|LETTER TO THE EDITOR
|Year : 2018 | Volume
| Issue : 5 | Page : 353-355
Giant shagreen patch in tuberous sclerosis complex
V Kuntoji1, PV Bhagwat2, C Kudligi2
1 Department of Skin and STD, Gadag Institute of Medical Sciences, Gadag, Karnataka, India
2 Department of Skin and STD, Karnataka Institute of Medical Sciences, Hubli, Karnataka, India
|Date of Web Publication||4-Sep-2018|
CUTIS Clinic MRCR Layout, Vijaynagar, Near Veeresh Theatre, Magadi Main Road, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kuntoji V, Bhagwat P V, Kudligi C. Giant shagreen patch in tuberous sclerosis complex. Indian Dermatol Online J 2018;9:353-5
|How to cite this URL:|
Kuntoji V, Bhagwat P V, Kudligi C. Giant shagreen patch in tuberous sclerosis complex. Indian Dermatol Online J [serial online] 2018 [cited 2020 Jun 6];9:353-5. Available from: http://www.idoj.in/text.asp?2018/9/5/353/240521
A 24-year-old male presented to the dermatology outpatient department for multiple raised skin lesions over nose and cheeks. He also had a single raised skin lesion over lower back which was noticed at 1 year of age and was gradually progressive in size. The patient was a known case of epilepsy for which he was on antiepileptics since 10 years. The patient was born out of non-consanguineous marriage with uneventful birth history. Family history was non-contributory and his two siblings were normal. On cutaneous examination, a solitary forehead plaque along with multiple, discrete to coalescent translucent, reddish waxy papules and nodules suggestive of angiofibromas were seen over the face with nearly symmetrical centrofacial distribution [Figure 1]. Further examination revealed a 12 cm × 10 cm pyriform-shaped hyperpigmented, firm, and non-tender plaque with horizontal and vertical grooves in the lumbosacral region [Figure 2]. Other cutaneous and mucosal findings such as ash leaf macules, Koenen's tumor, gingival fibroma, and dental pits were not seen. Systemic examination was insignificant. Echocardiography and ophthalmological examination including visual acuity and fundoscopy was normal. Routine blood investigations were within normal limits. However, ultrasonography (USG) of the abdomen revealed hamartomas in liver and angiomyolipomas in both kidneys. Magnetic resonance imaging (MRI) of the brain (plain) revealed calcified subependymal nodules, cortical and subcortical tubers, and diffuse cerebellar atrophy. Wedge biopsy from the giant plaque in lumbosacral region revealed dense dermal interlacing bundles of collagen with entrapped adnexal structures [Figure 3], suggestive of Shagreen patch, a type of collagenoma. Special stain (Masson's trichrome stain) for collagen was positive.
|Figure 1: Angiofibromas over face with nearly symmetrical centrofacial distribution|
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|Figure 2: A giant pyriform-shaped hyperpigmented plaque with grooves in lumbosacral area|
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|Figure 3: Histopathology of the biopsy done from the plaque over lumbosacral area showing dense dermal interlacing bundles of collagen with entrapped adnexal structures (Masson's trichrome stain, ×100)|
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Tuberous sclerosis complex (TSC) represents a genetic disorder of hamartoma formation in many organs, particularly the skin, brain, eye, kidney, and heart. About 90% of patients with TSC manifest with one or more cutaneous lesions such as hypomelanotic macules, facial angiofibromas, forehead plaque, Shagreen patch, periungual, and ungual fibromas. As per the revised criteria for diagnosis of TSC, our patient fulfilled the criteria with four major features owing to the presence of facial angiofibromas, cortical tubers, subependymal nodule, and renal angiomyolipomas. The work-up revealed a giant collagenoma in the lumbosacral area. Collagenoma can be inherited or acquired. The inherited ones include familial cutaneous collagenoma and Shagreen patch of tuberous sclerosis, whereas eruptive and isolated collagenomas comprise the acquired ones. Although unusual in its size and appearance, we could ascertain the collagenoma in this patient clinically and histopathologically as Shagreen patch. The literature has witnessed giant angiofibroma and giant soft fibroma in patients with tuberous sclerosis. Thappa et al. had reported a giant Shagreen patch associated with spina bifida occulta in tuberous sclerosis. There are only few such reports of giant Shagreen patch in literature which prompted us to report the case.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]