Acne keloidalis nuchae L73.0

Authors: Prof. Dr. med. Peter Altmeyer, Prof. Dr. med. Martina Bacharach-Buhles

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Last updated on: 16.03.2021

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Synonym(s)

acne keloidalis; Acne sclerotisans nuchae; Folliculite pilo-sebacée chronic; Folliculitis nuchae sclerotisans; Folliculitis scleroticans nuchae; keloidal folliculitis; Neck Keloid; papillary dermatitis capillitii (Kaposi); sycosis framboesiformis (Hebra); Sycosis nuchae sclerotisans

History
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Hebra and Kaposi, 1860

Definition
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Eminently chronic fibrosing and indurating folliculitis and perifolliculitis of the neck region, leading to irreversible hair loss and progressive, extensive, bulging keloid scarring.

Etiopathogenesis
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An initial staphylococcal folliculitis after short haircut or shaving is discussed. There is no pathogenetic relationship to acne (localization, absence of comedones). Due to an unknown cause, a transition into a chronic fibrosing inflammation with tissue hyperplasia and a reactive pressure atrophy of the follicles occurs.

Manifestation
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Occurring only in males. Onset after puberty. People with dark skin color are affected more often (peculiarities of the hair structure).

Localization
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Neck, neck hairline, possible extension to the back of the head.

Clinical features
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Initially individual, small, red, follicular papules and pustules, from which develop hard, dark red, hemispherical, shiny, differently sized, solid papules and nodules pierced by a terminal or vellus hair, which can confluent to form larger plate-like structures.

Later increasing sclerotic, almost board-like hardening of the skin between them.

Beam-like, transverse, coarse, bulging keloids with tufts of hair.

Deep epithelium-lined fistula ducts.

Peripheral continuous sometimes intermittent progression of the sclerosing inflammation over a period of years.

Differential diagnosis
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Furuncle: Highly painful, acute onset; originating from 1 follicle; no multiple pustules on the surface; markedly fluctuant.

Folliculitis decalvans: Eminently chronic course of the disease usually over years; scarring alopecia; highly red skin lesions in the center usually atrophic shiny and marginal standing follicular papules; later pustular transformation and crusting. Irregularly shaped foci of scarring with small-spotted, irreversible hair loss result. Formation of tufted hairs.

Tinea capitis profunda: Mostly occurring in children! Subacute or acute course; itchy or moderately painful, 0.5-5.0 cm in size, usually well demarcated, cushion-like raised, purulent, bright red plaques or nodules. Pus may be discharged on pressure. Hairs are absent from the lesion; hairs that still exist can be easily and usually painlessly extracted; preparation of a native specimen for fungal detection.

Therapy
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Intralesional injections with glucocorticoids such as triamcinolone acetonide (e.g. Volon A 10 crystal suspension) diluted 1:3 to 1:5 with physiological NaCl solution or local anaesthetic such as mepivacaine, several times at intervals of weeks. In inflammatory phases, if necessary, local disinfectant solutions polihexanide (Serasept, Prontoderm), polyvidone-iodine solution (e.g. Betaisodona).

Small lesions should be excised en bloc, larger lesions should be covered with a full-thickness skin graft or stretch plastic.

Treatment with tetracyclines (e.g. Tetracycline Wolff) 4 times/day 250 mg p.o. or with isotretinoin (e.g. Isotretinoin-ratiopharm; Aknenormin) 0.5 mg/kg bw/day p.o. Caution! Women of childbearing age!

Progression/forecast
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Chronic recurrent course.

Literature
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  1. Adamson HG (1914) Dermatitis papillaris capillittii (Kaposi). Acne keloid. Br J Dermatol 26: 69-83
  2. Alexis A et al (2014) Folliculitis keloidalis nuchae and pseudofolliculitis barbae: are prevention and effective treatment within reach? Dermatol Clin 32:183-191
  3. Gloster HM Jr (2000) The surgical management of extensive cases of acne keloidalis nuchae. Arch Dermatol 136: 1376-1379
  4. Hebra v. F, Kaposi M (1860) Textbook on skin diseases. Enke, Erlangen, vol. 1, p. 506
  5. Kaposi M (1894) On some unusual forms of acne (folliculitis). Arch Dermatol Syph 26: 87-96
  6. Kelly AP (2003) Pseudofolliculitis barbae and acne keloidalis nuchae. Dermatol Clin 21: 645-653
  7. Sparrow LC (2000) Acne keloidalis is a form of primary scarring alopecia. Arch Dermatol 136: 479-484
  8. Shapero J et al (2011) Acne keloidalis nuchae is scar and keloid formation condary
    to mechanically induced folliculitis. J Cutan Med Surg 15:238-240

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