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Case Report/Case Series |

Malignant Melanoma Arising in the Setting of Epidermolysis Bullosa Simplex:  An Important Distinction From Epidermolysis Bullosa Nevus

Thomas L. Hocker, MD1; Matthew C. Fox, MD2; Jeffrey H. Kozlow, MD3; Joseph V. Gonzalez, DPM4; Tor A. Shwayder, MD5; Lori Lowe, MD1,2; May P. Chan, MD1,2
[+] Author Affiliations
1Department of Pathology, University of Michigan, Ann Arbor
2Department of Dermatology, University of Michigan, Ann Arbor
3Department of Surgery, University of Michigan, Ann Arbor
4Sparrow Wound and Hyperbaric Clinic, Lansing, Michigan
5Department of Dermatology, Henry Ford Hospital, Detroit, Michigan
JAMA Dermatol. 2013;149(10):1195-1198. doi:10.1001/jamadermatol.2013.4833.
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Importance  Patients with epidermolysis bullosa (EB) do not carry a significantly increased risk of melanoma but are prone to developing large, markedly atypical melanocytic nevi (EB nevi), which may mimic melanoma clinically and histologically. Many authors now favor a conservative approach in managing atypical pigmented lesions in patients with EB.

Observations  We present the case of a 30-year-old woman with severe EB simplex who sought care for a large red and black ulcerated plaque. The clinical differential diagnosis included EB nevus and melanoma. An incisional punch biopsy specimen revealed an atypical melanocytic proliferation with focal florid pagetoid spread and involving elongated rete ridges, consistent with invasive acral lentiginous melanoma. The subsequent amputation was confirmatory. Micrometastasis was detected in 1 of 5 sentinel lymph nodes.

Conclusions and Relevance  To our knowledge, this is the first reported case of melanoma arising in EB simplex–affected skin. It highlights the difficulty in differentiating melanoma from an EB nevus. Despite the increasing awareness of EB nevi, a high index of suspicion for melanoma should be maintained, and early biopsy is recommended when evaluating large pigmented lesions in patients with EB.

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Figure 1.
Clinical Photographs of a Large, Atypical Pigmented Lesion on the Patient’s Left Foot and Ankle

A, Lateral view shows an intact epidermolysis bullosa simplex bulla above the ankle and a few smaller vesicles within the pigmented plaque. B, Plantar view of the red and black ulcerated plaque covering the entire heel.

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Figure 2.
Initial 4-mm Punch Biopsy Specimen

Characteristic basilar clefting of epidermolysis bullosa simplex is present. In addition, there is a contiguous lentiginous proliferation of atypical melanocytes involving elongated rete ridges. Florid pagetoid spread is also noted toward the center of this photomicrograph (hematoxylin-eosin, original magnification ×100).

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Figure 3.
Amputation Specimen

Sections from the pigmented plaque show unequivocal acral lentiginous melanoma with areas of prominent epidermolysis bullosa simplex–related basilar clefting (hematoxylin-eosin, original magnification ×40). Higher magnification reveals sheets of markedly atypical melanocytes with mitotic activity (inset, hematoxylin-eosin, original magnification ×600).

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Figure 4.
Example of a Classic Epidermolysis Bullosa (EB) Nevus in a 9-Year-Old Girl With EB Simplex

A, Lateral view shows a large, irregular, darkly pigmented patch with multiple satellite lesions. Since the clinical and dermoscopic features of EB nevi overlap significantly with melanoma, a biopsy is often required. A similar-appearing lesion occurring in a patient without EB would be highly suggestive of melanoma, and a biopsy would be advisable. B, Punch biopsy of this EB nevus lacks any features highly suggestive of melanoma, thereby supporting the clinical impression of EB nevus (hematoxylin-eosin, original magnification ×100). Reproduced with permission from Tor A. Shwayder, MD, Department of Dermatology, Henry Ford Hospital, Detroit, Michigan.

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