๐ ็ธฝ็ฎ้ ๏ฝ ๐ ่ฑๆๅๆ๏ผๆฌ็ฏ๏ผ ๏ฝ ๐ ๅฎๆด็ฟป่ญฏ ๏ฝ โญ ็ฒพ่ฏ็ญ่จ
Centrofacial lentiginosis
Centrofacial lentiginosis
Clinical features Centrofacial lentiginosis is a rare autosomal dominant condition in which patients develop a characteristic zone of pigmented macules, particularly about the central region of the face (Fig. 25.12).1โ3 They may also have a variety of skeletal abnormalities including high arched palate, dental
malpositions, cervical or cervicothoracic kyphosis, funnel chest, winged scapulae, spina bifida, hammer toes, bilateral pes cavus, and sacral dehiscence.3 They can also suffer from neuropsychiatric disturbances including mental retardation, behavioral disturbances, and epilepsy.3 Endocrine dysfunction, including goiter, hypothyroidism, and calcium metabolism abnormalities, has occasionally been documented.3 An association of centrofacial lentiginosis and giant nevus spilus developing on the left side of the upper back in a dermatomal distribution has been reported in a child.4
Histologic features The histologic features of the pigmented macules have not been described.
1238 Melanocytic nevi
Fig. 25.12 Centrofacial lentiginosis: innumerable lentigines are present on the face of this young girl. By courtesy of the Institute of Dermatology, London, UK.
Fig. 25.13 PUVA lentigines: multiple lentigines are evident against a background of variable hypo- and hyperpigmentation. By courtesy of the Institute of Dermatology, London, UK.
Fig. 25.14 PUVA lentigo: the features are indistinguishable from a lentigo simplex.