Folliculitis decalvans L66.2

Charles Quinquaud 1888

Rare, eminently chronic follicular inflammation of the capillitium, with inflammatory follicular papules and pustules, which leads to destruction of the hair follicles and scarring and perpetuates itself. The resultant condition is a mirror-like, scarred alopecia with resulting pseudopélade state, s.a. Folliculitis sycosiformis atrophicans. Characteristic is the detection of Staphylococcus aureus from pustule contents and the appearance of tuft hairs.

Unexplained, possibly infection with Staphylococcus aureus (S. aureus is almost always detectable from pustules), possibly immunodeficiencies. Familial clusters are observed in isolated cases, but are the exception. In rare cases an association with acne vulgaris is detectable (see case report).

Preferably occurring in adulthood (20-40 years).

Men are affected much more often than women (ratio about 5:1).

capillitium:

• Parietal region (by far the most common region)
• Temple region

Facial area: Similar skin changes can also occur in the facial area (folliculitis decalvans faciei).

Eminent chronic inflammatory, pyogenic, folliculitis of the capillitium.

Often continuous or intermittent course of the disease for years (sometimes decades).

Initially disseminated, small, follicular, moderately painful, discrete red papules. In the course of the disease progression, increasing inflammatory symptoms with follicular pustules, incipient spot-like, later also extensive, mirror-like skin atrophy. Keratotic hair shaft incisions near the hairline at the edge of the lesions are characteristic. Rice field-like conglomerate follicles are formed (tuft hair formation).

After the formation of a central, plate-like, mirror-like scarring (shiny skin), folliculitis is only found in the peripheral zone of the lesions. These are the sign of the progression of the disease.

Findings: The typical symptoms are a few or numerous, irregularly shaped, atrophic, 2.0-5.0 cm large, hairless, plate-like, sunken scar foci with a peculiarly reflective, atrophic glossy skin which is particularly conspicuous under lateral illumination and observation.

Formation of so-called tufted hairs, of peculiarly large-lumen, dilated pores from which 5-15 tightly set hair bundles emerge.

The edges of the lesions appear frayed with an active papulo-pustular inflammatory border.

Fresh lesion: Perifollicular abscesses of poly- and mononuclear cells, destruction of the hair follicles. Older lesions show perifollicular granulation tissue consisting of lymphocytes, fibroblasts and plasma cells, often with giant foreign body cells.

In burnt out foci, only fibrosis with sparse lymphocytic infiltrates is seen.

Scarring alopecia of other aetiology

Rule out tinea capitis.

Disinfecting Exteriors: with antimicrobial additives such as diluted potassium permanganate solution(light pink), hydroxyquinoline e.g. quinolinol (e.g. Chinosol 1:1000), R042, Clioquinol cream R049

Alternative antibiotics: solutions containing erythromycin(e.g. R086, acne mycin solution).

Alternative glucocorticoids: topical glucocorticoids as creams or solutions, e.g. betamethasone 0.05% lotio or ointment(betamethasone valerate emulsion hydrophilic), possibly under occlusive conditions. Also alternately with topical antibiotics

Calcineurin inhibitors: Single results are available for Tacrolimus.

Tetracycline (e.g. Tetracycline-Wolff) initially 1 g/day p.o., later reduction to 500 mg/day. Therapy trial with isotretinoin (e.g., isotretinoin-ratiopharm; acnenormin) 0.5 mg/kg bw/day.

In some cases, good therapeutic effects are described with the combination of clindamycin and rifampicin (Bunagan MJ et al. 2015): Clindamycin/rifampicin 300mg 2x/day for 10 weeks (Vañó-Galván S et al 2015).

In case of more severe inflammation, short-term glucocorticoids in medium dosage 60-80 mg/day (e.g. Decortin H) in decreasing dosage.

Single case reports showed positive success with combination therapy of dapsone (100 mg/day) and isotretinoin (1 mg/kgKG/day) and adalimumab (Shireen F et al. 2018).

In the rare combination with acne vulgaris, therapy with isotretinoin is recommended.

Also reported in the literature are successes from therapy with PDT with Metvix® and LED red light, 37 J/cm², 4x at 4-week intervals.

A casuistry reports on the successful therapy with Apremilast

It is recommended to remove the tuft hairs surgically, as they are the entry point for bacteria. A disinfectant shampoo should be used to prevent recurrence. Blow dry the scalp after washing the head.

1. Bolz S et al (2008) Successful combined isotretinoin and dapsone therapy for perifolliculitis capitis abscedens et suffodiens. JDDG 6: 44-47
2. Bunagan MJ et al (2015) Retrospective Review of Folliculitis Decalvans in 23 Patients with Course andTreatment
   Analysis of Long-standing Cases.J Cutan Med Surg 19:45-49.
3. Chandrawansa PH et al (2003) Folliculitis decalvans--a retrospective study in a tertiary referred centre, over five years. Singapore Med J 44: 84-87
4. Vañó-Galván S et al (2015) Folliculitis decalvans: a multicentre review of 82 patients. J Eur Acad Dermatol Venereol 29:1750-1757.
5. Karakuzu A et al (2001) A case of folliculitis decalvans involving the beard, face and nape. J Dermatol 28: 329-331
6. Kaur S, Kanwar AJ (2002) Folliculitis decalvans: successful treatment with a combination of rifampicin and topical mupirocin. J Dermatol 29: 180-181
7. Powell JJ et al (1999) Folliculitis decalvans including tufted folliculitis: clinical, histological and therapeutic findings. Br J Dermatol 140: 328-333
8. Quinquaud CE (1888) Folliculite épilate décalvante. Réunions clin Hôpital St. Louis, Comptes rendus (Paris) 9: 17.
9. Shireen F et al.(2018) A Case of Isotretinoin Therapy-Refractory Folliculitis Decalvans TreatedSuccessfully
   with Biosimilar Adalimumab (Exemptia).Int J Trichology 10:240-241.
10. Vañó-Galván S et al (2015) Folliculitis decalvans: a multicentre review of 82 patients. J Eur Acad Dermatol Venereol 29: 1750-1759.
11. Fässler M et al (2020) Successful treatment of refractory folliculitis decalvans with apremilast. JAAD Case Rep. 20;6:1079-1081.