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Cutaneous hamartoma in tuberous sclerosis - Orange peel sign on magnetic resonance imaging
*For correspondence: madhoo116@gmail.com
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This article was originally published by Wolters Kluwer - Medknow and was migrated to Scientific Scholar after the change of Publisher.
A 28 yr old male† musician was referred by the department of Plastic Surgery to the department of Radiology, Christian Medical College & Hospital, Vellore, India, in November 2018, for magnetic resonance imaging (MRI) of a progressive painless swelling at the nape of the neck since the age of six years. There was associated itching and discharge. On local examination, there was a firm non-tender lobulated swelling in the nape of the neck with hyperpigmentation, skin hypertrophy and irregular corrugated surface with scanty foul smelling whitish discharge (Fig. 1). Multiple Shagreen's patches (Fig. 2) were noted in the lower back and thigh. A clinical diagnosis of tuberous sclerosis was made. MRI of the neck was done to assess the extent of the nape of neck swelling. In addition, MRI of the brain was done to look for other findings of tuberous sclerosis (Figs 3 and 4). The patient underwent excision of the neck lesion with skin grafting. Histopathology revealed folliculosebaceous and cystic hamartoma, a rare cutaneous manifestation of tuberous sclerosis. There was no recurrence at six month follow up.
- Lobulated swelling (white arrow) in the nape of the neck with hyperpigmentation and skin hypertrophy, irregular puckering of the skin surface.
- Multiple Shagreen's patches in the upper back (white arrow).
- (A) T2-weighted sagittal, (B) STIR coronal and (C) STIR axial images revealed an elevated and folded markedly T2 hypointense area of skin thickening in the nape of the neck with multiple tubular T2 hyperintense cystic spaces within (thin white arrow), which on axial sections had a typical 'Peau d' orange' appearance (thick white arrow).
- (A) T2-weighted axial and (B) FLAIR axial images of the brain revealed multiple cortical and subcortical tubers in the bilateral cerebral hemispheres (thin white arrow) and sub-ependymal nodule along the right lateral ventricle (thick white arrow).
Acknowledgment:
Authors acknowledge Dr Aparna Irdoi, department of Radiology, CMC, Vellore, for helping in diagnosis for helping in diagnosis.
Conflicts of Interest: None.
