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Correspondence |

Encephalocraniocutaneous Lipomatosis With Didymosis Aplasticopsilolipara

Karola Maria Stieler, MD; Susanne Astner, MD; Georg Bohner, MD; Natalie Garcia Bartels, MD; Hans Proquitté, MD; Wolfram Sterry, MD, PhD; Norbert Haas, MD, PhD; Ulrike Blume-Peytavi, MD, PhD
Arch Dermatol. 2008;144(2):266-268. doi:10.1001/archdermatol.2007.56.
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Haberland  CPerou  M Encephalocraniocutaneous lipomatosis: a new example of ectomesodermal dysgenesis. Arch Neurol 1970;22 (2) 144- 155
PubMed Link to Article[[XSLOpenURL/10.1001/archneur.1970.00480200050005]]
Happle  RKuster  W Nevus psiloliparus: a distinct fatty tissue nevus. Dermatology 1998;197 (1) 6- 10
PubMed Link to Article[[XSLOpenURL/10.1159/000017968]]
Happle  RHorster  S Nevus psiloliparus: report of two nonsyndromic cases. Eur J Dermatol 2004;14 (5) 314- 316
PubMed
Torrelo  ABoente  MCNieto  O  et al.  Nevus psiloliparus and aplasia cutis: a further possible example of didymosis. Pediatr Dermatol 2005;22 (3) 206- 209
PubMed Link to Article[[XSLOpenURL/10.1111/pde.2005.22.issue-3]]
Happle  RKonig  A Didymosis aplasticosebacea: coexistence of aplasia cutis congenita and nevus sebaceus may be explained as a twin spot phenomenon. Dermatology 2001;202 (3) 246- 248
PubMed Link to Article[[XSLOpenURL/10.1159/000051645]]
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lipomatosis

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Figure 1.

Linear soft lesion extending from the vertex to the left eye and associated membranous skin defects (membranous aplasia cutis congenita) with hair collars form the didymosis aplasticopsilolipara. Note typical papules on the left eyelid and proptosis of the left eye.

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Figure 2.

Magnetic resonance images of the patient. A, Sagittal, fat-suppressed, T2-weighted image showing hyperintense cystic areas within the fat-equivalent extracranial mass (arrows). B, Axial, postcontrast, T1-weighted image with focal thickening of the meninges underlying the lipomatous tissue (arrow).

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