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Skin-Melanocytic Tumors
Epidermolysis bullosa nevus
Last major update: November 2008 - next update November 2009
Revised: 28 June 2009
Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Definition
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● Large acquired melanocytic nevi that occur in patients with hereditary epidermolysis bullosa
Clinical
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● First described in detail in 2001 (J Am Acad Dermatol 2001;44:577)
● Resembles melanoma, but benign behavior
● Melanocytic growth factors in blister fluid may promote proliferation, migration and melanogenesis of disconnected melanocytes (Acta Derm Venereol 2003;83:332)
Case reports
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● Large nevus in toddler (Arch Dermatol 2007;143:1164)
● 2 infants with recessive dystrophic epidermolysis bullosa (Clin Exp Dermatol 2005;30:636)
● Similar findings in childhood vulvar pemphigoid (Dermatology 2006;213:159)
Clinical description
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● Asymmetrical, irregularly pigmented (initially very dark, then loses pigment)
● Foci of stippled pigmentation and scarring
Clinical images
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Large pigmented lesion with irregular border Regression after 18 months
Dermoscopy description
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● Some features of melanoma are common - irregular pigmentation (96%), multicomponent pattern (87%), atypical pigment network (74%), irregular dots/globules (70%), atypical vascular pattern (30%)
● Other features associated with melanoma progression are rare - irregular streaks, blue-white veil, regression structures (blue-white areas), black dots (Br J Dermatol 2005;153:97)
Dermoscopic images
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Irregular dots and globules, blue-gray pigment and hypopigmented areas
Micro description
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● Features of persistent melanocytic neoplasm
Micro images
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Cleft between dermoepidermal junction, effaced Dermal fibrosis, numerous melanophages
rete ridges and pigmented nested melanocytes and nested melanocytes
in superficial dermis
End of Skin-Melanocytic Tumors > Epidermolysis bullosa nevus
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