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 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 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.     

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: 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.     

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.     

Sarcoidosis presenting as non‐scarring non‐scalp alopecia

In this article we describe a 39‐year‐old man who presented with non‐scarring non‐scalp alopecia of his limbs as the initial presentation of sarcoidosis. Alopecia is a rare cutaneous manifestation of sarcoidosis. A literature review has found only one other example of sarcoidosis presenting as non‐scarring non‐scalp alopecia in an area other than the scalp in a patient who was otherwise asymptomatic. Several reported cases have described scarring alopecia of the scalp, which is the area of skin most commonly affected by sarcoidosis. There has been one documented case of sarcoidosis manifesting as total body non‐scarring alopecia in a patient who had systemic symptoms of sarcoidosis. Other cases have presented rare cutaneous manifestations of sarcoidosis but in all these cases several other organ systems have been involved, and the patient has had systemic symptoms on presentation or the cutaneous presentation did not include non‐scalp non‐scarring alopecia.     

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.     

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.     

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.     

Testicular choriocarcinoma with cutaneous metastasis in a 19‐year‐old man

A 19‐year‐old man suffering from testicular choriocarcinoma presented to the dermatology department with a cutaneous metastasis on his head. This metastasis was the first sign of disease that led to medical consultation. Histopathology revealed cytotrophoblasts and syncytiotrophoblasts, the later expressing human chorionic gonadotropin antigen. Whole body computed tomography showed multiple metastases of the brain, lung, liver, bone, paraaortic lymph nodes and left uvea; the primary was found in the left testicle. Despite neurosurgical intervention and chemotherapy the patient died 9 days after the biopsy of the cutaneous metastasis. Cutaneous metastases of testicular choriocarcinoma are exceptionally rare, with fewer than a dozen cases reported in the English‐language literature. The present case highlights that testicular choriocarcinoma metastatic to the skin should be included in the differential of cutaneous scalp tumors.     

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.     

Plica “Neuropathica” in an 11‐Month‐Old Boy

Plica neuropathica is an acquired, irreversible tangling and matting of scalp hair presenting as discrete masses. Many cases are reported in the literature, but it has never been reported in an infant. Herein we report the first case of plica neuropathica in an 11‐month‐old.     

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...     

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 .     

CD30‐positive primary cutaneous anaplastic large cell lymphoma with coexistent pseudocarcinomatous hyperplasia

CD30‐positive primary cutaneous anaplastic large cell lymphoma (C‐ALCL) is an indolent type of cutaneous lymphoma with favourable clinical prognosis. Pseudocarcinomatous hyperplasia (PCH) is a rare benign epithelial condition that can resemble invasive squamous cell carcinoma both clinically and histopathologically. PCH predominantly occurs in CD30‐positive lymphoproliferative disorders. We report a 75‐year‐old woman with PCH in a multifocal C‐ALCL located on the scalp and right retroauricular area, which rapidly responded to treatment with psoralen ultraviolet A photochemotherapy. Comprehensive virological analyses for potential oncogenic viruses, including Epstein–Barr virus, human herpesvirus‐8, human papillomaviruses, the recently discovered cutavirus and nine different human polyomaviruses, were negative.     

Apocrine hidrocystoma on the genitalia of a 9‐year‐old girl

Apocrine hidrocystomas are mostly found on the cheeks and eyelids but also on the scalp and neck. The age distribution is from 30 to 70 years old. We report a case of an apocrine hidrocystoma on the genitalia of a 9‐year‐old girl.     

Successful Treatment of Vitiligo on the Scalp of a 9‐Year‐Old Girl Using Autologous Cultured Pure Melanocyte Transplantation

Leukotrichia frequently accompanies vitiligo on hairy areas such as the scalp. Treatment with conventional medical therapy is usually unsuccessful because of deficiencies in the melanocyte reservoir. We describe transplantation of autologous cultured pure melanocytes for scalp vitiligo with leukotrichia in a 9‐year‐old girl, resulting in almost complete and stable repigmentation of skin and hair.     

Angioedema After Squaric Acid Treatment in a 6‐Year‐Old Girl

Alopecia areata (AA) involves the immune‐related destruction of hair follicles, resulting in patches of complete hair loss, most often on the scalp. The topical sensitizer squaric acid dibutylester (SADBE) is a popular treatment option given its low side‐effect profile, hair regrowth potential, and lack of cross‐reactivity with other chemicals. We describe a unique case of a 6‐year‐old girl who developed angioedema after SADBE treatment for AA.     

Concurrent Hidradenitis Suppurativa,Inflammatory Acne,Dissecting Cellulitis of the Scalp,and Pyoderma Gangrenosum in a 16‐Year‐Old Boy

We report the case of a 16‐year‐old boy who presented to our clinic with concomitant hidradenitis suppurativa, inflammatory acne, dissecting cellulitis of the scalp, and pyoderma gangrenosum. Recent reports describe the co‐occurrence of pyoderma gangrenosum, acne, and hidradenitis suppurativa. This case further expands the spectrum of concomitant pyoderma gangrenosum and dissecting cellulitis of the scalp.