Pustular Dermatosis of the Scalp Associated with Autoimmune Diseases

Abstract

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.

Erosive Pustular Dermatosis of the Scalp Following Herpes Zoster: Successful Treatment with Topical Tacrolimus

“…Moreover, autoimmune disorders have been described in association with EPDS. Therefore, it has been suggested that physical trauma to the skin might cause auto-antibodies production against skin structures, resulting in a secondary inflammatory reaction 8,9 . The fact that EPDS responds to topical antiinflammatory agents supports this hypothesis.…”

Erosive Pustular Dermatosis of the Scalp: A Case Report and Review of the Literature

“…The pathogenesis remains unknown, but predisposing factors such as recent or past local trauma, skin grafting and excessive exposure to sunlight on the bald scalp [12] have been reported. Furthermore, auto-immune diseases have been described in association with EPDS such as rheumatoid arthritis [13], Hashimoto’s thyroiditis, auto-immune hepatitis and Takayasu’s aortitis [15], suggesting the possibility of a common immunological dysfunction. It can be supposed that physical damage to the skin could induce the production of auto-antibodies against epidermal and dermal structures with a secondary inflammatory reaction; furthermore, EPDS responsiveness to steroids and anti-inflammatory drugs may confirm this auto-immune hypothesis.…”

“…According to most authors, these organisms represent secondary colonization rather than primary infection without playing a role in the pathogenesis of EPDS as it is confirmed by unsuccessful results of all therapeutic attempts with antibacterial or antimycotic drugs. Laboratory data are usually normal even if sometimes elevated values of inflammation markers (erythrocyte sedimentation rate, C-reactive protein, polyclonal hypergammaglobulinaemia) and rarely the presence of various auto-antibodies (rheumatoid factor, thyroglobulin and microsomal antibodies, liver antibodies and so on) are described [2, 13, 14]. …”

“…It affects prevalently elderly white women. Predisposing factors such as local trauma, skin grafting and prolonged exposure to sunlight on the bald scalp [12] and associations with auto-immune diseases [13, 14] have been described, even if the causes and pathogenesis still remain uncertain.…”

“…Pustules are generally sterile even if sometimes swabs from the pustular or crusted areas are positive for bacterial or mycological colonization [2]. Laboratory tests are usually normal except for the presence of significant inflammation markers and the possible finding of auto-antibodies [13]. …”