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[Multiple eccrine spiradenomas. Apropos of 2 cases in 2 sisters]
Medicina Cutanea Ibero-Latino-Americana
|January 1, 1977
Summary
Two siblings presented with multiple eccrine spiradenoma, a rare tumor. This familial occurrence suggests a potential genetic link, and radiotherapy showed promising results for managing this condition.
Area of Science:
- Dermatology
- Oncology
- Medical Genetics
Background:
- Eccrine spiradenoma is a rare benign adnexal tumor of sweat glands.
- The histogenesis of eccrine spiradenoma remains debated, with theories including neoplastic or hamartomatous origins.
- Multiple eccrine spiradenoma is exceptionally rare, with few cases documented globally.
Observation:
- The study reports two sibling cases of multiple eccrine spiradenoma, representing the 7th and 8th documented instances worldwide.
- This is the first reported occurrence of multiple eccrine spiradenoma in siblings.
- Both patients presented with multiple distinct lesions.
Findings:
- The familial occurrence and multifocal nature of the lesions in these siblings support a potential naevic (congenital abnormality) origin for eccrine spiradenoma.
- Low-dose radiotherapy (1,500 rads over ten applications) yielded excellent results in managing the multiple lesions.
- The histopathological features were consistent with eccrine spiradenoma.
Implications:
- The findings suggest a possible genetic predisposition or inherited component in the development of multiple eccrine spiradenoma.
- Radiotherapy may be a viable and effective therapeutic option for multiple eccrine spiradenoma.
- Long-term follow-up is crucial as remission, even after successful treatment, may be temporary.