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Editorial |

Malignant Melanoma and Epidermolysis Bullosa Simplex

Jo-David Fine, MD, MPH, FRCP1
[+] Author Affiliations
1Departments of Medicine (Dermatology) and Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee
JAMA Dermatol. 2013;149(10):1148-1149. doi:10.1001/jamadermatol.2013.5628.
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In the current issue of JAMA Dermatology, Hocker et al1 describe an adult with Dowling-Meara epidermolysis bullosa (EB) simplex (EBS-DM) who developed a large variegated pigmented lesion on the heel, subsequently proven to be an acral lentiginous melanoma, the appearance of which initially raised the differential diagnosis of an EB nevus. Although the presence of such a lesion arising underneath a callus on the heel would be unusual for an EB nevus, this is an important clinical observation, since it emphasizes that malignant melanoma may arise in EBS, as well as in other EB types and subtypes, and reiterates the need for careful surveillance of pigmented lesions in all types of EBS, especially given the predilection for patients with EB, especially children and young adults, to develop unusually large and oftentimes morphologically markedly irregular-appearing areas of pigmentation on their skin.

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Cumulative Risk of Cutaneous Malignant Melanoma Arising in Epidermolysis Bullosa (EB)

Stratified by major EB subtype, based on data from the National EB Registry, 1986 to 2002. DDEB indicates dominant dystrophic EB; EBS, EB simplex; EBS-loc, localized EBS; EBS-O, generalized EBS; JEB, junctional EB; RDEB–sev gen, severe generalized recessive dystrophic EB.

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