Ulerythema ophryogenes L66.4

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).

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

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

• 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).

Flat alopecia of the eyebrows.

• 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.

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.

Ophryogenes ulerythema can occur as a partial manifestation of Noonan syndrome.

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