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Case Reports
. 2016 Nov-Dec;7(6):523-525.
doi: 10.4103/2229-5178.193901.

Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report

Ashish Jagati  1 , Bela J Shah  1 , Rima Joshi  1 , Trusha Gajjar  1
Affiliations
Case Reports

Encephalocraniocutaneous lipomatosis (Haberland syndrome): A rare case report

Ashish Jagati et al. Indian Dermatol Online J. 2016 Nov-Dec.

Abstract

Haberland syndrome or Fishman syndrome also known as encephalocraniocutaneous lipomatosis (ECCL) is a rare, congenital neurocutaneous disorder. It is characterized by unilateral involvement of skin, eyes and central nervous system. We report the case of a 28-year-old woman who presented with soft lipomatous swelling over right temporal area with nonscarring alopecia of part of frontal and parietal region. The patient had a history of seizures and ipsilateral scleral dermoid. Computed tomography scan findings were suggestive of lipomas and calcification of falx. Magnetic resonance imaging showed right-sided hemi atrophy and two intracranial cysts. We report this case because of its rarity.

Keywords: Encephalocraniocutaneous lipomatosis; Fishman syndrome; Haberland syndrome; lipomatosis.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Single linear plaque extending from scalp to eyebrow, clinically suggestive of lipomatosis
Figure 2
Figure 2
Dermoid tumor of sclera limb, single soft nodular lesion over right eyebrow and few papules over right eyelid
Figure 3
Figure 3
Histopathology: Lobules of diffusely arranged mature adipocytes separated by congested capillaries (H and E, ×10)
Figure 4
Figure 4
Magnetic resonance imaging showing cyst in right temporal lobe and in velum interpositum and dilated lateral ventricle
See this image and copyright information in PMC

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