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- Presentation
Newly Defined Vascular and Alopecia Entities: SECVAS, Macular Alopecia, and Midline Infantile Hemangiomas
Description
The speaker reviews three recently described or clarified clinical entities. First, SECVAS (segmental congenital vascular anomaly with atrophy, ulceration, and scarring) is presented as a newly defined vascular condition that overlaps with port-wine birthmarks and congenital hemangiomas, shows segmental red-purple vascular plaques or nodules with central atrophy, ulceration, and scarring, often in characteristic distributions, and may involve activating GNAQ or GNA11 codon 209 mutations. Second, macular alopecia is proposed as a distinct form or subset of alopecia areata characterized by very small alopecic macules, typically in children, more common in females and Hispanic/Latino patients, often on the parietal scalp, with fewer lesions, less coalescence, and faster spontaneous regrowth and excellent prognosis, making biopsy and treatment usually unnecessary. Third, the speaker discusses segmental infantile hemangiomas involving the midline and their relationship to LUMBAR syndrome, emphasizing that lesions crossing or involving the midline appear higher risk and should prompt further evaluation, while lesions that do not cross the midline may not require extensive workup. The talk concludes that these entities improve diagnostic precision and can guide more appropriate management.
View moreConclusions
- SeCVAUS appears to be a newly recognized segmental vascular anomaly with features overlapping capillary malformations and congenital hemangiomas, characterized by atrophy, ulceration, and scarring, and it may be driven by activating GNAQ or GNA11 codon 209 mutations.
- Standard treatments such as laser, excision, and sirolimus seem to offer limited benefit for SeCVAUS, suggesting that effective targeted therapy may eventually be needed.
- Macular alopecia may represent a distinct or at least underrecognized form within the alopecia areata spectrum, with smaller lesions, different demographic associations, and a generally excellent prognosis.
- Because macular alopecia often regrows spontaneously and may not respond meaningfully to treatment, biopsy and aggressive therapy are usually unnecessary.
- Segmental infantile hemangiomas that involve or cross the midline are more strongly associated with LUMBAR syndrome and should prompt further evaluation.
- Segmental lower-body hemangiomas that do not cross the midline appear to be lower risk for LUMBAR syndrome and may not require the same extent of workup.
- The presentation suggests that midline embryologic disruption likely explains the association between transmedian infantile hemangiomas and associated genitourinary, spinal, and bony anomalies.
- Pediatric Dermatology. 2024;41:1063–1076.
- Macular Alopecia: A Single-Center Retrospective Cohort Study to Describe an Underrecognized Pattern of Alopecia.#10.1111/pde.15946
- Segmental Infantile Hemangiomas That Involve the Midline Define Risk for LUMBAR Syndrome.#10.1111/pde.70178
- a 2024 Journal of Pediatrics paper