Content

»	Syndromes with Premature Aging
	»	Werner Syndrome (Adult Progeria)
	»	Progeria (Hutchinson–Gilford Syndrome)
»	Disorders with Immunodeficiency
	»	Familial Chronic Mucocutaneous Candidiasis (FCMC)
	»	Hyper-IgE Syndrome (Job Syndrome)
	»	Wiskott–Aldrich Syndrome (WAS)
	»	Severe Combined Immunodeficiency (SCID)
	»	Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED)
»	Disorders of Pigmentation
	»	Oculocutaneous Albinism (OCA)
	»	Chédiak–Higashi Syndrome
	»	Hermansky–Pudlak Syndrome
	»	Griscelli Syndrome
	»	Hypomelanosis of Ito
	»	Incontinentia Pigmenti (Bloch–Sulzberger Syndrome)
	»	Piebaldism
	»	Waardenburg Syndrome
»	Disorders with Pigmented Lesions
	»	McCune–Albright Syndrome (Polyostotic Fibrous Dysplasia)
	»	Russell–Silver Syndrome
»	Vascular Disorders
	»	Sturge–Weber Syndrome (SWS)
	»	Klippel–Trénaunay Syndrome (KTS)
	»	Proteus Syndrome
	»	Cobb Syndrome
	»	Von Hippel–Lindau Syndrome (VHL)
	»	Beckwith–Wiedemann Syndrome
	»	Rubinstein–Taybi Syndrome
	»	Mafucci Syndrome
	»	Blue Rubber Bleb Nevus Syndrome (Bean Syndrome)
	»	Cornelia de Lange Syndrome
	»	Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu)
	»	Hereditary Lymphedema (Milroy Disease)
	»	Lymphedema–Distichiasis Syndrome
	»	Noonan Syndrome
	»	Turner Syndrome
	»	Meige Lymphedema (Hereditary Lymphedema II)

Sturge–Weber Syndrome (SWS)

Figure 2.27
A: Sturge–Weber syndrome
(Reprint from Burgdorf WH,
Plewig G, Wolff HH,
Landthaler M, eds. Braun-
Falco’s Dermatology. 3rd ed.
Heidelberg: Springer; 2009)
B: Klippel–Trenaunay syndrome
(Courtesy of Dr. Michelle
B. Bain)
C: Klippel–Trenaunay syndrome
(Courtesy of Dr Michelle
B. Bain)

(Figure 2.27A)

Sporadic neurologic disorder, facial PWS associated with ipsilateral o cular and leptomeningeal anomalies
Facial PWS typically involves V1 distribution (can be more extensive or bilateral), congenital or acquired ocular abnormalities (glaucoma), neurologic abnormalities (seizures, motor dysfunction, mental retardation), intracranial “tram-track” calcification
10–15% patients with PWS of V1 distribution have underlying SWS