Ulerythema ophryogenes L66.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

keratosis pilaris faciei; Keratosis pilaris rubra atrophicans faciei; Keratosis pilaris rubra faciei; keratosis pilaris syndrome; Taenzer's disease; Unna-Taenzer's disease

History
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Taenzer, 1889; Unna, 1896

Definition
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Hereditary cornification disorder with follicular hyperkeratosis, vascular dilatation and consecutive follicular atrophy in the facial area (eyebrows). The entity is seen as a partial manifestation of the keratosis-pilaris group (see the explanations under keratosis-pilaris syndrome).

Etiopathogenesis
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Probably irregular autosomal dominant inheritance. So far, the gene locus is unknown. Associations with Noonan Syndrome have been described several times.

Manifestation
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Occurring in childhood or early adulthood. The disease can extend over an entire lifetime.

Localization
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Symmetrical in the eyebrow area, on cheeks, hairline, beard area.

Clinical features
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  • A flat, flat, symptomless, scaly erythema with 0.1 cm large, follicularly bounded hyperkeratosis, which always occurs symmetrically on both eyebrow regions and is initially limited to the lateral eyebrows.
  • With longer persistence, a persistent flat redness leads to a gradual loss of the eyebrows, starting laterally. The hair follicles also disappear completely.
  • In the course of years, analogous atrophying processes can also occur at the forehead-hairline(alopecia postmenopausale frontal fibrosis) and in the cheek area (see below erythema perstans faciei). In the cheek area, a deep red, symmetrical, persistent, flat erythema is impressive.
  • Accompanying: Accumulation of keratosis follicularis in the extremities.
  • Accompanying, although less frequent, is the occurrence of comedones and pustules(ulerythema acneiforme).

Histology
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Vascular dilation. Orthokeratotic hyperkeratosis in the acro-infundibula. Possibly perifollicular sclerosis, remains of follicles and sebaceous glands or absence of sebaceous glands.

Complication(s)
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Flat alopecia of the eyebrows.

External therapy
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  • Since it is a harmless disease with mainly cosmetically disturbing changes, only mild external therapy attempts should be used. Restrained and only short-term use of glucocorticoid-containing external preparations!
  • Keratolysis: At hyperkeratotic sites, keratolytic topicals (see keratolytics) such as 3-10% salicylic acid ointments/gels/collodions R215 R216 R227 (do not reach mucous membranes or eyes) or 0.05-0.1% vitamin A acid cream (e.g. R256, Cordes VAS cream) should be tried. Hydration with alpha-hydroxycarboxylic acid, lactic acid (e.g. Episoft A) and/or urea preparations(e.g. ureotope, carbamide, calmuride).
  • Anti-inflammatory: Glucocorticoids locally in fatty external preparations such as 0.25% prednicarbate (e.g. Dermatop Fatty Ointment), 0.1% methylprednisolone (e.g. Advantan Ointment) combined with urea and lactic acid preparations (e.g. Remederm, R104 ). Alternatively: combinations with hydrocortisone and urea (e.g. Hydrodexan). Also possible are glucocorticoid-containing intralesional injections with triamcinolone (e.g. Volon A crystal suspension or triamcinolone Lichtenstein diluted 1:3 with LA like scandicain). Cave! Painful!
  • Care: Accompanying oil baths (e.g. Lipikar, Balneum Hermal, Linola Fett N).
  • Hair area: Entering alopecia are irreversible, therefore early therapy with e.g. intralesionally applied glucocorticoids see above. In the final stage possibly hair replacement.

Internal therapy
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In severe progressive courses with generalized occurrence and progressive alopecia therapy in terms of the keratosis-pilaris syndrome. S.u. Keratosis pilaris syndrome.

Progression/forecast
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Over decades there was evidence of a chronically creeping (completely resistant to therapy) course. Sympotaxis only improves with increasing age. The process leads to irreversible (scarring) alopecia of the eyebrows.

Note(s)
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Ophryogenes ulerythema can occur as a partial manifestation of Noonan syndrome.

Literature
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  1. Azambuja R et al (1987) Ulerythema ophryogenes and folliculitis ulerythematosa reticulata. dermatologist 38: 411-413
  2. Burnett J W et al (1988) Ulerythema ophryogenes with multiple congenital anomalies. J Am Acad Dermatol 18: 437-440
  3. Guidry JA et al (2013) Ulerythema ophryogenes and Noonan syndrome. Dermatol Online J 19:14
  4. Li K et al (2013) Ulerythema ophryogenes, a rarely reported cutaneous manifestation of noonan syndrome: case report and review of the literature. J Cutan Med Surgery 17:212-218
  5. Patrizi A et al (2002) Ulerythema ophryogenes and keratosis pilaris. Eur J Dermatol 12: 572
  6. Snell JA et al (1990) Ulerythema ophryogenes in Noonan syndrome. Pediatric dermatol 7: 77-78
  7. Tänzer P (1889) On the ulerythema ophryogenes, a skin disease not yet described Monatsh prakt Dermatol (Hamburg) 8: 197-208
  8. Unna PG (1896) The Histopathology of the Diseases of the Skin. Clay, New York, S. 1086-1089

Disclaimer

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

Authors

Last updated on: 29.10.2020