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: 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 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.     

Recurrence of erosive pustular scalp dermatosis after a skin graft]

INTRODUCTION: Erosive pustular dermatosis of the scalp is a rare and chronic dermatosis of unknown etiology with non specific histology and without effective treatment. It affects mostly old women. We have observed a 80 year-old male suffering from an erosive pustular dermatosis of the scalp following application of 5 p. 100 fluorouracile cream (Efudex) and his resistance to various treatments including skin graft. OBSERVATION: An 80 year-old man had been suffering, for many years from recurrent episodes of pustules, erosions and crusts of the scalp, following treatment with 5 p. 100 fluorouracile cream for skin lesions diagnosed as actinic keratosis. Different topical and systemic treatments were tried without much improvement. A skin graft of the scalp lesional area was finally done, showing a severe recurrence as a Koebner's reaction. Despite this recurrence, we have observed an easier control with a topical mixture of steroid and antibiotic. CONCLUSION: This recurrence of erosive pustular dermatosis of the scalp following skin graft had never been previously observed before, showing that removing affected skin did not control the disease, suggesting that anti-inflammatory agents probably originating from trauma persist.     

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.     

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 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.     

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 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.     

Pentoxyfilline as a treatment for subcorneal pustular dermatosis

Subcorneal pustular dermatosis (SPD) is a rare pustular neutrophilic dermatosis in which groups of sterile pustules appear in the superficial (subcorneal) skin. This chronic condition can be associated with significant morbidity and decreased quality of life. Dapsone is the first‐line therapy for SPD, but some patients fail to respond or cannot tolerate it. In these instances, patients may be treated with second‐line therapies such as phototherapy, topical corticosteroids, or systemic agents including glucocorticoids, acitretin, immunosuppressive, or biologic medications. These therapies may not always be efficacious and can be associated with intolerable adverse effects. Here, we report a case of a patient who sustained long‐term remission and no side effects with the novel use of pentoxifylline, a tumor necrosis factor‐alpha inhibitor, as monotherapy. Pentoxifylline should be considered as a possible therapy in patients with SPD intolerant to dapsone.     

High‐Potency Topical Steroids: An Effective Therapy for Chronic Scalp Inflammation in Rapp–Hodgkin Ectodermal Dysplasia

Chronic erosive pustular dermatitis with a predilection for the scalp is a hallmark of ectodermal dysplasias (EDs) caused by mutations in TP63, including Rapp–Hodgkin and Hay–Wells EDs. It is among the most troublesome and symptomatic complications and is typically refractory to classic wound care approaches. We report two cases of Rapp–Hodgkin ED with refractory scalp erosions that markedly improved with the use of potent topical steroids. We also note marked similarities between this scalp inflammation and “erosive pustular dermatosis of the scalp,” a condition more typically found in elderly individuals with severe scalp sun damage, and speculate about possible shared pathogenetic mechanisms.     

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.     

Eosinophilic pustular folliculitis in infancy: an unusual case

INTRODUCTION: Eosinophilic pustular folliculitis in children is a follicular inflammatory dermatosis, usually occurring early in life. The disease progresses in flares of prurigenous plaques studded with papules and sterile pustules of the scalp and other areas of the skin. OBSERVATION: A 7 year-old boy presented with itching papular vesicular and pustular plaques on the scalp and the face. Pigmented plaques with pustular border, located on the trunk, were associated with pustular and erosive lesions of the side of the lower lip and in the nostrils. A specimen taken from the pustules did not show bacterial or fungal infection. Histologic examination of a biopsy specimen showed subcorneal pustules with eosinophilic and neutrophilic infiltrates of follicles. Clinical improvement was obtained only by the combination of steroids and dapsone, but recurrence followed withdrawal of treatment. DISCUSSION: Eosinophilic pustular folliculitis in children is rare. Our case report combines features of the infancy form (lesions located on the scalp and face) and the adult form (location on the trunk and limbs with annular distribution), expressing the conceptual confusion that remains between both forms. The mucosal involvement seen in our patient has never been reported in the literature neither in the infancy nor in the adult form.     

Erosive pustular dermatosis—a common development in atrophic skin

Erosive pustular dermatosis (EPD), an ulcerated eruption with loosely attached scale overlying macerated tissue with pustulation, has been described on the scalp, mainly in women, and responds to topical steroids.¹ However, many patients with long-standing damaged skin show identical changes, particularly well seen around stasis ulcers.
Thirty consecutive patients with venous ulceration were examined for the changes of erosive pustular dermatosis using a 9-point check list which included pustulation, loose scale, maceration etc. Twenty-eight patients gave a history of varicose veins and five of deep venous thrombosis.
Seventeen patients showed definite clinical changes of erosive pustular dermatosis, four showed some evidence and nine showed no changes.
Patient details are shown in the Table.  

  TABLE 1.   Patient details     

15.

Dermatosis pustulosa erosiva del cuero cabelludo     

《Actas dermo-sifiliográficas》2003,94(3):161-164

—Erosive pustular dermatosis of the scalp is a disease characterized by eroded, crusted and pustular lesions resulting in scarring alopecia. It usually occurs in 60 to 90 year old patients. Histopathology is nonspecific, showing an atrophic, eroded epidermis, subcorneal pustules, and an infiltrate of lymphocytes, plasmocytes and, less frequently, neutrophils and giant cells. Inmunofluorescence and microbiological studies are negative. The differential diagnoses include bacterial or fungal infections, discoid lupus erythematosus, lichen planus, and cicatricial pemphigoid. The treatment is difficult, and potent topical steroids under occlusion are sometimes effective. Oral zinc sulphate, isotretinoin, dapsona have been anecdotally active.We report two patients with pustular dermatosis confined to the scalp, who developed chronic, extensive, pustular, crusted lesions of the scalp which produced scarring alopecia. Studies were essentially negative and skin biopsies showed only nonspecific changes. The condition did not respond to antibiotics, but was suppressed by potent topical steroids.     

16.

Erosive pustular dermatosis of the scalp: reappraisal of an underrecognized entity          下载免费PDF全文

Michael Wilk  Bettina G. Zelger  Undine Hauser  Reinhard Höpfl  Bernhard Zelger 《Journal der Deutschen Dermatologischen Gesellschaft》2018,16(1):15-19

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.     

17.

Pustular ulcerative dermatosis of the scalp     

W.K. JACYK 《The British journal of dermatology》1988,118(3):441-444

Six young African patients are described with erosive pustular scalp lesions leading to extensive ulceration. No bacterial or fungal cause was found. Biopsies showed non-specific changes of atrophy with subacute or chronic inflammation. Four patients were malnourished and anaemic and three were infested with hookworm. The condition did not respond to antibiotics. Healing was obtained in one patient with systemic steroids. The relationship of this disorder to erosive pustular dermatosis of the scalp and to pyoderma gangrenosum of atypical distribution is discussed.     

18.

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

Dall'Olio E  Rosina P  Girolomoni G 《The Australasian journal of dermatology》2011,52(1):e15-e17

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.     

19.

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

Christopher Teng  JiaDe Yu  Jesse Taylor  Adam I. Rubin  James R. Treat 《Pediatric dermatology》2019,36(5):697-701

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.     

20.

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

Rudy Yeh  Margit Polcz  David Wong 《The Australasian journal of dermatology》2019,60(4):e272-e278

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.     

Dermatosis pustulosa erosiva del cuero cabelludo

—Erosive pustular dermatosis of the scalp is a disease characterized by eroded, crusted and pustular lesions resulting in scarring alopecia. It usually occurs in 60 to 90 year old patients. Histopathology is nonspecific, showing an atrophic, eroded epidermis, subcorneal pustules, and an infiltrate of lymphocytes, plasmocytes and, less frequently, neutrophils and giant cells. Inmunofluorescence and microbiological studies are negative. The differential diagnoses include bacterial or fungal infections, discoid lupus erythematosus, lichen planus, and cicatricial pemphigoid. The treatment is difficult, and potent topical steroids under occlusion are sometimes effective. Oral zinc sulphate, isotretinoin, dapsona have been anecdotally active.We report two patients with pustular dermatosis confined to the scalp, who developed chronic, extensive, pustular, crusted lesions of the scalp which produced scarring alopecia. Studies were essentially negative and skin biopsies showed only nonspecific changes. The condition did not respond to antibiotics, but was suppressed by potent topical steroids.     

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.     

Pustular ulcerative dermatosis of the scalp

Six young African patients are described with erosive pustular scalp lesions leading to extensive ulceration. No bacterial or fungal cause was found. Biopsies showed non-specific changes of atrophy with subacute or chronic inflammation. Four patients were malnourished and anaemic and three were infested with hookworm. The condition did not respond to antibiotics. Healing was obtained in one patient with systemic steroids. The relationship of this disorder to erosive pustular dermatosis of the scalp and to pyoderma gangrenosum of atypical distribution is discussed.     

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.     

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 – 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.