Acral lesions in tuberous sclerosis complex: insights into pathogenesis.
BACKGROUND
Patients with tuberous sclerosis complex (TSC) are predisposed to developing ungual fibromas and other acral lesions.


OBJECTIVE
We sought to determine the numbers, types, and locations of acral skin lesions in TSC.


METHODS
We examined and photographed 76 adult women with TSC.


RESULTS
The age of the patients ranged from 20 to 69 years, with a mean age of 39 +/- 11 years. Ungual fibromas were observed in 61 of 76 patients (80%). Periungual fibromas were more common than subungual fibromas, were more common on the feet than the hands, and showed the greatest frequency on the fifth toe. Longitudinal grooves in the nails occurred with or without a visible fibroma. Longitudinal short red streaks--lesions that we term "red comets"--were observed in 22 patients (29%). Longitudinal leukonychia was observed in 14 patients (18%). One patient had isolated digital overgrowth and one patient had pachydermodactyly.


CONCLUSIONS
No men or children were included in this study.


CONCLUSIONS
Examination of patients for skin lesions of TSC could be improved by including inspection for longitudinal nail grooves, red comets, longitudinal leukonychia, and splinter hemorrhages in addition to ungual fibromas. The anatomic distribution of TSC ungual fibromas is not random and appears consistent with trauma-promoted tumor formation.
Aldrich
Capt Shelley L
CS
Department of Dermatology, Uniformed Services University of the Health Sciences, Bethesda, Maryland 20814, USA.
Hong
Chien-Hui
CH
Groves
Leslie
L
Olsen
Cara
C
Moss
Joel
J
Darling
Thomas N
TN
D000328:Adult; D000368:Aged; D005260:Female; D005350:Fibroma / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008875:Middle Aged; D009260:Nail Diseases / Q000209:etiology* / Q000473:pathology; D009262:Nails / Q000473:pathology; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology; D014402:Tuberous Sclerosis / Q000150:complications* / Q000473:pathology; D055815:Young Adult
Arch Dermatol. 1995 Dec;131(12):1465-6
7492148
J Am Acad Dermatol. 2007 Aug;57(2):189-202
17637444
J Am Acad Dermatol. 2004 Feb;50(2):289-92
14726890
Arch Dermatol. 2008 Jul;144(7):886-92
18645140
J Med Genet. 1968 Dec;5(4):273-80
5713638
J Eur Acad Dermatol Venereol. 2003 Jan;17(1):100-1
12602986
Proc Natl Acad Sci U S A. 1971 Apr;68(4):820-3
5279523
J R Soc Med. 1994 Jul;87(7):419-20
8046733
Australas J Dermatol. 2002 May;43(2):81-90; quiz, 91-2
11982563
Am J Clin Dermatol. 2003;4(2):97-105
12553850
Postgrad Med J. 1964 Oct;40:595-600
14220406
J R Coll Physicians Lond. 1988 Oct;22(4):240-3
3230540
J Dermatol. 2004 Dec;31(12):993-7
15801264
Br J Dermatol. 1992 Mar;126(3):275-82
1554604
Adv Dermatol. 2006;22:181-200
17249302
Genet Med. 2007 Feb;9(2):88-100
17304050
J Child Neurol. 1998 Dec;13(12):624-8
9881533
Dermatol Surg. 2008 Mar;34(3):364-9
18177395
Br J Dermatol. 1996 Jul;135(1):1-5
8776349
Cutis. 2007 Aug;80(2):137-40
17944173
J Exp Med. 2005 Sep 5;202(5):617-24
16129702
Arch Dermatol. 1962 Feb;85:209-26
14479476
Sarcoidosis Vasc Diffuse Lung Dis. 2005 Dec;22 Suppl 1:S49-66
16457017
Proc Natl Acad Sci U S A. 2008 Mar 4;105(9):3539-44
18292222
Clin Exp Dermatol. 1993 Mar;18(2):146-7
8481991
J Invest Dermatol. 2001 Nov;117(5):1027-35
11710909
Int J Dermatol. 1998 Dec;37(12):911-7
9888331
Ann Intern Med. 2005 Aug 16;143(4):313-4
16103482
Australas J Dermatol. 2002 Aug;43(3):157-68; quiz 169-70
12121391
N Engl J Med. 2006 Sep 28;355(13):1345-56
17005952
J Am Acad Dermatol. 1988 Feb;18(2 Pt 1):369-72
3346418
Arch Dermatol. 2004 Oct;140(10):1253-7
15492189
J Am Acad Dermatol. 2007 May;56(5):786-90
17239986