Table of Contents
What Is Erosive Pustular Dermatosis of the Scalp?
Erosive pustular dermatosis of the scalp (EPDS) is a rare, inflammatory, chronic, slow-onset disease that primarily affects elderly males with sun-damaged and bald scalps. However, cases have been documented in people as young as six months. Burton originally described this in 1977. The lesions are sterile pustules, erosions, thick yellow crusts, and superficial ulcerations on an atrophic skin bed.
Alopecia with scarring results from EPD's damage to hair follicles. It can sometimes be seen on the legs of those with atrophic skin and venous hypertension and may cause itching and pain. Due to its nonspecific clinical symptoms, clinical diagnosis may be difficult since it mimics more frequent disorders such as scarring alopecia, bullous diseases, and scalp infections.
Histology can reveal different results, depending on the stage of the disease. For example, EPDS may have an atrophic epidermis and chronic inflammation with neutrophils, lymphocytes, and occasionally large cells from foreign bodies. Although histology is required for the definitive diagnosis, dermoscopy has recently been reported as a helpful diagnostic tool since it demonstrates absent follicular ostia with severe skin atrophy and the resulting visibility of dermal veins. Tacrolimus, calcipotriol, and topical and systemic corticosteroids are some treatment options. EPDS may need long-term management.
What Caused Erosive Pustular Dermatosis of the Scalp?
Even if the exact etiology of the syndrome is still unknown, actinic damage, immunosenescence, androgenetic alopecia, trauma, hormones, and autoimmune disorders have all been identified as predisposing factors for EPDS. Additionally, reports of EPDS developing following photodynamic therapy (PDT) or topical Ingenol mebutate treatment exist. Mechanical trauma is probably the most frequent initiating trigger; however, stopping the stress does not cause the EPDS to resolve. Usually, there is a median interval of six months between the triggering event and the start of the first EPDS symptoms.
The most commonly recognized theory holds that physical harm to the actinic or atrophic skin results in chronic ulcers or delayed wound healing, which triggers abnormal neutrophil chemotaxis and immune dysregulation against unidentified skin antigens. An early stage of wound healing is characteristic of EPD. Delayed healing may be caused by a combination of factors, including chronic inflammation, poor restorative capacity of elderly keratinocytes, estrogen deficiency, and a paucity of hair bulge stem cells. Numerous studies have examined the connection between hair follicles and the emergence of EPDS, and some writers have concentrated on the immunologic dysregulation that trauma-induced hair follicles induce, leading to persistent inflammation.
What Are the Signs and Symptoms of Erosive Pustular Dermatosis of the Scalp?
The clinical presentation of EPDS is frequently confused with various hair and scalp conditions, ranging from infections to inflammatory conditions to cancerous growths on the scalp. Proper examination and biopsy are necessary for a differential diagnosis of tumors, and it is essential for all EPDS patients who are not responding to treatment.
The clinical presentations of EPDS are as follows:
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Numerous large, crusty, red patches affect the bald parts of the scalp, primarily the edges, with pustules surrounding and overlaying them and serum discharge.
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The erosion may be hidden by tissue with excessive granulation.
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An ongoing cycle of healing and recurrence in the affected areas causes atrophic skin with new pustules and erosions in the center or around the edges of the original lesion.
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The bumps develop into greenish-brown or yellow-brown crusts or lakes of pus. If the pustules are removed, the skin beneath may appear red and wet. Scarring and widespread baldness can be the direct effects of significant illness.
How is Erosive Pustular Dermatosis of the Scalp Diagnosed?
A physician diagnoses EPD clinically. The time to diagnosis may be extended due to frequent confusion with infectious or malignant processes. The lesions' bacterial and fungal cultures are usually negative.
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Biopsy - To rule out other diseases that show similarly, it is crucial to biopsy the lesions. Subcorneal pustules, epidermal hypertrophy, or atrophy and erosions are seen in histopathology. One significant distinction from bacterial folliculitis is that if the pustules occur, they are not follicular. A polymorphous cutaneous inflammatory infiltration consisting of neutrophil leukocytes, lymphocytes, and specific plasma cells may accompany these findings.
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Other Special Tests - The doctor may use additional specialized testing, such as Tzanck smears and immunofluorescence studies, to rule out an autoimmune blistering illness.
How Is Erosive Pustular Dermatosis of the Scalp Treated?
EPD is a chronic illness, so it needs to be managed over an extended period.
Tropical Therapy:
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Corticosteroids - High-potency topical corticosteroids are comparatively safe and effective first-line treatments for EPD. The primary drawback of this treatment is the potential for skin atrophy, which must be considered when using it over an extended period. Furthermore, reports of relapse following the cessation of topical steroids have been noted.
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Topical Tacrolimus - An alternative to topical steroids with comparable efficacy is topical tacrolimus 0.1% ointment. When applied once or twice a day, it works well for lesions on the legs and scalp. Treatment results show improvement within 1-2 weeks; however, literature reports indicate that full resolution may take up to 16 weeks. Compared to topical corticosteroids, tacrolimus has the advantage of not causing skin atrophy.
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Topical Calcipotriol - For a patient with alopecia with scalp EPD, topical calcipotriol 0.005% improved significantly after eight weeks of treatment, with partial hair regrowth after 12 weeks.
Photodynamic Treatment
A patient receiving photodynamic therapy showed notable improvement, yet there have been indications that the therapy may also cause EPD. Although photodynamic therapy is a potentially useful third-line treatment option, it should be utilized cautiously due to inconsistent reports.
Surgery
Surgery has been employed on occasion because the scalp's EPD mimics some characteristics of field cancerization and squamous cell carcinoma. Split-skin grafting and deep excisions achieve quick healing. Naturally, this should not be used as a first-line treatment.
Conclusion:
Erosive pustular dermatosis of the scalp is an unusual condition that mainly affects older males with bald scalps caused by photodamage. Pustules, erosions, and hemorrhagic crusts on atrophic skin are typically associated with it. Clinicians find it difficult to diagnose and give it little thought because it has similar clinical characteristics to other frequent cutaneous disorders like skin cancer. With better clinical understanding and histopathologic features, physicians can differentiate this condition from other similar situations and provide appropriate treatment.
