Erosive pustular dermatosis of the capillitium L73.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 18.12.2020

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Synonym(s)

Dermatosis erosive pustular of the head; EPDK; Erosive pustular dermatosis of the capillitium; Erosive pustular dermatosis of the head; Erosive pustular dermatosis of the scalp; Pustular ulcerative dermatosis of the scalp

History
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Burton and Peye, 1977

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

Etiopathogenesis
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Unsolved. No microbial component. The occurrence after zoster, skin grafts or other surgical interventions has been described. Also occurred under therapy with EGFR inhibitors (genftinib).

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

Clinical features
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Herd-shaped, erythematous, hairless areas on the capillitium with central atrophy and mostly marginal, follicularly bound papules and pustules. Severe itching. No response to antibiotic and fungal therapy.

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

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

Differential diagnosis
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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.

External therapy
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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).

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

Progression/forecast
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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).

Literature
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  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. Laffitte E et al (2003) Erosive pustular dermatosis of the scalp: treatment with topical tacrolimus. Arch Dermatol 139: 712-714
  7. Layton AM et al (1995) Erosive pustular dermatosis of the scalp following surgery. Br J Dermatol 132: 472-473
  8. 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
  9. Theiler M et al (2016) An Effective Therapy for Chronic Scalp Inflammation in Rapp-Hodgkin Ectodermal Dysplasia. Pediatric Dermatol 33: e84-e87.
  10. Wilk M et al (2018) Erosive pustular dermatosis of the scalp: re-evaluation of an underrated entity. JDDG 16: 15-20

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 18.12.2020