Erosive Pustular Dermatosis of the Scalp Following Herpes Zoster: Successful Treatment with Topical Tacrolimus

Erosive pustular dermatosis of the scalp (EPDS) is a rare disorder of the elderly characterized by multiple pustular lesions with erosions and crusting that result in scarring alopecia. EPDS typically develops in aged or sun-damaged skin with a history of trauma. Histopathologically, EPDS is nonspecific, showing atrophic epidermis and chronic inflammation. Bacteriological and mycological investigations of EPDS are generally negative. Although herpes zoster is a common disorder in elderly people, previously reported cases of EPDS were rarely associated with herpes zoster. We present a rare case of EPDS following herpes zoster treated successfully with topical tacrolimus.     

Erosive pustular dermatosis of the scalp: a successful treatment with photodynamic therapy

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory disease of unknown aetiology that usually occurs in the elderly. It is characterized by sterile pustules, chronic crusted erosions, cicatricial alopecia, and skin atrophy. The histopathology is nonspecific, and its pathophysiology remains undetermined, with various types of local trauma possibly acting as the triggering factor. We describe a case of EPDS in a 75-year-old female in whom there was a marked response to photodynamic therapy with methyl 5-aminolaevulinic acid.     

Pustular dermatosis of the scalp associated with autoimmune diseases

A 36-year-old woman visited our hospital with a five month history of persistent pustulation, crusting, and alopecia on the vertex of the scalp. No pathological organisms were isolated from the lesions. Histological examination revealed non-specific changes of chronic inflammation with destroyed follicles. Antibiotic therapy produced no response, but steroid therapy was effective. From these observations, a diagnosis of erosive pustular dermatosis of the scalp (EPDS), as described by Pye et al., was made. The patient also had Hashimoto's thyroiditis, autoimmune hepatitis, and Takayasu's aortitis. The laboratory studies revealed an increased erythrocyte sedimentation rate, C-reactive protein 3+, hypergammaglobulinemia, and various auto-antibodies, suggesting the possibility of a pathogenesis common to both this dermatosis and the autoimmune diseases.     

Erosive pustular dermatosis of the scalp after aplasia cutis congenita in a 9‐year‐old patient: A 5‐year follow‐up

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory skin disease that occurs mainly in elderly adults with a history of trauma to the scalp, but a few cases of EPDS in children have been reported. We report a rare case of EPDS after aplasia cutis congenita in a child.     

Erosive pustular dermatosis of the scalp: reappraisal of an underrecognized entity

Erosive pustular dermatosis of the scalp (EPDS) is an inflammatory dermatosis of unknown etiology. Herein, we present a review of the disease and report our own clinical and histopathological experience in eleven patients. EPDS tends to spontaneously affect bald areas of the scalp in elderly individuals. A history of previous surgery at the same site – as observed in four of our patients – is common. Coronary artery disease, cerebrovascular insult, arterial hypertension, diabetes mellitus, and severe cases of cancer were frequent comorbidities. Most patients show an undulating clinical course despite topical anti‐inflammatory treatment; in some individuals, the lesions heal with scarring. Histopathology reveals scaly crusts or erosions and granulation tissue‐like changes in the dermis, evolving into a scar in more advanced stages. Apart from actinic/local damage, impaired immunity and microcirculation may be predisposing factors of the disease. Similar to pyoderma gangrenosum, EPDS must be considered in the context of nonhealing wounds in the elderly after the differential diagnoses mimicking EPDS have been ruled out. Given that previous or concomitant adjacent basal cell or squamous cell carcinoma is a common finding and that infiltrative variants extending beyond the clinically visible tumor may occur, histological mapping of the surrounding skin may be advisable in doubtful cases.     

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. As there are no guidelines for treating EPDS yet, we are a group of researchers in the U.K. who wanted to find out which treatments work best, by looking at all previously published research. We found that many different treatments have been used for EPDS, with the most common being steroid creams: 91.3% of patients improved completely or partially by using these creams, but they may also develop side effects such as skin thinning if used for too long. Other treatments that have been used include tablet steroids, antibiotics, tacrolimus cream, nonsteroidal drugs, zinc, calcipotriol cream, antifungals, retinoids, photodynamic therapy, dressings, grafts, methotrexate and tofacitinib. Some of these treatments worked better than others, and sometimes they were used in combination. Although the papers we looked at did not give enough information to give definite answers, we think that using a strong steroid cream, followed by tacrolimus cream (and possibly adding a zinc tablet), is the best way to treat EPDS. Photodynamic therapy (a type of treatment where a chemical is put on the skin and then a light is shone on it) also seems to work well. Linked Article:   Junejo et al. Br J Dermatol 2021; 184 :25–33 .     

Erosive pustular dermatosis of the scalp: a case report and review of the literature

Erosive pustular dermatosis of the scalp (EPDS) is a rare entity characterized by pustular, erosive and crusted lesions of the scalp with progressive scarring alopecia. The aetiology is unknown, but predisposing factors have been reported such as trauma, skin grafting, prolonged exposure to UV light of a bald scalp as well as co-existence of auto-immune diseases. Laboratory data, bacteriological and mycological investigations and histopathology are generally not diagnostic. A 45-year-old Caucasian man with 1-year-old pustular, erosive and crusted lesions on his bald scalp was seen. Laboratory data, including auto-immunity, bacteriological and mycological investigations were negative. Histopathology was not diagnostic showing a diffuse polymorphous infiltrate involving the dermis. A diagnosis of EPDS was made. The patient was treated with topical and systemic antibiotics and steroids as well as oral nimesulide with no or partial response. Consequently, isotretinoin (0.75 mg/kg/day) was started obtaining complete resolution in few months. No relapse after 1 year of follow-up was seen. EPDS represents a distinct disease with a history of relapsing and unsatisfactory response to common treatments. Systemic retinoids may be considered as a potentially resolutive choice.     

A Case of Pustular Ulcerative Dermatosis of the Scalp Associated with Ulcerative Skin Lesions on the Scrotum

A 79-year-old man developed pustules, erosions, and ulcer with severe pain on the parietal regions of the scalp and the scrotum. In another hospital, mycobacterium was detected from the pustules of the scalp at Gaffky scale 1, and this patient was referred to our clinic. In our hospital, repeated cultures of the pus from the skin lesions of the scalp were negative for mycobacterium. Biopsy specimens obtained from the skin lesions on the scalp disclosed ulcerative granulation with infiltration of inflammatory cells. Antibiotics and antituberculotic agents were ineffective. In contrast, the ulcerative skin lesions on the scalp and scrotum responded well to topical and systemic glucocorticosteroids. Although this patient developed pustular ulcerative skin lesions not only on the scalp but also on the scrotum, we considered this case to be pustular ulcerative dermatosis of the scalp developing ulcerative skin lesions on the scrotum.     

头皮糜烂性脓疱性皮病及其治疗

头皮糜烂性脓疱性皮病是一种少见的皮肤病.其临床特点为老年人头皮部红斑,无菌性脓疱,糜烂甚至溃疡,斑块结痂;伴疼痛为主;慢性病程易反复,最终可导致瘢痕性脱发,并可在瘢痕上发生鳞状细胞癌.其病因不明,目前认为相关的致病因素包括,头皮外伤、日光损伤及自身免疫性疾病.诊断为排除性,组织病理及微生物培养后排除其他疾病后方可诊断此病.已有文献报道多种治疗方法,其中以口服异维A酸疗效最佳.

Abstract：

Erosive pustular dermatosis of the scalp (EPDS ) is an uncommon and painful skin disease characterized by erythema,sterile pustules,erosion,ulceration,and crusted plaques of the scalp of elderly patients,which is often chronic and relapsing,and can eventually cause scarring alopecia and even squamous cell carcinoma.The etiology is unknown,but predisposing factors have been reported such as trauma,prolonged exposure to sunlight as well as coexistence of autoimmune diseases.EPDS is a diagnosis of exclusion based on histological examination and microbial cultures.Multiple therapies have been documented for the treatment of EPDS,and oral isotretinoin is supposed to be the most effective.     

Erosive pustular dermatosis of the scalp after zoster ophthalmicus and trauma]

Erosive pustular dermatosis of the scalp (EPDS), first described in 1979, is a rare, chronic, pustular condition with scarring alopecia, and nonspecific histological findings. While the initial responded to steroids is good, it can be treated successfully by oral administration of zinc sulphate. Local trauma has recently been suggested to play a role in the pathogenesis of EPDS. The differential diagnosis of EPDS includes folliculitis decalvans, sterile eosinophilic pustulosis Ofuji, pustular psoriasis vulgaris, trichophytosis, Perifolliculitis capitis abscedens et suffodiens, pemphigus vulgaris and cicatricial pemphigoid. We present the cases of a 74-year-old woman suffering from EPDS following herpes zoster ophthalmicus and of a 54-year-old man in whom EPDS followed a head injury.     

Localized erosive pustular dermatosis of the scalp at the site of a cochlear implant: successful treatment with topical tacrolimus

Erosive pustular dermatosis of the scalp (EPDS) is a rare form of nonmicrobial pustulosis mainly occurring in elderly patients with long-term sun damage to the skin. Clinically, it is characterized by pustular lesions that progressively merge into erosive and crusted areas over the scalp. The histology of EPDS is nonspecific, and its pathophysiology remains undetermined, with various types of local trauma possibly acting as the triggering factor. We describe a 24-year-old woman who developed EPDS after cochlear implant surgery for profound sensorineural hearing loss. We speculate that either the cutaneous surgery during cochlear implantation or the skin inflammation that commonly occurs near the magnet might have triggered the disorder. It is of note that the patient's skin lesions healed completely after treatment with topical tacrolimus, a relatively novel immunosuppressive molecule. Thus, topical tacrolimus may be indicated as a therapeutic alternative to the widely used steroids for this disease, mainly to avoid steroid-related cutaneous atrophy.     

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.     

Topical tacrolimus in the treatment of erosive pustular dermatosis of the scalp

BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is a rare condition characterized by chronic, sterile, pustular erosions leading to scarring alopecia. Although the etiology is unknown, it appears to be associated with ultraviolet light exposure and trauma. Histologic findings include nonspecific atrophy of the epidermis and chronic inflammation. CASE HISTORY: A 71-year-old female presented with a 1-year history of a boggy, erythematous, friable plaque on the vertex of her scalp. A diagnosis of EPDS was made based on presentation, negative cultures, and histologic findings. Initial therapy with intralesional and topical steroids and oral antibiotics resolved the inflamed plaques; however, steroid-induced atrophy became prominent after 5 months of use. The treatment was discontinued, resulting in recurrence of disease. Topical tacrolimus 0.1% ointment was initiated, which has been successfully controlling the lesions with reversal of skin atrophy and clinical evidence of hair growth. CONCLUSION: This is the fourth reported case of the successful treatment of EPDS with topical tacrolimus for the resolution of atrophy and the prevention of relapse of inflammation. Although its long-term use warrants close follow-up for side effects, tacrolimus may constitute a novel therapeutic option for the treatment of EPDS.     

Post-traumatic erosive dermatosis of the scalp: A hypergranulated variant

Erosive pustular dermatosis of the scalp is a slowly progressive chronic inflammatory disease that predominantly affects elderly male patients with marked actinic damage. The clinical evolution consists firstly of keratotic and erosive plaques surmounted by yellow-brown crusts and non-follicular pustules; later, the active crusting lesions regress in number and the scarring process causes diffuse cutaneous thinning and loss of hair follicles. However, manifestations may be atypical, leading to frequent misdiagnosis. We present a case series of post-traumatic erosive pustular dermatosis on the scalp of 4 elderly patients. The characterising feature was the presence of erosion consisting of abundant hypergranulation tissue, with an almost total lack of crusts and pustules. Dermoscopy showed a unique pattern of stretched and dilated linear, telangiectatic and polymorphous on-focus vessels, milky-red areas and white scarring areas. This clinical entity is rarely reported in the literature. The majority of reported cases were located on the legs.     

头屑异常增多的相关因素研究进展

头屑是一种常见的头皮症状,会造成头皮的不适,如瘙痒等,如果脱屑较多,可能影响患者的社交,严重者甚至会导致出现患者心理问题。本文就头屑异常增多的可能因素进行综述。     

Erosive pustular dermatosis of the scalp – an Australian perspective: Insights to aid clinical practice

Erosive pustular dermatosis of the scalp has particular relevance in Australia, due to its association with actinic damage. Despite its rarity, the recalcitrant nature of erosive pustular dermatosis of the scalp dictates a protracted recovery fraught with relapse and recurrence, posing inherent challenges to successful treatment and complete recovery. In Australia, erosive pustular dermatosis of the scalp is prevalent in the elderly, who are negatively affected due to the condition. We propose a management outline to aid clinical practice, to improve the quality of life in the elderly, whilst providing insight into the current understanding and treatment of erosive pustular dermatosis of the scalp.     

头皮糜烂性脓疱性皮病一例

患者,女,50岁。头皮红斑、脓疱伴疼痛6个月。皮肤科查体：头皮弥漫性分布紫红色浸润性斑片,其上见脓疱,局部见斑片状脱发区。实验室检查：取脓疱疱液多次行细菌及真菌培养均阴性。组织病理：表皮角化过度伴角化不全;真皮浅中层弥漫性淋巴细胞、浆细胞、中性粒细胞浸润。抗生素治疗效果不佳,口服糖皮质激素及外用他克莫司治疗后有效。诊断：头皮糜烂性脓疱性皮病。     

Erosive pustular dermatosis of the leg: long-term control with topical tacrolimus

Erosive pustular dermatosis of the leg is an unusual form of sterile pustulosis that typically affects the lower limbs of elderly patients. We report the cases of two women who developed erythematous skin plaques with pustules that coalesced and evolved into erosions and crusted areas. Histology showed epidermal spongiosis with subcorneal pustules and a dermal infiltrate with eosinophils and neutrophils. Lesions were treated with topical clobetasol propionate 0.05% for 10 days followed by topical tacrolimus daily until complete resolution, and then twice weekly for 1 year, without relapse. The response to topical corticosteroids and tacrolimus further support the close relationship with erosive pustular dermatosis of the scalp. Topical therapy with tacrolimus may offer good long-term disease control.     

Linear IgA dermatosis: Report of an infantile case and analysis of 213 cases in Japan

A 3‐year‐old boy presented with multiple vesicles, showing a rosette‐like arrangement around the crusts. Histopathological and immunohistochemical examinations demonstrated subepidermal blistering with neutrophilic infiltration associated with deposition of IgA, but not IgG, linearly distributed along the basement membrane zone (BMZ) of the epidermis. Indirect immunofluorescence revealed circulating antibodies (IgA class, ×160) against the BMZ of guinea pig lip skin. Based on the diagnosis of linear IgA dermatosis (LAD) of childhood, administration of dexamethasone (2 mg/day) was started, and the eruptions diminished immediately. Western blot analysis using extract of the HaCaT cell as a substrate, demonstrated the corresponding antigen at 120‐kDa molecular weight. There have been 213 cases of LAD reported in Japan including conference abstracts and these were studied to determine whether infantile cases differed from adult ones, and whether cases associated with IgG as well as IgA (IgA/G type), differed from the cases associated with IgA only (IgA type). IgG contributed less frequently to the infantile type (age of onset, ≤15 years) than to the adult type (age of onset, ≥16 years). Clinical appearance did not show any obvious difference between the IgA/G type and IgA type. However, three‐quarters of cases showing localization of antigen to the dermal side were the IgA/G type.     

Efficacy and safety of calcipotriol plus betamethasone dipropionate scalp formulation compared with calcipotriol scalp solution in the treatment of scalp psoriasis: a randomized controlled trial

Background Current topical therapies for scalp psoriasis are difficult or unpleasant to apply, resulting in decreased adherence and efficacy. Objectives To compare the efficacy and safety of once‐daily treatment with a combination of calcipotriol 50 μg g^?1 plus betamethasone 0·5 mg g^?1 (as dipropionate) (Xamiol^®; LEO Pharma A/S, Ballerup, Denmark) and twice‐daily calcipotriol 50 μg mL^?1 scalp solution in patients with scalp psoriasis. Methods This 8‐week, multicentre, randomized, investigator‐blind, parallel‐group study compared two‐compound calcipotriol/betamethasone scalp formulation with calcipotriol scalp solution in patients with moderately severe scalp psoriasis. Primary efficacy outcome was the proportion of patients who achieved ‘clear’ or ‘minimal’ disease severity according to investigator’s global assessment of disease severity at week 8. Secondary efficacy outcomes and adverse events were also evaluated. Relapse and rebound were assessed in an 8‐week, post‐treatment observation phase. Results In total, 207 patients received the two‐compound scalp formulation and 105 patients received calcipotriol scalp solution. The proportion of patients with ‘clear’ or ‘minimal’ disease at week 8 was significantly greater in the two‐compound scalp formulation group (68·6%) than in the calcipotriol scalp solution group (31·4%; P < 0·001). Improvement was more rapid with the two‐compound scalp formulation than with calcipotriol scalp solution. Further evidence of the superiority of the two‐compound scalp formulation over the scalp solution was demonstrated through greater improvements in clinical signs and fewer adverse events. Conclusions A once‐daily combination of calcipotriol plus betamethasone dipropionate was significantly more effective and better tolerated than twice‐daily calcipotriol scalp solution in the treatment of scalp psoriasis.