Two patients with Hailey-Hailey disease, multiple primary melanomas, and other cancers.

BACKGROUND: Hailey-Hailey Disease (HHD) is an autosomal dominant skin disorder that is characterized by erythematous and sometimes vesicular, weeping plaques of intertriginous regions. Squamous cell carcinoma and basal cell carcinoma arising in lesions of HHD have been described in the literature. To our knowledge, there are no reports of melanoma or noncutaneous malignant neoplasms associated with HHD. OBSERVATIONS: We discuss the mechanisms of oncogenicity, including genetic, environmental, and iatrogenic factors, in 2 patients with HHD, multiple primary melanomas, and other cancers. Patient 1 had a mucoepidermoid carcinoma of the parotid gland. Patient 2 had a history of acute monoblastic leukemia and malignant peripheral nerve sheath tumor as well as radiologic evidence of an acoustic neurilemmoma.
CONCLUSIONS: The cause of the cancers in these 2 patients is likely multifactorial. We describe the patients to draw attention to the possible association between HHD and cancer. Additional research should be performed to determine whether patients with HHD have an increased incidence of cancer. ©2011 American Medical Association. All rights reserved.