A child is diagnosed to have tuberous sclerosis. Which of the following skin lesion is not associated?
Correct Answer: Xanthoderma
Description: (D) Xanthoderma # TUBEROUS SCLEROSIS is a genetic disorder affecting cellular differentiation and proliferation, which results in hamadoma formation in many organs (eg, skin, brain, eye, kidney, and heart).> Skin lesions (found in 70-80% of cases)> Characteristic lesions are angiofibromas, previously known by a misnomer, adenoma sebaceum.> These are pink or skin-colored telangiectatic papules commonly observed in the nasolabial folds and on the cheeks and chin.> They usually appear in children younger than 10 years and increase in size and number until adolescence, remaining unchanged thereafter.> Other areas in which they may be observed include in and around nails (ungual fibromas), scalp, and forehead, in the latter location reaching sizes up to several centimeters.> In the oral mucosa, they may be observed in the lips, dorsa of tongue, and palate.> Dental pitting occurs in about 90% of patients. A hand lens examination aids detection of these pits, which are less obvious in deciduous teeth.> Periungual fibromas (Koenen tumors) are smooth, firm, flesh-colored papules emerging from the nail folds. They can be the only manifestation in some individuals. These are noted around puberty and may increase in frequency as the patient ages.> Shagreen patches are flesh-colored soft plaques that are frequently found in the lumbosacral area but may occur anywhere on the trunk. The surface may be pebbly (resembling pigskin or untanned leather) with prominent follicular openings. They are usuallynoticed during the first decade.> White macules are ovoid, hypopigmented, ash leaf-shaped macules that can be found on the trunks or limbs. White macules offer an excellent opportunity for early diagnosis because they may be found at birth or early infancy.> Use of Wood lamp accentuates these macules.> Color, even though described as white, lacks the depigmented white appearance of vitiligo. A careful examination is necessary before making any firm diagnosis because hypopigmented macules may be a normal finding in newborn babies.> One suggestion is that 3 or more white macules at birth should alert the clinician regarding the possibility of tuberous sclerosis.> Other skin signs include guttate leukoderma, cafe-au-lait macules, and poliosis.
Category:
Skin
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