Correct!
5. 1 and 2
The angiofibromas / angioleiomyomata seen with tuberous sclerosis complex often are found on the face, in the nasolabial folds, cheeks, and skin, unlike the upper scalp in this patient. Angiofibromas / angioleiomyomata are typically pink or skin-colored telangiectatic papules, most commonly red, rather than the flesh color in this patient. The lung cyst distribution (random) is entirely consistent with lymphangioleiomyoma-tosis, as is the cyst’s thin-walled morphology; therefore, answers 3 and 4 are not correct. However, small lung nodules are commonly present in patients with lymphangioleiomyomatosis, multifocal micronodular pneumocyte hyperplasia, but no such nodules are seen in this patient. The lack of these small nodules does not entirely mitigate against the diagnosis of TSC-LAM, but it does raise some suspicion for the accuracy of that diagnosis; therefore, answer 5 (both the absence of lung nodules and appearance of the skin lesions are atypical for the diagnosis of TSC-LAM) is the best answer.
Which of the following represents the next most appropriate step for the evaluation / management of this patient? (Click on the correct answer to proceed to the seventh of eight panels)