Erosive pustular dermatosis of the scalp in an adolescent with near‐total hair regrowth: Case report and review of the literature

Erosive pustular dermatosis of the scalp (EPDS) is an uncommon chronic inflammatory response to scalp trauma that usually resolves with cicatricial alopecia. It most commonly affects elderly patients with a history of actinic damage. Herein, we describe a 16‐year‐old girl with acrofacial dysostosis type 1 presenting after surgery with crusting purulent scalp lesions, whose clinical presentation and histopathologic findings were consistent with EPDS. A review of the literature on EPDS in children is also detailed.     

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 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.     

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: 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.     

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.     

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.     

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.     

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 .     

THERAPEUTIC HOTLINE: Effectiveness of the association of cetirizine and topical steroids in lichen planus pilaris – an open‐label clinical trial

Lichen planus is considered a T cell‐mediated immunological disease. Even mast cells may contribute to the pathogenesis of the disease. Keratinocytes of the basal layer of the skin and/or the hair follicle may represent the “target/victim” of an immune aggression, determining the destruction of the hair follicle and thus scarring alopecia. Therefore, there is a compelling urgency for effective treatment of this potentially disfigurating dermatosis. Our data provide a further therapeutic opportunity: the use of an antihistaminic drug – cetirizine (CTZ) – in an “anti‐inflammatory” regimen. We propose the use of CTZ at the dosage of 30 mg/daily. Twenty‐one patients affected by lichen planus pilaris (LPP) of the scalp have been treated. Topical application of steroids has been coadministrated in all cases during the therapy. Clinical effects, in the sense of stabilization with cessation of the inflammation (erythema, follicular hyperkeratosis, loss of anagen hair), were achieved in all patients but three. One patient developed cardiac arrhythmia after 3 months of successful treatment and dropped out. Our cases indicate that a combined therapy of topical steroid with CTZ can be a safe and effective choice even in severe cases of lichen planus pilaris, so often refractory to the therapy.     

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.     

Erosive pustular dermatosis of the scalp successfully treated with oral prednisone and topical tacrolimus

Erosive pustular dermatosis of the scalp is a rare inflammatory disorder of the scalp, affecting elderly patients after local trauma and leading to scarring or cicatricial alopecia. Case Report: An elderly female patient complained of painful pustules on the parietal region bilaterally with progressive enlargement and ulceration. A biopsy suggested erosive pustular dermatosis of the scalp and the patient was treated with prednisone 40 mg/day and 0.1% topical tacrolimus. After 10 weeks complete closure of the eroded areas was observed and a stable scarring alopecia developed.     

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

Introduction::Erosive pustular dermatosis of the scalp (EPDS) is an uncommon condition with unknown etiology. The clinical exclusive diagnosis needs to be diffe...     

Erosive pustular dermatosis of the scalp may not be mutually exclusive with other dermatoses

Erosive pustular dermatosis of the scalp is an uncommon neutrophilic process representing a diagnostic and therapeutic challenge. It often occurs in older patients with prior sun exposure and manifests with hyperkeratosis and crust that may be difficult to distinguish from other inflammatory or neoplastic processes. Although erosive pustular dermatosis of the scalp may respond effectively to high potency topical steroids or other antiinflammatory regimens, caution is advised to avoid overlooking differential diagnoses that may not be mutually exclusive, especially squamous cell carcinoma.     

Evidence for a role of local trauma in the pathogenesis of erosive pustular dermatosis of the scalp

Eleven of 12 cases with erosive pustular dermatosis of the scalp gave a history of antecedent physical or infective trauma. We propose that this clinical disorder may be a non-specific inflammatory response to injury of ageing and sun-damaged scalps.     

Neutrophilic Dermatoses

Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses – conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis in Sweet syndrome and pyoderma gangrenosum; however, in subcorneal pustular dermatosis, they are found in the upper layers of the epidermis. Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful erythematous cutaneous lesions that have an infiltrate of mature neutrophils typically located in the upper dermis, and prompt clinical improvement following the initiation of systemic corticosteroid therapy. Classical, malignancy-associated, and drug-induced variants of Sweet syndrome exist. Pyoderma gangrenosum is characterized by painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema; its clinical variants include: ulcerative or classic, pustular, bullous or atypical, vegetative, peristomal, and drug-induced. Subcorneal pustular dermatosis is an uncommon relapsing symmetric pustular eruption that involves flexural and intertriginous areas; it can be idiopathic or associated with cancer, infections, medications, and systemic diseases. Since Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis share not only the same inflammatory cell but also similar associated systemic diseases, it is not surprising that the concurrent or sequential development of these neutrophilic dermatoses has been observed in the same individual. Also, it is not unexpected that several of the effective therapeutic interventions – including systemic drugs, topical agents, and other treatment modalities – for the management of these dermatoses are the same. The treatment of choice for Sweet syndrome and idiopathic pyoderma gangrenosum is systemic corticosteroids; however, for subcorneal pustular dermatosis, dapsone is the drug of choice. Yet, tumor necrosis factor-α antagonists are becoming the preferred choice when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Potassium iodide and colchicine are alternative first-line therapies for Sweet syndrome and indomethacin (indometacin), clofazimine, cyclosporine (ciclosporin), and dapsone are second-line treatments. Cyclosporine is effective in the acute management of pyoderma gangrenosum; however, when tapering the drug, additional systemic agents are necessary for maintaining the clinical response. In some patients with subcorneal pustular dermatosis, systemic corticosteroids may be effective; yet, systemic retinoids (such as etretinate and acitretin) have effectively been used for treating this neutrophilic dermatosis – either as monotherapy or in combination with dapsone or as a component of phototherapy with psoralen andUVAradiation. Topical agents can have an adjuvant role in themanagement of these neutrophilic dermatoses; however, high-potency topical corticosteroids may successfully treat localized manifestations of Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis. Intralesional corticosteroid therapy for patients with Sweet syndrome and pyoderma gangrenosum, hyperbaric oxygen and plasmapheresis for patients with pyoderma grangrenosum, and phototherapy for patients with subcorneal pustular dermatosis are other modalities that have been used effectively for treating individuals with these neutrophilic dermatoses.     

Tinea capitis inflamatoria por Trichophyton violaceum simulando dermatosis pustulosa erosiva

—The differential diagnosis of erosive pustular dermatosis of the scalp and inflammatory tinea capitis may be difficult on clinical grounds. Fungal cultures may be negative in some cases of scalp ringworm infection. This fact, together with the lack of specific diagnostic features for erosive pustular dermatosis, makes sometimes repeated cultures and a trial of griseofulvine convenient to differentiate both conditions.We present a 75-year old woman with a 3-month history of exudative alopecia affecting the scalp, negative fungal cultures, and a non-specific inflammatory infiltrate. We suggested the diagnosis of erosive pustular dermatosis of the scalp. However, repeated cultures yielded the growth of Trichophyton violaceum. The patient was treated with oral griseofulvine, with a dramatic and complete clearing of the pustular changes. Tinea capitis due to T. violaceum is considered to be an uncommon condition in adults from developed areas. Nevertheless, current migratory streams may account for the re-emergence of antropophilic dermatophytes in developed regions.     

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.     

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.