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Skin-Melanocytic tumors
Café-au-lait spot
Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Revised: 4 July 2009, last major update November 2008
Copyright: (c) 2005-2009, PathologyOutlines.com, Inc.
Definition
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● Pigmented birthmark, at least 0.5 mm, flat, round/oval, sharply demarcated, smooth or irregular borders with even pigmentation, long axis is along cutaneous nerve tract
● Pigment histologically restricted to basal layer of epidermis
● Usually present at birth
Terminology
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● Café au lait is French for coffee with milk, refers to light brown color of lesions
Epidemiology
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● Present in up to 20% of population; also in neurofibromatosis type 1 (any person with 6 or more lesions should be presumed to have neurofibromatosis until proven otherwise), McCune-Albright syndrome, other syndromes (eMedicine)
● In U.S. study of children under 5 years, 19% had one, 0.75% had more than 2, more present in African-Americans (Arch Dis Child 1966;41:316)
Case reports
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● Newborn with McCune-Albright syndrome and multiple bilateral café au lait spots ( Pediatr Dermatol 1991;8:35)
Clinical images
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Lesion of face Neurofibromatosis type I patient Various images
Micro description
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● Basal hyperpigmentation of epidermis, no deeper pigmentation
● Increased amount of pigment in melanocytes, some have giant pigment granules (Tohoku J Exp Med 1976;118:255)
Micro images
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Macromelanosomes-pigment restricted to basal epidermis
Electron microscopy
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● Macromelanosomes
Differential Diagnoses
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● Mongolian spots - blue pigmentation, larger
● Pigmented nevi - deeper pigment clinically, melanocytes not restricted to basal epidermis
Additional references
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End of Skin-Melanocytic Tumors > Café-au-lait spot
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