Capillary malformation with segmental distribution and central atrophy: A series of 7 cases

Published: 8 June 2020| Version 1 | DOI: 10.17632/533yn2x6ky.1
Marta Ivars, Jose Manuel Azaña, Lisa Weibel, Martin Theiler, Pablo Boixeda, Diana Islas, Victor Martinez-Glez, Noelia Agra, Lara Rodriguez-Laguna, Isabel Colmenero, Maria José Beato-Merino, Juan Carlos López-Gutiérrez


We present a vascular anomaly not previously described with clinical-pathological features of a CM. Its distinctive clinical appearance and the fact that it is not associated with mutations in GNAQ/GNA11 suggest that this may represent a new entity. We have named it segmental CM with central atrophy. FIGURE LEGENDS: Figure 3. A-B: CM in patient 1. A: A 3-month-old girl with a bright red stain present since birth in the left temporal region that is distributed with a segmental pattern over the occipital and cervical regions. B: Patient 1 at 3 years of age. Note the white-gray central atrophic areas within the red stain. C. CM in patient 2. A 9-year-old girl with a congenital red stain distributed from the arm to the wrist, with well-defined margins and whitish areas of central atrophy. D-E. CM in patient 3. Girl at 3 months (D) and at 10 months of age (E) with multiple red stains on the trunk (pectoral region, back and flank) as well as on the right upper extremity. Note the whitish areas of atrophy on its surface Figure 4. A-B. CM in patient 4. A12-year-old boy with a stable vascular lesion present from birth distributed with a segmental pattern from the buttock to the right thigh. B: Note the central white-gray areas of atrophy. He also presents other similar lesions on the lateral side of the foot (figure not shown). C. CM in patient 5: A 30-year-old woman with a vascular stain with well-defined margins, present from birth, which extends from the inguinal region to the popliteal fossa. D. CM in patient 6: An adolescent with a stable vascular lesion present from birth, with geographic morphology, extending from the armpit to the left flank. E-F. CM in patient 7: A newborn with multiple stains of intense red color on the buttock and posterior region of the thigh, with areas of central cutaneous atrophy (F). Figure 5. A: Hematoxylin and eosin–stained sections (200X) of a skin biopsy demonstrating ectatic small vessels indistinguishable from a simple CM located in the superficial dermis. B-C: Compact solid lobules of capillaries are present in the deeper dermis (arrow). The vessels lack of evident luminal differentiation (Hematoxylin and eosin, 200X & 400X). D: Orcein-stained section (100X) showing focal absence of elastic fibers in the superficial dermis, as well as collapsed elastic fibers in the reticular dermis. E: CD31 immunostaining (40X) showing highly increased numbers of vessel throughout the dermis. F: D2-40 (40X). Negative immunohistochemical staining of most of the vascular endothelial cells for D2-40. Positive vessels represent normal background lymphatics. G: Negative immunohistochemical staining of endothelial cells for GLUT-1 (200X). H: Negative immunohistochemical staining of endothelial cells for WT1 (200X) (asterisk). Note that positive pericytes surround the dilated vessels.



Universidad de Navarra, Universidad Autonoma de Madrid


Vascular Anomaly