Skin-melanocytic tumor
Nevi
Epidermolysis bullosa nevus

Author: Christopher Hale, M.D. (see Authors page)

Revised: 1 February 2017, last major update April 2013

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: epidermolysis bullosa nevus
Cite this page: Epidermolysis bullosa nevus. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumormelanocyticepidermbullnevus.html. Accessed September 25th, 2017.
Definition / general
  • Large acquired melanocytic nevi that occur in patients with hereditary epidermolysis bullosa
  • Note: similar lesions in ichthyoses (Pediatr Dermatol 2010;27:453), childhood vulvar pemphigoid (Dermatology 2006;213:159) and other inflammatory disorders
  • Two theories: 1) repetitive basement membrane disruption => local nevus cell nests or single melanocytes break senescence and proliferate; 2) melanocytes, probably from incipient nevi or subclinical nests of nevus cells, float in fluid-filled cavity of blister, eventually settling down and proliferating in microenvironment of epidermal regeneration (Dermatol Clin 2010;28:179)
Clinical features
  • 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)
  • Asymmetrical, irregularly pigmented (initially very dark, then loses pigment)
  • Foci of stippled pigmentation and scarring
Case reports
Dermoscopy
  • Some features of melanoma are common: irregular pigmentation (96%), multicomponent pattern (87%), atypical pigment network (74%), irregular dots / globules (70%) and atypical vascular pattern (30%)
  • Other features associated with melanoma progression are rare: irregular streaks, blue-white veil, regression structures (blue-white areas) and black dots (Br J Dermatol 2005;153:97)
Dermoscopic images

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Irregular dots and globules, blue-gray
pigment and hypopigmented areas
Clinical images

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Large pigmented lesion with irregular border

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Regression after 18 months

Microscopic (histologic) description
  • Features of persistent melanocytic neoplasm
Microscopic (histologic) images

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Various images