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Pseudoxanthoma elasticum with abnormal nailfold microcirculatory findings
Po-Hung Chen1, Chung-Hsing Chang, Hsin-Su Yu
1Department of Dermatology, Kaohsiung Medical University, No. 100, Shih-Chuan 1st Rd., Kaohsiung, Taiwan. mckee@ksts.seed.net.tw
The Kaohsiung Journal of Medical Sciences
|October 3, 2002
Summary
Pseudoxanthoma elasticum (PXE) is a genetic disorder affecting elastic tissue. This study highlights nailfold capillary microcirculatory disturbances in PXE patients, offering new insights into the condition.
Area of Science:
- Dermatology
- Genetics
- Vascular Biology
Background:
- Pseudoxanthoma elasticum (PXE) is an inherited disorder characterized by the degeneration of elastic tissues.
- Previous research has linked cardiovascular issues in PXE to vascular elastic fiber degeneration.
Observation:
- A 34-year-old male presented with asymptomatic yellowish papules on his neck, similar to his sister's lesions and mild visual impairment.
- Skin biopsy revealed fragmented, calcified elastic fibers in the dermis.
- Funduscopic examination showed angioid streaks, but no other internal organ involvement was detected.
Findings:
- The study identified significant microcirculatory disturbances in nailfold capillaries of PXE patients.
- Observed changes included increased capillary tortuosity, dilated venous loops, and reduced red blood cell velocity.
- Electron microscopy confirmed calcified, degenerated elastic fibers in the skin.
Implications:
- This research is the first to emphasize nailfold capillary microcirculatory disturbances in PXE.
- Findings suggest that microcirculatory changes are a key feature of PXE, impacting blood flow.
- Understanding these microvascular alterations may lead to improved diagnostic and therapeutic strategies for PXE.