An 8-year-old boy presents with multiple, asymptomatic red to brown-colored, variably sized papules on the face. He is taking several anti-epileptic drugs and is doing poorly in school.
Key point: These facial lesions are most typical for adenoma sebaceum, or facial angiofibromas. This is a hallmark manifestation of the neurocutaneous syndrome tuberous sclerosis. Other prominent skin findings may include hypopigmented patches (“ash leaf macules”), thickened plaques on the back (“shagreen patches”), and firm papulonodules under and around finger and toenails (periungual fibromas). Patients are genetically predisposed to neoplasms of the brain, kidney, lung, and heart.
Treatment: Since the manifestations vary widely among patients, symptomatic problems are dealt with as medically appropriate.
Note: The prognosis for individuals with this disorder depends on the severity of symptoms, which range from mild skin abnormalities to varying degrees of learning disabilities, epilepsy and severe mental retardation, renal or cardiac failure, and hydrocephalus. Individuals with mild symptoms generally do well, while those with the more severe form have serious disabilities.
A hematologically stable CML patient developed a solitary, exquisitely painful ulceration, 7 cm × 5 cm, located on the mid-medial foreleg. There was never any evidence of venous insufficiency. The patient denied the possibility of a spider bite. What is the likely cause of this lesion?
