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Six or more café au lait macules over 5 mm in diameter in prepubertal persons or over 15 mm in postpubertal persons.Ĥ. NF-1 may be diagnosed when any 2 of the following are present:ġ. In contrast, the 9-year-old girl in Figure B shows classic features of neurofibromatosis type 1 (NF-1) with axillary freckling and multiple café au lait macules. Fortunately, the vast majority of solitary café au lait macules have no clinical significance. Parents frequently ask about these lesions. The macules may be inconspicuous until the affected area is exposed to the sun.
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They vary in size from a few millimeters to several centimeters. Solitary café au lait macules may be present in as many as 30% of newborns. The 12-year-old boy in Figure A has one large and one small café au lait macule and a single hypopigmented macule. Solitary and Multiple Café au Lait Macules The risk of melanoma developing within the lesion is the same as that for uninvolved skin. The hairs usually become coarser and darker with time.A variety of bone and tissue abnormalities have been reported in patients with Becker nevi (eg, arm hypoplasia, pectus excavatum, absent pectoralis major muscle), albeit in a minority of those affected.īecker nevus is not a true nevus. Half of Becker nevi are associated with hypertrichosis. The male-to-female ratio is approximately 5:1.Becker nevi are usually unilateral and are commonly located on the shoulder or upper chest. This common lesion is characterized by the abrupt onset of hyperpigmentation that gradually expands it appears at or before adolescence. This 13-year-old boy has a Becker nevus-also called Becker melanosis, because the lesions do not contain nevus cells.
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We hope this Photo Essay will serve as a reminder that hyperpigmented macules may appear in a wide variety of conditions-both benign and pathologic. For example, patients with Silver-Russell syndrome or Bloom syndrome may have multiple café au lait macules, but photographs of such patients are not included. The collection is by no means comprehensive. Here we present a selection of such lesions and their associated conditions. However, a variety of normal and pathologic conditions are associated with hyperpigmented macules. When a patient presents with a hyperpigmented macule, one of the first conditions that comes to mind is neurofibromatosis.
#Proteus syndrome photos free#
The clot can break free and travel throughout the body. Deep vein thrombosis is a blood clot that occurs in the body’s deep veins, usually in the leg. People with Proteus syndrome are more prone to deep vein thrombosis because it can affect blood vessels. The growths can also cause neurological complications that may affect mental development, and lead to loss of vision and seizures. Overgrowth of bone can also lead to loss of mobility. Tumors can compress organs and nerves, resulting in things like a collapsed lung and loss of sensation in a limb. These can be disfiguring and lead to severe mobility issues. Proteus syndrome can cause numerous complications.