Capillary malformations: a classification using specific names for specific skin disorders.

Area of Science:

• Dermatology
• Clinical Genetics
• Vascular Malformations

Background:

• The term 'capillary malformation' is broadly applied, causing diagnostic confusion for various distinct disorders.
• Existing classifications lack clarity, encompassing conditions like nevus flammeus, salmon patch, and syndromes such as megalencephaly-capillary malformation.

Purpose of the Study:

• To delineate 20 different capillary malformations by their distinct clinical and genetic criteria.
• To assign specific, unambiguous names to each identified capillary malformation for improved diagnostic accuracy.

Main Methods:

• Review and synthesis of clinical and genetic data for 20 distinct capillary malformations.
• Categorization of lesions into capillary nevi, potential nevi, and non-nevi malformations.

Main Results:

• Detailed descriptions of capillary nevi, including nevus flammeus, Proteus type port-wine nevus, CLOVES type port-wine nevus, and others.
• Distinction between various port-wine stain types, differentiating lateralized and mesotropic presentations.
• Clarification of conditions such as cutis marmorata telangiectatica congenita versus congenital livedo reticularis, and telangiectatic nevi versus nevus anaemicus.
• Identification of hereditary conditions like Fabry disease and hereditary hemorrhagic telangiectasia, distinguishing them from nevi.

Conclusions:

• The proposed classification system provides clear diagnostic criteria for 20 capillary malformations.
• Accurate differentiation between various capillary nevi and non-nevi vascular lesions is crucial for correct diagnosis and management.
• The salmon patch is definitively excluded from the category of nevi based on presented arguments.