Ulerythema ophryogenes

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Ulerythema ophryogenes ( ancient Greek οὐλή oúlē " scar ", ἐρύθημα erúthēma " reddening ", ὀφρύς ophrýs " eyebrow " and -genes ), also: Keratosis pilaris atrophicans faciei ; older: hornbill, Taenzer's disease, Unna 's disease -Taenzer 's disease is a sub-form of keratosis pilaris atrophicans , which also includes the clinical pictures of Atrophoderma vermiculata and Keratosis follicularis spinulosa decalvans .

It is a hereditary skin disease that occurs in children and leads to extensive reddening of the skin, excessive cornification of the hair follicles and scarring of the affected skin areas on the face, especially in the area of ​​the temples. The course of the disease is protracted, but often comes to a standstill with puberty. Treatment options include preparations containing cortisone , salicylic acid and vitamin A acid and, in severe cases, the superficial ablation of the diseased skin by grinding, cold or laser.

Classification according to ICD-10
L66.4 Atrophodermia vermiculata
Folliculitis ulerythematosa reticulata
Ulerythema acneiforme
ICD-10 online (WHO version 2019)

distribution

The disease affects children, with skin changes already present at birth or developing in early childhood. Children with an increased tendency to allergic diseases ( atopy ) fall ill more often than healthy children.

Occasionally the disease occurs together with other hereditary diseases, for example in the context of Noonan , Woolly-Hair, Rubinstein-Taybi or Cornelia de Lange syndrome , as well as cardiofaciocutaneous syndrome or partial monosomy 18 .

Cause and origin

By a probable irregular autosomal dominant inherited genetic defect (to be discussed, inter alia, for desmoglein 4 and the lrp1 encoding genes) it comes to hyperkeratosis (cornification excessive), thereby to seal the hair follicles. This leads to an inflammatory reaction which, if it persists for a long time, causes scarring and the follicle to die.

Clinical appearance

Preferably on the temples, but also on the cheeks, forehead and neck, hair follicles blocked by a horn plug appear as small papules on reddened, scaly skin. If it persists for a long time, the areas will scar. On hairy skin this leads to a loss of hair, typically the lateral parts of the eyebrows. On the extensor sides of the arms and thighs, there can also be follicle-bound papules in the sense of a keratosis follicularis.

Differential diagnoses

  • Atrophoderma vermiculata: A sub-form of keratosis pilaris atrophicans with more severe scarring and therefore a pit-shaped ("worm-like", Latin vermis , "worm" ) retraction of the skin around the hair follicle, is also considered to be the final state of keratosis pilaris atrophicans
  • Keratosis follicularis spinulosa decalvans : Subtype of keratosis pilaris atrophicans with manifestations on the scalp
  • Keratosis pilaris rubra faciei: variant of keratosis pilaris with isolated reddening of the skin without scarring or hair loss
  • Erythromelanosis follicularis faciei et colli: variant of keratosis pilaris, which is associated with increased pigmentation of the skin
  • Frontal fibrosing alopecia: variant of the lichen planopilaris with manifestations along the hairline of the forehead
  • Lupus erythematosus : autoimmune disease associated with butterfly-shaped reddening of the facial skin and excessive keratinization of the hair follicles

Investigation methods

As a rule, the diagnosis can be made during the clinical examination by the dermatologist. The removal of a tissue sample for the histological examination under the microscope is not necessary in most cases, especially since it is painful for the children concerned and, given the removal point on the face, possibly cosmetically impairing.

pathology

Histologically, the hair follicles are enlarged due to excessive cornification. In the upper dermis (dermis) there are minor inflammatory infiltrates of lymphocytes and histiocytes around the affected hair follicles, which, if left in place for a long time, lead to fibrosis (increase in connective tissue) and atrophy (shrinkage) of the follicular structures. There is also little inflammatory infiltration of the dermis in the surrounding tissue of superficial small blood vessels.

treatment

Since it is primarily a cosmetically impairing disease with a mostly harmless course, cautious therapy with few side effects should be sought. As effectively have keratolytic agents such as salicylic acid , vitamin A acid , lactic acid or urea proved cream, optionally in combination with an anti-inflammatory effective cortisone ointments. Refatting oil baths are also used for care.

In the case of early onset of hair loss, therapy with intralesional cortisone injection (injection of cortisone into the changed skin areas) should be carried out if necessary. In severe cases, vitamin A acid and cortisone can be used systemically (in tablet form); there are also the options of dermabrasion (micro-grinding process), cryosurgery (freezing) and laser treatment .

Prospect of healing

The course of the disease is chronic, with slowly increasing scarring of the affected skin areas and frequent resistance to therapy. When puberty is reached, the disease usually comes to a standstill.

Web links

  • Ulerythema ophryogenes. Clinical appearance photos. In: DERMATOLOGY ATLAS. Samuel Freire da Silva, accessed June 15, 2020 .

Individual evidence

  1. ^ Duden : The dictionary of medical technical expressions , 4th edition, Georg Thieme Verlag , Stuttgart, New York 1985, ISBN 3-411-02426-7 , p. 699.
  2. ^ Otto Dornblüth : Dictionary of clinical art expressions , Verlag von Veit & Comp., Leipzig 1894 ( Willibald Pschyrembel , 1st edition), p. 141.
  3. ↑ Ludwig August Kraus: Kritisch-etymologisches medicinisches Lexikon , 3rd edition, Verlag der Deuerlich- und Dieterichschen Buchhandlung, Göttingen 1844, p. 692 and 1072.
  4. ^ Willibald Pschyrembel : Clinical Dictionary , 123rd - 153rd edition, Walter de Gruyter , Berlin 1959, p. 908.
  5. ↑ Paul Taenzer: About Ulerythema ophryogenes, a skin disease not yet described , in: " MONTHS FOR PRACTICAL Dermatology", Volume 8, 1889, pp. 197-208.
  6. ↑ Günter Thiele (Ed.): Handlexikon der Medizin , Urban & Schwarzenberg , Volume 4 (S − Z), Munich, Vienna, Baltimore 1980, p. 2529.
  7. ↑ RL Mertens: ulerythema Ophryogenes and atopy . In: Archives of Dermatology . tape 97 , no. 6 , June 1968, ISSN  0003-987X , pp. 662-663 , PMID 5652971 .
  8. ^ JA Snell, SB Mallory: Ulerythema ophryogenes in Noonan syndrome . In: Pediatric Dermatology . tape 7 , no. 1 , March 1990, ISSN  0736-8046 , p. 77-78 , doi : 10.1111 / j.1525-1470.1990.tb01080.x , PMID 2343011 .
  9. ↑ VS Neild, JS Pegum, RS Wells: The association of keratosis pilaris atrophic and woolly hair, with and without Noonan's syndrome . In: The British Journal of Dermatology . tape 110 , no. 3 , March 1984, ISSN  0007-0963 , pp. 357-362 , doi : 10.1111 / j.1365-2133.1984.tb04644.x , PMID 6696850 .
  10. Jump up ↑ P. Gómez Centeno, E. Rosón, C. Peteiro, M. Mercedes Pereiro, J. Toribio: Rubinstein - Taybi syndrome and ulerythema ophryogenes in a 9-year-old boy . In: Pediatric Dermatology . tape 16 , no. 2 , March 1999, ISSN  0736-8046 , p. 134-136 , doi : 10.1046 / j.1525-1470.1999.00032.x , PMID 10337678 .
  11. ↑ Angeles Flórez, Virginia Fernández-Redondo, Jaime Toribio: Ulerythema ophryogenes in Cornelia de Lange syndrome . In: Pediatric Dermatology . tape 19 , no. 1 , January 2002, ISSN  0736-8046 , p. 42-45 , doi : 10.1046 / j.1525-1470.2002.00003.x , PMID 11860570 .
  12. ^ JF Reynolds, G. Neri, JP Herrmann, B. Blumberg, JG Coldwell: New multiple congenital anomalies / mental retardation syndrome with cardio-facio-cutaneous involvement - the CFC syndrome . In: American Journal of Medical Genetics . tape 25 , no. 3 , November 1986, ISSN  0148-7299 , pp. 413-427 , doi : 10.1002 / ajmg.1320250303 , PMID 3789005 .
  13. ↑ CC Zouboulis, CA Stratakis, G. Rinck, RD Wegner, H. Gollnick: Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p . In: Pediatric Dermatology . tape 11 , no. 2 , June 1994, ISSN  0736-8046 , p. 172-175 , doi : 10.1111 / j.1525-1470.1994.tb00575.x , PMID 8041661 .
  14. ^ E Cohen-Barak, N Danial-Farran, H Hammad, O Aleme, J Krauz: Desmoglein 4 Mutation Underlies Autosomal Recessive Keratosis Pilaris Atrophicans . In: Acta Dermato Venereologica . tape 98 , no. 8 , 2018, ISSN  0001-5555 , p. 809-810 , doi : 10.2340 / 00015555-2976 .
  15. ↑ Joakim Klar, Jens Schuster, Tahir Naeem Khan, Muhammad Jameel, Katrin Mäbert: Whole exome sequencing identifies LRP1 as a pathogenic gene in autosomal recessive keratosis pilaris atrophicans . In: Journal of Medical Genetics . tape 52 , no. 9 , September 2015, ISSN  1468-6244 , p. 599-606 , doi : 10.1136 / jmedgenet-2014-102931 , PMID 26142438 .
  16. ↑ a b Eduardo Calonje, Thomas Brenn, Alexander Lazar, Steven D. Billings: McKee's pathology of the skin with clinical correlations . Fifth ed. Without place, ISBN 978-0-7020-7552-0 , p. 79 (1085350565 [accessed June 15, 2020]).
  17. ↑ Olivia Egan: Keratosis pilaris atrophicans faciei. In: DermNetNZ. Retrieved June 15, 2020 .
  18. ^ A b c Peter Altmeyer: Ulerythema ophryogenes. In: Altmeyer's Encyclopedia. Retrieved June 15, 2020 .
  19. ↑ Peter Altmeyer: Keratosis pilaris syndrome. In: Altmeyer's Encyclopedia. Retrieved June 15, 2020 .