Background
Ulerythema ophryogenes, a rare cutaneous disorder, is characterized by inflammatory keratotic facial papules that may result in scars, atrophy, and alopecia. This disorder has been described in association with other congenital anomalies such as Noonan syndrome, de Lange syndrome, and Rubinstein-Taybi syndrome.[1, 2, 3] Ulerythema ophryogenes falls within the broader category of keratosis pilaris atrophicans.[4]
In 1889, Tancer introduced the term ulerythema ophryogenes. In 1892, Unna coined the term. In 1925, Gans used the designation keratosis pilaris rubra atrophicans faciei.
Pathophysiology
Ulerythema ophryogenes is a relatively infrequent disorder that mainly affects children and young adults. It is usually sporadic, although cases inherited in an autosomal dominant pattern also are described. Although the condition is benign, it is still worrisome for parents. The inclusion of ulerythema ophryogenes into the concept of pilar keratoses has been proposed. Griffiths proposes the inclusion into the broader concept of keratosis pilaris atrophicans, while Dawber proposes the inclusion into scarring follicular keratosis.
Epidemiology
Frequency
United States
The frequency is not known.
International
Worldwide incidence rates are not known.
Mortality/Morbidity
The disorder is completely benign; it does not endanger the patient's life.
Race
Individuals of all races can be affected.
Sex
This condition can affect males and females.
Age
Children and young adults are most commonly affected with this condition.
Burnett JW, Schwartz MF, Berberian BJ. Ulerythema ophryogenes with multiple congenital anomalies. J Am Acad Dermatol. Feb 1988;18(2 Pt 2):437-40. [Medline].
Gómez Centeno P, Rosón E, Peteiro C, Mercedes Pereiro M, Toribio J. Rubinstein--Taybi syndrome and ulerythema ophryogenes in a 9-year-old boy. Pediatr Dermatol. Mar-Apr 1999;16(2):134-6. [Medline].
Snell JA, Mallory SB. Ulerythema ophryogenes in Noonan syndrome. Pediatr Dermatol. Mar 1990;7(1):77-8. [Medline].
Luria RB, Conologue T. Atrophoderma vermiculatum: a case report and review of the literature on keratosis pilaris atrophicans. Cutis. Feb 2009;83(2):83-6. [Medline].
Nazarenko SA, Ostroverkhova NV, Vasiljeva EO, Nazarenko LP, Puzyrev VP, Malet P, et al. Keratosis pilaris and ulerythema ophryogenes associated with an 18p deletion caused by a Y/18 translocation. Am J Med Genet. Jul 16 1999;85(2):179-82. [Medline].
Zouboulis CC, Stratakis CA, Rinck G, Wegner RD, Gollnick H, Orfanos CE. Ulerythema ophryogenes and keratosis pilaris in a child with monosomy 18p. Pediatr Dermatol. Jun 1994;11(2):172-5. [Medline].
Zouboulis CC, Stratakis CA, Gollnick HP, Orfanos CE. Keratosis pilaris/ulerythema ophryogenes and 18p deletion: is it possible that the LAMA1 gene is involved?. J Med Genet. Feb 2001;38(2):127-8. [Medline].
Klein OD, Cotter PD, Schmidt AM, et al. Interstitial deletion of chromosome 12q: genotype-phenotype correlation of two patients utilizing array comparative genomic hybridization. Am J Med Genet A. Nov 1 2005;138(4):349-54. [Medline].
Zegarska B, Kallas D, Schwartz RA, Czajkowski R, Uchanska G, Placek W. Graham-Little syndrome. Acta Dermatovenerol Alp Panonica Adriat. Oct 2010;19(3):39-42. [Medline].
Dajani ZA, Kerns MJ, Mutasim DF. Unilateral keratosis pilaris atrophicans faciei mimicking follicular mucinosis. J Am Acad Dermatol. May 2011;64(5):e71-2. [Medline].
Layton AM, Cunliffe WJ. A case of ulerythema ophryogenes responding to isotretinoin. Br J Dermatol. Nov 1993;129(5):645-6. [Medline].
Handrick C, Alster TS. Laser treatment of atrophoderma vermiculata. J Am Acad Dermatol. Apr 2001;44(4):693-5. [Medline].
Rodriguez-Lojo R, Pozo JD, Barja JM, et al. Keratosis pilaris atrophicans: treatment with intense pulsed light in four patients. J Cosmet Laser Ther. Aug 2010;12(4):188-90. [Medline].
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