle. Even so, nails, hair and epidermal ridging are preserved. Prolonged rehydration and staining can reveal the stratum corneum, keratinocytes, melanin, collagen and elastic fibres, along with adnexal structures and subcutaneous tissue. Mummies with identifiable dermatological disease include Ramses V (d. 1145 Bc), whose face, neck, shoulders, lower abdomen and scrotum bear the marks of smallpox. The diagnosis was later confirmed by electron microscopy, which identified the variola viral corpuscle in his skin. Skin tumours have also been found in mummified remains, as have syndromic features of diseases, which predispose to multiple dermatological malignancies, such as Gorlin–Goltz syndrome, which is suspected in two fraternal mummies from around 3000 Bc. One specimen from c. 1300 Bc was identified as having a venous ulcer, which was subsequently confirmed when histology demonstrated perivascular haemosiderin deposition. Other tentative diagnoses have been made on appearance alone, including pustular dermatosis, impetigo and pemphigus foliaceus. Too often do we perceive medical history as starting in earnest during the last few centuries. The Ancient Egyptians have bequeathed to us a wealth of information, both historical and archaeological, which, combined with modern techniques, has allowed us to peer through the millennia into a dermatological world that can feel simultaneously alien and familiar. This presentation/poster will showcase what we understand of this world and what future research might hold in store.

Incidence and time to development of malignancies arising on the scalp of patients with erosive pustular dermatosis based on sex: A retrospective analysis

Incidence and time to development of malignancies arising on the scalp of patients with erosive pustular dermatosis based on sex: A retrospective analysis

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