Painful piezogenic pedal papules in patients with Ehlers-Danlos syndrome
Abstract
Painful piezogenic pedal papules were observed in 10 of 29 patients with Ehlers-Danlos syndrome. The diagnosis was made by examining each patient while he or she stood with full body weight on the heels and by observing the appearance of the painful papules on the medial, posterior, and lateral aspects of both heels. Biopsy specimens of papules demonstrated a thickened and dense dermis. The subcutaneous fibrous trabeculae were thin, with resultant poor compartmentalization of the fat. Painful piezogenic pedal papules are due to herniation of subcutaneous fat into the dermis, which is possibly due to structural defects of the connective tissue. The high prevalence of painful piezogenic pedal papules in our group of patients (34.5%) makes its direct association with the Ehlers-Danlos syndrome highly feasible, most probably because of the connective tissue defect that occurs in patients with Ehlers-Danlos syndrome.
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In a sex- and age-matched study of 29 Dutch EDS patients, piezogenic papules were found in 34.5% but none in the control group.
“However, in a sex- and agematched study of 29 Dutch EDS patients, piezogenic papules were found in 34.5% but none in the control group [Kahana et al., 1987].”
From The 2017 international classification of the Ehlers–Danlos syndromes (2017) · cites this paper as Kahana et al., 1987
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The 2017 international classification of the Ehlers–Danlos syndromes
Malfait, F., Francomano, C., Byers, P., Belmont, J., Berglund, B., Black, J., Bloom, L., Bowen, J. M., Brady, A. F., Burrows, N. P., et al. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31552 · Cited by 1,936 · see citing works
Aubry-Rozier, B., Schwitzguebel, A., Valerio, F., Tanniger, J., Paquier, C., Berna, C., Hügle, T., & Benaim, C. (2021) · Rheumatology International · doi:10.1007/s00296-021-04968-3 · Cited by 66 · see citing works
“We recorded piezogenic papules separately, added as a diagnostic feature in 2017 [11], based on a small study [32].”
MethodMcGillis, L., Mittal, N., Santa Mina, D., So, J., Soowamber, M., Weinrib, A., Soever, L., Rozenberg, D., Liu, L., Tse, Y., et al. (2020) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.61459 · Cited by 35 · see citing works
- Authors
- Kahana, M., Feinstein, A., Tabachnic, E., Schewach-Millet, M., & Engelberg, S.
- Corresponding author
- Kahana, Michael
- ORCID
- Kahana, Michael
- Venue
- Journal of the American Academy of Dermatology
- Publisher
- Elsevier BV
- Published
- 1987-08
- Volume
- 17
- Issue
- 2
- Pages
- 205-209
- ISSN
- 0190-9622 (ISSN-L)
- OpenAlex
- W2153876779
- Cited by
- 49
- References
- 0
- Citations by year
- 2026: 2 · 2025: 1 · 2024: 1 · 2023: 1 · 2022: 1 · 2021: 2 · 2020: 2 · 2019: 2 · 2018: 1 · 2017: 3 · 2016: 2 · 2015: 3 · 2014: 1 · 2013: 3 · 2012: 3
- FWCI
- 0.00
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Journal of the American Academy of Dermatology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Dermatological and Skeletal Disorders
- Health Sciences › Medicine › Dermatology › Skin Diseases and Diabetes
- Keywords
- Ehlers-Danlos syndrome, piezogenic papules, connective tissue defects, skin thickening, hereditary connective tissue disorders
- MeSH terms
- Adolescent, Adult, Body Weight, Child, Child, Preschool, Ehlers-Danlos Syndrome, Female, Foot Dermatoses, Heel, Humans, Male, Middle Aged, Pain, Pressure, Skin
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