Pustular Dermatosis Research Articles

Erosive pustular dermatosis of the scalp induced by gefitinib: case and review of the literature

Gefitinib is a selective tyrosine kinase inhibitor of epidermal growth factor receptor (EGFR) used for the treatment of malignant neoplasms. The most frequent skin complication during gefitinib therapy is an acneiform papulopustular eruption, usually distributed in the seborrheic areas but occasionally widespread. We report a patient with erosive pustular dermatosis of the scalp, a neutrophil-mediated skin disease presenting with sterile pustules evolving into erosions and crusts on the scalp, during treatment with the EGFR inhibitor gefitinib for lung cancer. A literature review of the drug-induced cases of this rare entity is provided.

How well do treatments for erosive pustular dermatosis of the scalp work?

Erosive pustular dermatosis of the scalp (EPDS) is a skin disease that causes non‐healing wounds on the scalp. It may be more common than previously thought, as it can be misdiagnosed. EPDS usually affects older patients with lots of sun damage, and it may also be caused by injury, surgery, or certain treatments used by dermatologists, such as 5‐fluorouracil cream, which is used to treat pre‐cancerous lesions.

Subcorneal pustular dermatosis associated with IgG monoclonal gammopathy of undetermined significance

Subcorneal pustular dermatosis is a rare chronic relapsing bullous neutrophilic dermatosis. Because it can be associated with monoclonal gammopathy of undetermined significance and multiple myeloma, screening for these conditions is necessary. Herein, we present a case of subcorneal pustular dermatosis, with concurrent monoclonal gammopathy of undetermined significance, successfully treated with acitretin.

Subcorneal Pustular Dermatosis Successfully Treated with Acitretin in a Dog

Subcorneal Pustular Dermatosis Successfully Treated with Acitretin in a Dog

Neutrophilic dermatoses with unusual and atypical presentations

Neutrophilic dermatoses (NDs) are a group of reactive, noninfectious autoinflammatory diseases characterized by (1) infiltration of the epidermis, dermis, and or/hypodermis by neutrophils; (2) their association with distinct diseases (eg, hematologic malignancy and chronic inflammatory diseases); (3) potential extracutaneous involvement; and (4) response to anti-inflammatory drugs, such as corticosteroids, dapsone, colchicine, and novel biologic therapies, such as the anti–interleukin-1 blockade. Although distinct NDs have been described, transitional forms with overlapping features are often identified. These justify a simplified classification of NDs with three major forms: superficial (epidermal or pustular) NDs, dermal (en plaques) NDs, and deep NDs.We review selected or novel variants of NDs, including subcorneal pustular dermatosis, the group of immunoglobulin A neutrophilic dermatoses, amicrobial pustular dermatosis of the folds, and neutrophilic urticarial dermatosis, as well as atypical forms of Sweet syndrome and pyoderma gangrenosum closely mimicking severe infectious diseases. Knowledge of these variants is essential for proper diagnosis, adequate management, and avoidance of a dangerous escalation of therapy, such as unnecessary immunosuppression or extensive surgery.

Efficacy and safety of treatment with anti‐tumor necrosis factor‐α drugs for severe impetigo herpetiformis

Impetigo herpetiformis (IH) is a rare pustular dermatosis. It can be life-threatening for both the mother and fetus and often causes therapeutic problems. However, there is no specific guideline for the treatment of IH and the evidence regarding the efficacy of treatments for IH has not been established. Herein, we report two cases of IH, which were successfully treated with anti-tumor necrosis factor (TNF)-α drugs. The serum levels of the drugs in the infants and mothers were examined using enzyme-linked immunosorbent assay (ELISA). Case 1 was a 35-year-old, gravida 2, para 1, female patient in week 20 of pregnancy; she was treated with adalimumab (ADA) until delivery. Case 2 was a 26-year-old, gravida 1, para 0, female patient in week 30 of pregnancy; she was treated with certolizumab pegol (CZP) until delivery. In both cases, the skin lesions started regressing considerably after administration of the biologic agents. We examined the serum levels of the biologic agents in the mothers and infants using ELISA. In case 1, the ADA serum level in the infant was as high as that in the mother at birth; it then decreased below the lower limit of quantification at week 12 post-delivery. In case 2, the CZP serum level in the infant was below the lower limit of quantification at birth. In this report, we revealed that biologic agents could be an effective treatment for severe IH and that CZP treatment can be considered safe for the mothers and fetuses.

Subcorneal Pustular Dermatosis associated with psoriasis vulgaris — acitretin treatment

Subcorneal Pustular Dermatosis associated with psoriasis vulgaris — acitretin treatment

熱傷後瘢痕に生じたerosive pustular dermatosisの1例

熱傷後瘢痕に生じたerosive pustular dermatosisの1例

Vesicles, pustules and vesiculopustules in early childhood

Vesicles, pustules and vesiculopustules are frequent in newborns and infants and mostly self-limiting. In order to differentiate the rare life-threatening diseases and to therefore initiate the treatment in a timely manner, knowledge of vesicular, pustular and vesiculopustular dermatoses in early childhood is necessary.

Erosive pustular dermatosis of the scalp: causes and treatments.

Erosive pustular dermatosis of the scalp is a rare condition which primarily affects older women after local trauma and has historically been treated with topical steroids. As it is a rare entity and resembles other dermatologic conditions, it may easily be misdiagnosed. Identifying the causes and evaluating the efficacy of treatments of erosive pustular dermatosis of the scalp (EPDS) is of great importance to both avoid misdiagnosis and ensure optimal treatment of this rare condition. There are numerous causes. In addition to surgeries and physical injuries, topical and procedural treatments for actinic keratoses and androgenetic alopecia can trigger the development of lesions. There are also documented associations with several autoimmune and systemic conditions. Besides corticosteroids, topical tacrolimus and photodynamic therapy were the most commonly used treatments for EPDS. They were effective with few recurrences and adverse effects. Other successful treatment options were topical dapsone, silicone gels, calcipotriol, acitretin, and isotretinoin. Oral dapsone can be used in cases of disseminated disease. Zinc sulfate should be considered with low-serum zinc levels. While cyclosporine was effective, there were adverse effects that may limit its use. It is important for dermatologists to be aware of the wide array of potential causes of erosive pustular dermatosis and include it on their differential. Additionally, although high-potency topical steroids have been historically used as the first-line treatment, there are many other effective treatments that may avoid recurrence and skin atrophy, particularly in the elderly population.

Subcorneal Pustular Dermatosis with Temporary Autoimmune Features during COVID-19 Pandemic

Subcorneal pustular dermatosis, Sneddon-Wilkinson, is an uncommon neutrophilic dermatosis of unknown etiology. We report on a 51-year-old woman who presented with multiple superficial erythematous erosions surrounded by annular arranged sterile pustules concentrated on the trunk, the neck, and the proximal extremities during the coronavirus disease-19 pandemic. Larges pustules and flaccid bullae showed a hypopyon. There were no fever and no pruritus, general health was unaffected. Laboratory investigations revealed leukocytosis, neutrophilia, lymphopenia, and increased C-reactive protein. Initially, antinuclear antibodies, pemphigoid antibodies, and antibodies to BP 230 were positive, but negative 5 days later. Nasopharyngeal swabs were negative for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) RNA tested by real-time reverse-transcriptase-polymerase chain reaction. A diagnostic skin biopsy showed epidermal spongiotic vesiculation and subcorneal pustulation with acantholysis and an inflammatory infiltrate composed of neutrophils and lymphocytes. The confirmed diagnosis was subcorneal pustular dermatosis Sneddon-Wilkinson. She was treated by dapsone and corticosteroids with the latter tempered down. Clinical response was rapid. We suggest that the autoimmune features seen on admission may be due to an undefined viral infection, but not SARS-CoV-2.

English

English

Therapeutic options for erosive pustular dermatosis of the scalp: a systematic review.

Erosive pustular dermatosis of the scalp (EPDS) is a chronic condition characterized by erosive plaques and subsequent scarring alopecia as a result of local trauma or inflammation. A number of therapeutic approaches have been described in the literature but there is no consensus of opinion on optimal treatment of the disease. To provide evidence-based recommendations for topical and systemic treatment of adult patients with EPDS by performing a systematic review. The MEDLINE, MEDLINE In-Process, Embase and Cochrane databases were searched from inception to 26 June 2019 in accordance with the PRISMA guidelines for studies involving adult patients treated for EPDS with at least one reported response to treatment. The study was registered on PROSPERO. Texts were reviewed independently by two authors. The risk of bias and quality of the studies were assessed using the Quality Appraisal Checklist for Case Series Studies. In total 75 studies were included, involving 168 patients. Many treatments have been reported in the literature, with varying degrees of therapeutic success. The results were highly heterogeneous and methodological quality was very low. We were unable to perform a meta-analysis on the data. The limited available evidence supports use of topical corticosteroids, with or without oral zinc, followed by maintenance therapy with topical calcineurin inhibitors as being effective in managing this condition. Topical photodynamic therapy is also potentially beneficial in the management of EPDS. Prospective, comparative, randomized controlled trials are required in order to provide further evidence to guide treatment.

Novel Therapeutic Approaches and Targets for the Treatment of Neutrophilic Dermatoses, Management of Patients with Neutrophilic Dermatoses and Future Directions in the Era of Biologic Treatment.

Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders characterized by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. Universally accepted and validated guidelines for the management of neutrophilic dermatoses do not exist, also given the paucity of randomized controlled study and high-quality data. However, the literature on the effective use of biologic therapies is rapidly expanding. This article reviews the epidemiology, clinical characteristics, histopathologic features, and management of pyoderma gangrenosum as well as Sweet's syndrome, sub-corneal pustular dermatoses and bowel-associated dermatosis arthritis syndrome. The use of biologic agents, including tumor necrosis factor α-inhibitors, anti-IL1, anti-IL-17, and IL-23 are discussed in detail.

English

English

Case reports on erosive pustular dermatosis of scalp: a cross sectional study at a tertiary care centre

<p class="abstract">Erosive pustular dermatosis of the scalp (EPDS) has been reported as a rare chronic and progressive non-infectious inflammatory condition of the actinically damaged scalp characterized by crusts, erosions and pustules. It tends to occur on the scalp of older people who have usually been treated for diffuse actinic damage and actinic keratoses. The list of possible differential diagnosis is long and includes tinea capitis, folliculitis decalvans, neutrophilic dermatosis, autoimmune vesiculobullous disorders, malignancy etc. Notoriously difficult to treat, these cases tend to be chronic and progressive. These patients often present after repeated treatment to various scalp dermatosis that doesn’t result in clearing instead persistence and perpetuation of the process. A cross sectional prospective study was conducted in department of dermatology in a tertiary care centre in which six cases of EPDS attending outpatient department from 2017 to 2019 were included. EPDS is more common but under recognized condition, hence less cases are reported till date. Increased awareness of and proper diagnosis of this condition changes the approach towards these patients, protects the scalp from involuntary insults and other precipitating factors that hinders with a better outcome.</p>

Subcorneal pustular dermatosis: Comprehensive review and report of a case presenting during pregnancy

Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a rare, relapsing, sterile pustular eruption of unknown etiology that develops most commonly in middle-aged or mature women. This article reviews the presentation, associations, and management of the condition and highlights advances in pathophysiology. Onset of SPD during pregnancy has not been reported. Herein, we report a case of SPD that developed during pregnancy. The patient was treated with dapsone without complications for her or the fetus. An association between T helper (Th) 17 and Th2 environments in the development of SPD has been advocated. Pregnancy is characterized by a predominance of Th2 responses and increased interleukin-17 levels and thus may favor the development of the condition

北京协和医院29 987例皮肤活检组织的临床病理诊断构成

Objective To analyze the disease constitution, accuracy of clinical and pathological diagnoses of skin biopsy samples in Peking Union Medical College Hospital. Methods A total of 29 987 patients subjected to skin biopsy were collected from Department of Dermatology, Peking Union Medical College Hospital from June 2010 to November 2018, and clinical and histopathological diagnoses of these skin biopsy samples were analyzed retrospectively. Results According to the results of histopathological diagnosis, confirmed diagnoses of these patients could be classified into 33 categories and 242 kinds. Common disease categories included epidermal tumors (2 931 cases, 9.77%) , connective tissue diseases (2 809 cases, 9.37%) , melanocytic tumors (2 078 cases, 6.93%) , erythematous scaly pustular dermatoses (1 376 cases, 4.59%) , lichenoid dermatoses (1 291cases, 4.31%) , allergic or eczematous skin diseases (1 282 cases, 4.28%) and infectious skin diseases (1 156 cases, 3.86%) . Common skin diseases included scleroderma (1 887 cases, 6.29%) , pigmented nevus (1 755 cases, 5.85%) , seborrheic keratosis (1 136 cases, 3.79%) , eczema (1 089 cases, 3.63%) , psoriasis (881 cases, 2.94%) , lichen planus (867 cases, 2.89%) , lupus erythematosus (638 cases, 2.13%) , pemphigus (549 cases, 1.83%) , and basal cell carcinoma (501 cases, 1.67%) . Poor consistency was observed between clinical diagnosis and histopathological diagnosis of lichen planus, bullous pemphigoid, granuloma annulare and hypereosinophilic dermatitis. Conclusions Common disease categories of the skin biopsy samples in Peking Union Medical College Hospital were epidermal tumors, connective tissue diseases, melanocytic tumors, erythematous scaly pustular dermatoses, lichenoid dermatoses, and allergic or eczematous skin diseases. Poor consistency was observed between clinical and pathological diagnosis in some skin diseases, and understanding of these diseases should be improved. Key words: Biopsy; Connective tissue diseases; Nevi and melanomas; Skin diseases, papulosquamous; Lichenoid eruptions; Skin diseases, eczematous

Erosive pustular dermatosis of the scalp: a multicentre study.

Erosive pustular dermatosis of the scalp (EPDS) is characterized by crusted erosions or superficial ulcerations that lead to scarring alopecia. We performed a multicentre retrospective clinical study including 56 patients (29 females and 27 males, mean age 62.7) with a confirmed EPDS in order to describe epidemiology, clinical findings and therapeutic choices of this disease. Mechanical/chemical trauma was reported in 28.6%, a previous infection in 10.7%, a previous cryotherapy in 5.4% androgenetic alopecia in 48.2% and severe actinic damage in 25%. Trichoscopy showed absence of follicular ostia, tufted and broken hair, crusts, serous exudate, dilated vessels, pustules and hyperkeratosis. Histopathology revealed three different features, depending on the disease duration. The most prescribed therapy was topical steroids (62.5%), followed by the combination of topical steroids and topical tacrolimus (8.9%), systemic steroids (7.1%) and topical tacrolimus (5.4%). A reduction of inflammatory signs was observed in 28 patients (50%) treated with topical steroids and in all three patients treated with topical tacrolimus. The relatively high number of patients collected allowed us to identify a better diagnostic approach, using trichoscopy and a more effective therapeutic strategy, with high-potency steroids or tacrolimus, which should be considered as first-line treatment.

Neutrophilic dermatoses: Sweet syndrome and gangrenous pyoderma. Literature review and own clinical observations

Neutrophilic dermatoses are a heterogeneous group of inflammatory skin diseases that have unique clinical features, but are combined by the presence of a sterile, predominantly neutrophilic infiltrate at histopathological examination. Neutrophilic dermatoses include Sweet syndrome, gangrenous pyoderma, Behcet’s disease, neutrophilic urticaria, subcorneal pustular dermatosis, palmoplantaris pustulosis, acrodermatitis Allopo, acute generalized exentematous pustulosis, infant pustulosis, etc. The morphology of skin lesions associated with these diseases is heterogeneous, which makes diagnosis challenging. In addition, a thorough clinical evaluation of patients is necessary to exclude diseases that mimic these disorders and to diagnose potential concomitant infectious, inflammatory, and tumor processes. While some neutrophilic dermatoses may disappear spontaneously, most require treatment to achieve remission. A delay in diagnosis and treatment can lead to significant morbidity and even mortality in these patients. Glucocorticoids are the drugs of choice for the treatment of neutrophilic dermatoses. In case of their ineffectiveness, other biological disease-modifying antirheumatic drugs may be administered depending on the severity of the skin lesion, chronicity of the disease, the presence of systemic lesions, and refractory status. This work is devoted to epidemiology, etiopathogenesis, clinical features, diagnostic signs and modern methods of treating Sweet syndrome and gangrenous pyoderma based on a review of modern literature and our own clinical observations.