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Eccrine syringofibroadenomatosis associated with hidrotic ectodermal dysplasia
E L Simpson1, A R Styles, C J Cockerell
1Division of Dermatopathology, University of Texas South-western Medical Center, Dallas, USA.
The British Journal of Dermatology
|July 17, 1998
Summary
Eccrine syringofibroadenoma (ES) can be a reaction pattern, not just a solitary tumor. This skin condition may present as erosive plaques, particularly on palms and soles, and is linked to hidrotic ectodermal dysplasia.
Area of Science:
- Dermatology
- Genetics
Background:
- Eccrine syringofibroadenoma (ES) is typically considered a benign, solitary skin neoplasm.
- Recent observations suggest ES can represent a reactive pattern in specific clinical contexts.
Observation:
- This study details two individuals where ES manifested as part of hidrotic ectodermal dysplasia.
- The diagnosis was delayed for years, with the condition initially mistaken for inflammatory dermatosis of the palms and soles.
- Affected family members were identified, indicating a hereditary component.
Findings:
- Eccrine syringofibroadenoma can present clinically as erythematous, erosive plaques, especially on the palms and soles.
- Hidrotic ectodermal dysplasia can present with unusual cutaneous manifestations mimicking other dermatoses.
Implications:
- Clinicians should consider ES in the differential diagnosis of palmoplantar erosive lesions, particularly when familial cases are present.
- Awareness of ES as a potential reactive pattern in hidrotic ectodermal dysplasia is crucial for accurate diagnosis and management.
- Recognizing unusual cutaneous manifestations of genetic disorders like hidrotic ectodermal dysplasia is important for timely intervention.