Erosive pustular dermatosis of the scalp and multiple sclerosis: just a coincidence?

Abstract

We report the case of a 23-year-old woman with an erythematous, crusted patch of the scalp, lacking pustular lesions, with partial hair loss, developed after mechanical scalp trauma. Histopathological examination showed a dermal infiltrate, predominantly peri-adnexal and peri-vascular, rich in plasma cells and lymphocytes, but lacking neutrophils, possibly as a consequence of the time elapsed since the onset of the skin disease. Reduction of functional hair follicles was evidenced. Stains for bacterial or fungal infections were negative. Direct immunofluorescence was negative. Erosive pustular dermatosis of the scalp was diagnosed on the basis of clinical-anamnestic findings, supported by histology. Topical clobetasol propionate led to clinical improvement after a couple of weeks. Erosive pustular dermatosis of the scalp (EPDS) was diagnosed and soon after the patient developed also multiple sclerosis. Up to date, the pathogenesis of erosive pustular dermatosis of the scalp remains unknown, though a possible role of immunosenescence and autoimmunity has been suggested. Indeed, high levels of neutrophil-stimulating cytokines and chemokines have been found in erosive pustular dermatosis patients, possibly causing activation of an aberrant systemic neutrophilic reaction. Furthermore, the role of neutrophils has been recently highlighted also in the pathogenesis of multiple sclerosis. Herein we hypothesize a possible common immunological etiology of multiple sclerosis and erosive pustular dermatosis of the scalp, conceivably involving a hyperactivation of neutrophils.

Erosive Pustular Dermatosis of the Scalp in a Patient With Vitiligo Successfully Treated With Topical Antibiotics

“…Some examples of skin insults include burns, physical injury, cryotherapy, topical treatments such as 5-fluorouracil, imiquimod, minoxidil, and tretinoin, and systemic medications such as afatinib, gefitinib, and erlotinib [1,2,5,6]. It can be associated with autoimmune disorders, including Hashimoto's thyroiditis, autoimmune hepatitis, Takayasu's arteritis, rheumatoid arthritis, and myasthenia gravis [1][2][3]. To the best of our knowledge, this is the only case to be reported in a patient with vitiligo.…”

“…Erosive pustular dermatosis is an uncommon chronic inflammatory skin disorder [1]. The lesions typically present on the scalp of elderly patients with sun-damaged skin and hair loss [1][2][3][4]. It develops over months to years, presents as asymptomatic erosions covered with a yellow-crusted discharge, and is frequently associated with pustules, skin atrophy, and scarring alopecia [1][2][3][4].…”

“…The lesions typically present on the scalp of elderly patients with sun-damaged skin and hair loss [1][2][3][4]. It develops over months to years, presents as asymptomatic erosions covered with a yellow-crusted discharge, and is frequently associated with pustules, skin atrophy, and scarring alopecia [1][2][3][4]. There are several precipitating factors for the development of erosive pustular dermatosis, all of which ultimately lead to skin injury [1,2,5].…”

“…There are several precipitating factors for the development of erosive pustular dermatosis, all of which ultimately lead to skin injury [1,2,5]. An association with multiple autoimmune conditions has been reported [1][2][3].…”

Immunosenescence and Skin: A State of Art of Its Etiopathogenetic Role and Crucial Watershed for Systemic Implications

“…Very recently, a well-founded hypothesis has emerged to further support the etiopathogenetic interplay involving cutaneous immunosenescence and systemic immunosenescence. It proposes that an aberrant release of neutrophil-stimulating cytokines and chemokines may result in putative neutrophilic hyperactivation, which serves as an etiologically common immunologic trigger for erosive pustular dermatosis of the scalp (EPDS) and two autoimmune neuropathies: multiple sclerosis and myasthenia gravis [181]. The interplay between cutaneous and systemic immunosenescence is represented in Figure 5.…”