Erosive pustular dermatosis of the scalp L73.8

Burton and Peye, 1977

Rare, etiologically unexplained, chronic, non-microbially induced, non-follicular pustulosis of the scalp (and legs) with consecutive patchy scarring alopecia, affecting mainly elderly individuals. Considered a controversial entity. Important: clinical separation from folliculitis decalvans necessary.

The etiology is unknown. No microbial component. but predisposing factors such as trauma, zoster, skin grafts, prolonged UV irradiation of a bald scalp, and coexistence of autoimmune diseases have been reported. Also occurred during therapy with EGFR inhibitors (genftinib). Laboratory data, bacteriological and mycological examinations, and histopathology are generally nondiagnostic (Mastroianni A et al. 2005) .

Mostly older persons (average age: 70 years); no definite sex preference (w>m?); the disease tends to occur in actinically damaged skin.

Focal, erythematous, hairless red plaques on the capillitium with central atrophy and usually marginal, follicularly bound papules and pustules. Severe pruritus. No response to antibiotic and -mycotic therapy.

Coronary artery disease, cerebrovascular insult, arterial hypertension, diabetes mellitus, and severe cases of cancer are common comorbidities (Wilk M et al (2018).

Bacteriological and mycological examinations of the pustule contents mostly negative (partial detection of Staphylococcus aureus, which many authors interpret as secondary infections).

Non-specific: Highly erect, non follicular bound intraepidermal to subcorneal pustules, adnexal reification, mononuclear infiltrate.

Folliculitis decalvans: follicular bound process

Primary bacterial folliculitis: follicular bound process; microbiological detection of bacteria

Psoriasis pustulosa generalisata: pustular formations always also outside the capillitium

Tinea capitis superficialis: rare in adults

Perifolliculitis capitis abscedens et suffodiens: follicularly bound process

Sterile eosinophilic pustulose (Ofuji): disseminated, very itchy and reddened papules and plaques with development of sterile (follicular) pustules. Confluence to larger foci is possible; also anular and polycyclic foci with central regression and peripheral progression may occur. Histologically eosinophilic dermatitis. Often also hematoeosinophilia.

Remove the crusts with aqueous quinolinol solution(e.g. Chinosol 1:1000). Then anti-inflammatory and drying external preparations such as glucocorticoid-containing tinctures, e.g. 0.1% triamcinolone tincture or 0.1% betamethasone tincture (e.g. Betnesol V crinale) as well as moist compresses with antiseptic additives such as potassium permanganate (light pink) or quinosol (1:1000).

If necessary experiment with Tacrolimus (Protopic 0.1%) or Calcipotriol (Psorcutan).

In addition to external treatment, oral zinc therapy (e.g., Zinkit 3, 3-4 times 1 drg. or 1 effervescent tablet/day p.o.). In cases of severe inflammation, glucocorticoids such as prednisone (e.g. Decortin) 40-80 mg/day can be used internally in the short term; rapid dose reduction.

In case of therapy resistance DADPS, alternatively: isotretinoin in usual dosage.

Experimental: A trial with dimethylfumarates could be considered in case of therapy resistance.

The clinical course is protracted with intermittent improvements and a tendency to scarring and consecutive alopecia (scarring alopecia of the Pseudopélade type - Wilk M et als. 2018).

65-year-old fair-skinned man with, photodamaged atopic scalp, with alopecia androgenetica; pustular, erosive and crusted plaques seen on hairless scalp for 1 year. Rarely itching. Painfulness when scratched over.

History was preceded by intense (occupational)UV exposure and laser surgical removal of multiple basal cell carcinomas.

Laboratory data, including autoimmunity, multiple bacteriological and mycological examinations were negative.

Histo: Histopathology was non-diagnostic and showed a diffuse polymorphic infiltrate in the dermis.

Treatment was with topical and systemic antibiotics and steroids and oral DADPS. The therapeutic success was only moderately good. Isotretinoin (0.75 mg/kg/day) was then started, which resulted in complete healing within a few months. No relapse was observed after a follow-up period of 1 year.

1. Bieber T et al (1987) Erosive pustular dermatitis of the capillitium. Dermatologist 38: 687-689
2. Burton JL (1977) Case for diagnosis. Pustular dermatosis of the scalp. Br J Dermatol 97: Suppl 15: 67-69
3. Boffa MJ (2003) Erosive pustular dermatosis of the scalp successfully treated with calcipotriol cream. Br J Dermatol 148: 593-595.
4. Di Lernia V et al (2016) Familial erosive pustular dermatosis of the scalp and legs successfully treated with ciclosporin. Clin Exp Dermatol 41:334-335.
5. Ena P et al (1997) Erosive pustular dermatosis of the scalp in skin grafts: report of three cases. Dermatology 94: 80-84
6. Karanfilian KM et al (2021) Erosive pustular dermatosis of the scalp: causes and treatments. Int J Dermatol 60:25-32.
7. Laffitte E et al (2003) Erosive pustular dermatosis of the scalp: treatment with topical tacrolimus. Arch Dermatol 139: 712-714.
8. Layton AM et al (1995) Erosive pustular dermatosis of the scalp following surgery. Br J Dermatol 132: 472-473.
9. Mastroianni A et al (2005) Erosive pustular dermatosis of the scalp: a case report and review of the literature. Dermatology 211:273-276.
10. Pagliarello C et al. (2015) Calcipotriol/betamethasone dipropionate ointment compared with tacrolimus ointment for the treatment of erosive pustular dermatosis of the scalp: a split-lesion comparison. Eur J Dermatol 25: 206-208.
11. Theiler M et al (2016) An Effective Therapy for Chronic Scalp Inflammation in Rapp-Hodgkin Ectodermal Dysplasia. Pediatr Dermatol 33: e84-e87.
12. Wilk M et al (2018) Erosive pustular dermatosis of the scalp: reappraisal of an understudied entity. JDDG 16: 15-20