Keratosis pilaris atrophicans
- Megan N Landis, MD
Megan N Landis, MD
- Assistant Clinical Professor of Dermatology
- University of Louisville School of Medicine
Keratosis pilaris atrophicans is a group of related disorders characterized by inflammatory keratotic papules that may result in alopecia and scarring. They include keratosis pilaris atrophicans faciei (also called ulerythema ophryogenes), atrophoderma vermiculatum, and keratosis follicularis spinulosa decalvans .
This topic will review the clinical presentation and management of keratosis pilaris atrophicans. Keratosis pilaris is discussed separately. (See "Keratosis pilaris".)
Keratosis pilaris atrophicans is caused by abnormal keratinization of the follicular infundibulum, resulting in obstruction of the growing hair shaft and inflammation. Chronic inflammation leads to fibrosis, atrophy, shrinkage of the hair bulb, and alopecia. The association with several congenital syndromes due to partial monosomy or deletion in chromosome arm 18p suggests that the genes regulating the follicular keratinization may be located on chromosome 18p. (See 'Associated syndromes' below.)
The histopathologic features of keratosis pilaris atrophicans are nonspecific. In the early stages, there is an orthokeratotic keratin plug that blocks and dilates the orifice and upper portion of the follicular infundibulum (picture 1) . A twisted hair shaft may be trapped within this keratotic material and a mild perivascular mononuclear cell infiltrate is usually present in the adjacent dermis. In later stages, dermal fibrosis and loss of hair follicles may be seen.
There are three clinical variants of keratosis pilaris atrophicans: ulerythema ophryogenes, also called keratosis pilaris atrophicans faciei, atrophoderma vermiculatum, and keratosis follicularis spinulosa decalvans . Clinical features common to all forms include involvement of the face, in particular of the lateral eyebrows and cheeks; prominent erythema; and progression to an atrophic stage with loss of hairs in the involved areas .
- Callaway SR, Lesher JL Jr. Keratosis pilaris atrophicans: case series and review. Pediatr Dermatol 2004; 21:14.
- Ioffreda MD. Inflammatory diseases of the hair follicles, sweat glands, and cartilage. In: Lever's Histopathology of the Skin, 9th Edition, Elder D, Elenitsas R, Johnson BL, et al. (Eds), Lippincott Williams and Wilkins, Philadelphia 2005. p.469.
- Disorders of hair and nails. In: Hurwitz Clinical Pediatric Dermatology, 4th ed, Paller AS, Mancini AJ (Eds), Elsevier Saunders, New York 2011.
- Arnold AW, Buechner SA. [Keratosis pilaris and keratosis pilaris atrophicans faciei]. J Dtsch Dermatol Ges 2006; 4:319.
- Morton CM, Bhate C, Janniger CK, Schwartz RA. Ulerythema ophryogenes: updates and insights. Cutis 2014; 93:83.
- 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 2001; 38:127.
- Siegel DH, McKenzie J, Frieden IJ, Rauen KA. Dermatological findings in 61 mutation-positive individuals with cardiofaciocutaneous syndrome. Br J Dermatol 2011; 164:521.
- Guidry JA, Rees A, Chan AJ, et al. Ulerythema ophryogenes and Noonan syndrome. Dermatol Online J 2013; 19:14.
- Flórez A, Fernández-Redondo V, Toribio J. Ulerythema ophryogenes in Cornelia de Lange syndrome. Pediatr Dermatol 2002; 19:42.
- Gómez Centeno P, Rosón E, Peteiro C, et al. Rubinstein--Taybi syndrome and ulerythema ophryogenes in a 9-year-old boy. Pediatr Dermatol 1999; 16:134.
- Carvalho CA, Carvalho AV, Kiss A, et al. Keratosis pilaris and ulerythema ophryogenes in a woman with monosomy of the short arm of chromosome 18. An Bras Dermatol 2011; 86:S42.
- Chien AJ, Valentine MC, Sybert VP. Hereditary woolly hair and keratosis pilaris. J Am Acad Dermatol 2006; 54:S35.
- Luria RB, Conologue T. Atrophoderma vermiculatum: a case report and review of the literature on keratosis pilaris atrophicans. Cutis 2009; 83:83.
- Michaëlsson G, Olsson E, Westermark P. The Rombo syndrome: a familial disorder with vermiculate atrophoderma, milia, hypotrichosis, trichoepitheliomas, basal cell carcinomas and peripheral vasodilation with cyanosis. Acta Derm Venereol 1981; 61:497.
- Parren LJ, Frank J. Hereditary tumour syndromes featuring basal cell carcinomas. Br J Dermatol 2011; 165:30.
- van Dijk FS, Brittain H, Boerma R, et al. Atrophoderma Vermiculatum: A Cutaneous Feature of Loeys-Dietz Syndrome. JAMA Dermatol 2015; 151:675.
- Castori M, Covaciu C, Paradisi M, Zambruno G. Clinical and genetic heterogeneity in keratosis follicularis spinulosa decalvans. Eur J Med Genet 2009; 52:53.
- Bornholdt D, Atkinson TP, Bouadjar B, et al. Genotype-phenotype correlations emerging from the identification of missense mutations in MBTPS2. Hum Mutat 2013; 34:587.
- Fong K, Wedgeworth EK, Lai-Cheong JE, et al. MBTPS2 mutation in a British pedigree with keratosis follicularis spinulosa decalvans. Clin Exp Dermatol 2012; 37:631.
- Aten E, Brasz LC, Bornholdt D, et al. Keratosis Follicularis Spinulosa Decalvans is caused by mutations in MBTPS2. Hum Mutat 2010; 31:1125.
- Bellet JS, Kaplan AL, Selim MA, Olsen EA. Keratosis follicularis spinulosa decalvans in a family. J Am Acad Dermatol 2008; 58:499.
- Janjua SA, Iftikhar N, Pastar Z, Hosler GA. Keratosis follicularis spinulosa decalvans associated with acne keloidalis nuchae and tufted hair folliculitis. Am J Clin Dermatol 2008; 9:137.
- Goh MS, Magee J, Chong AH. Keratosis follicularis spinulosa decalvans and acne keloidalis nuchae. Australas J Dermatol 2005; 46:257.
- Richard G, Harth W. [Keratosis follicularis spinulosa decalvans. Therapy with isotretinoin and etretinate in the inflammatory stage]. Hautarzt 1993; 44:529.
- Oeffner F, Martinez F, Schaffer J, et al. Intronic mutations affecting splicing of MBTPS2 cause ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome. Exp Dermatol 2011; 20:447.
- Baden HP, Byers HR. Clinical findings, cutaneous pathology, and response to therapy in 21 patients with keratosis pilaris atrophicans. Arch Dermatol 1994; 130:469.
- Weightman W. A case of atrophoderma vermiculatum responding to isotretinoin. Clin Exp Dermatol 1998; 23:89.
- Apalla Z, Karakatsanis G, Papageorgiou M, et al. A case of atrophoderma vermiculatum responding to systemic isotretinoin. J Dermatol Case Rep 2009; 3:62.
- Alcántara González J, Boixeda P, Truchuelo Díez MT, Fleta Asín B. Keratosis pilaris rubra and keratosis pilaris atrophicans faciei treated with pulsed dye laser: report of 10 cases. J Eur Acad Dermatol Venereol 2011; 25:710.
- Rodríguez-Lojo R, Pozo JD, Barja JM, et al. Keratosis pilaris atrophicans: treatment with intense pulsed light in four patients. J Cosmet Laser Ther 2010; 12:188.
- Miranda BH, Farjo N, Farjo B. Eyebrow reconstruction in dormant keratosis pilaris atrophicans. J Plast Reconstr Aesthet Surg 2011; 64:e303.