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Fibrous Forehead Plaque

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A 30-year-old woman presented to our dermatology clinic for evaluation and treatment options of a large 4×2-cm, firm, flesh-colored plaque on the superior aspect of the mid forehead. The plaque initially presented at 10 years of age and had grown since then. She reported no prior treatment of this nontender lesion and denied a family history of skin problems. She also had numerous skin tags all over the face, neck, and upper back that had increased in number for most of her adult life. In reviewing her medical history, she reported experiencing her first seizure at 15 months of age. She was evaluated by the neurology department at that time and was diagnosed with epilepsy.

What’s the diagnosis?

a. basal cell carcinoma

b. benign cephalic histiocytosis

c. keloid

d. neurofibrosarcoma

e. tuberous sclerosis complex

Figure 3. Periungual fibromas.
Figure 4. Hypomelanotic café au lait macules on the anterior aspect of the lower leg.

Tuberous sclerosis complex is not the only syndrome that can present with facial angiofibromas or numerous skin tag–type lesions such as those in our patient. It is important to also consider Birt-Hogg-Dubé syndrome and multiple endocrine neoplasia type 1 in the differential diagnosis of multiple facial angiofibromas, particularly with adult onset.23

Our case was presented to review the cutaneous manifestations of TSC and their presentation frequency, with the intent to enable the dermatologist to make a more accurate and earlier diagnosis of TSC. In doing so, morbidity of TSC patients could be decreased and quality of life increased.

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