Hyperkeratosis follicularis et parafollicularis in cutem penetrans L87.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.04.2022

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Synonym(s)

Cyrle disease; Hyperkeratosis Cyrle; hyperkeratosis penetrans; Kyrle's disease; Kyrle Syndrome; M. Kyrle

History
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Kyrle, 1916

Definition
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Very rare disease (Omin 149500) characterized by follicular and perifollicular papules with firmly attached horn support, especially on the legs.

Etiopathogenesis
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In elderly patients, associations with diabetes mellitus and chronic renal insufficiency have been described, as well as triggering by dialysis. However, these cases are probably attributable to"reactive perforating collagenosis"!

Manifestation
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First manifestation in childhood or younger adulthood. Gynecotropy(?).

Localization
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Especially on the flexor sides of the lower legs and on the buttocks. Hands, feet and hairy head remain free. Generalized forms, which also affect the trunk, indicate internal concomitant diseases.

Clinical features
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Asymptomatic, isolated, occasionally grouped, follicular, yellowish to brown-red, sometimes swollen horn nodules. Confluence with larger polycyclic, hyperkeratotic plaques is possible. Easily detachable horny cones, which leave a bowl-shaped crater when detached. Healing with superficially pigmented scars. No itching. The Koebner phenomenon is negative.

Histology
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Invaginated epithelial plug with formation of a crater filled with parakeratotic horn masses. Focal perforation of the epidermis with focal, inflammatory reaction of the dermis with lymphocytes, neutrophilic granulocytes and a few giant cells of the foreign body type.

External therapy
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Keratolytic, e.g. with external retinoids R256, salicylic acid-containing external R227 or urea-containing external R102.

Internal therapy
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A proven effective therapy is not known: an experiment with acitretin (neotigason) can be performed, first for about 6 weeks in high doses of 0.5-1 mg/kg bw/day, then reduction to maintenance dose of 0.25-0.5 mg/kg bw/day. Successful therapy attempts with allopurinol (e.g. Zyloric) are described on the basis of casuistics (level of evidence IV).

Operative therapie
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Possibly curettage or excision of the herd. Try cryosurgery.

Progression/forecast
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Chronic progressive course. Healing with scarring.

Literature
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  1. Ataseven A et al (2014) Kyrle's disease. BMJ Case Rep doi: 10.1136/bcr-2013-009905.
  2. Cunningham MB et al. (1987) Kyrle's disease. J Am Acad Dermatol 16: 117-123
  3. Detmar M, Ruszczak Z, Imke E et al (1990) Kyrle disease in juvenile diabetes mellitus and chronic renal failure. Z Hautkr 65: 53-61
  4. Iyoda M et al (2003) Acquired reactive perforating collagenosis in a nondiabetic hemodialysis patient: successful treatment with allopurinol. Am J Kidney Dis 42: E11-13
  5. Kruger K et al (1999) Acquired reactive perforating dermatosis. Successful treatment with allopurinol in 2 cases. dermatologist 50: 115-120
  6. Kyrle J (1916) On an unusual case of universal follicular and parafollicular hyperkeratosis (hyperkeratosis follicularis et parafollicularis in cutem penetrans). Arch Derm Syph 123: 466-493
  7. Rallis E et al (2014) Squamous cell carcinoma developed on Kyrle's disease scar. J BUON 19:317-318

Disclaimer

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

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Last updated on: 29.04.2022