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Nevus psiloliparus: Newly described histopathological features from transverse sections
Jorge Roman1, Nicholas A Taylor1, Vikash S Oza1
1The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York.
Journal of Cutaneous Pathology
|February 9, 2020
Summary
Nevus psiloliparus, a rare scalp condition, presents as congenital alopecia and is linked to encephalocraniocutaneous lipomatosis. Histopathology reveals absent hair follicles with dermal fat, suggesting fibroblast growth factor receptor 1 mutations.
Area of Science:
- Dermatology
- Medical Genetics
- Histopathology
Background:
- Nevus psiloliparus is a rare congenital condition characterized by a fatty nevus, often presenting as alopecia on the scalp.
- It serves as a potential marker for encephalocraniocutaneous lipomatosis, a neurocutaneous syndrome involving ocular and central nervous system abnormalities.
Observation:
- This case report details the histopathological findings of nevus psiloliparus in an 11-year-old girl.
- Analysis of transverse sections revealed "shadow" follicular units, indicating a near-complete absence of mature hair follicles.
- These units were characterized by loosely arranged collagen and a relative paucity of elastic fibers, with preserved arrector pili muscles and dermal adipocytes.
Findings:
- Histopathology confirmed the characteristic features of nevus psiloliparus, including the absence of mature hair follicles and the presence of dermal adipocytes.
- The "shadow" follicular units observed in transverse sections provide detailed insight into the structural alterations in affected skin.
Implications:
- Understanding the histopathological features of nevus psiloliparus is crucial for accurate diagnosis and its association with encephalocraniocutaneous lipomatosis.
- The findings support the hypothesis that mosaic mutations in fibroblast growth factor receptor 1 may underlie the pathogenesis of nevus psiloliparus.
