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- Presentation
Clinicopathologic Self-Assessment
Description
The presentation discussed various dermatological cases, focusing on their pathology without providing initial clinical history. The first case involved a 69-year-old male with an asymptomatic rash on his legs, where the histology suggested Flagel's disease (hyperkeratosis lenticularis persistans). This condition, more prevalent in middle to late adulthood, is characterized by a disorder of keratinization and typically involves the lower legs. A specified deficiency related to the sphingolipid pathway was identified as a causative factor. The second case centered on pre-tibial pruritic papular dermatosis, demonstrating similar histopathological features to other conditions but presenting distinctively as coalescing papules on the shins with minimal epidermal changes. The discussion highlighted the importance of recognizing this entity as a specific condition and differentiated it from lichen simplex chronicus and other dermatoses based on key histological characteristics. The presentation culminated with reflections on patient variability and the need for increased clinical awareness of these conditions.
View moreConclusions
- Hyperkeratosis lenticularis perstans, also known as Flegel's disease, is primarily observed in middle to late adulthood and mainly affects the dorsal feet and lower legs.
- Flegel's disease can be sporadic or hereditary, typically following an autosomal dominant pattern.
- The disease is characterized by discrete keratotic lesions resulting from a defect in keratinization, caused by reduced desquamation or excessive keratin retention.
- The SPTLC1 gene has been identified as associated with Flegel's disease, representing a potential target for future therapies.
- Pretibial pruritic papular dermatosis is recognized as a distinct condition related to chronic rubbing, presenting as discrete papules in the lower legs and can be mistaken for other dermatological disorders.
- Histopathological features of pretibial pruritic papular dermatosis include dermal fibrosis, lymphohistiocytic infiltrate, and minimal epidermal changes, helping to differentiate it from conditions like lichen simplex chronicus and lichen amyloidosis.
- Bortoluzzi P. Internat J Dermatology 2020.
- Br J Dermato/ 2023; 188: 94-99
- Sabine Jägle, Hao-Hsiang Hsu, Hazem A. Juratli, Andreas D. Zimmer, Amelie Prieschl, Svenja Alter, Bernhard Wiedenhofer, Dieter Metze, Steffen Emmert and Judith Fischer. Pathogenic variants in the SPTLC1 gene cause hyperkeratosis lenticularis perstans.
- Ko-ichi Ando, MD, Hisao Hattori, MD, and Yohei Yamauchi, MD. Histopathological Differences Between Early and Old Lesions of Hyperkeratosis Lenticularis Perstans (Flegel's Disease).
- Giorgio Annessi, MD, Marsilia Petresca, MD, and Angela Petresca, MD. Am J Dermatopathol 2006;28:117-121.
- Shadai Flores, BMus, David A. Wada, MD, Scott R. Florell, MD, and Anneli R. Bowen, MD. Am J Dermatopathol 2020;42:16-19.
- Australasian Journal of Dermatology 2023:16 1589-1593.
- Dermatology World Insights and Inquiries By Warren R. Heymann, MD, FAAD May 31, 2023 Vol. 5, No. 22