General criteria

Mosaic distribution of lesions

Sporadic occurrence

Progressive course

Specific criteria

A: 1) Cerebriform connective tissue nevus

B: 1) Linear epidermal nevus

2) Asymmetric, disproportionate overgrowth

3) Specific tumors before 2nd decade (Bilateral ovarian cystadenoma; Parotid monomorphic adenoma)

C: 1) Dysregulated adipose tissue

2) Vascular malformations

3) Lung cysts

4) Facial phenotype