Lipoid proteinosis: towards predictive clinical clues

Lipoid proteinosis: towards predictive clinical clues I read with great interest a clinical follow‐up letter of lipoid proteoniosis (LP) by Hamie et al. published by Clinical and Experimental Dermatology. I thank the authors for providing the opportunity to discuss these points of a rare genodermatosis.The authors presented 17 cases of LP in 10 Lebanese families, and proposed a number of clinical clues to predict the clinical course of the disease. However, only a few of the presented patients had undergone genetic study. Further, as a general rule, it would be expected that the older the patient, the more prominent the lesions; however, in the study, most of the patients at time of examination were in the paediatric age group (5–16 years), except for one (22 years), and no details were given about the period between onset of the lesions and clinical presentation. In addition, there was no discussion about the length of time for sufficient follow‐up.It is noteworthy that LP is a generalized disorder involving many organs besides the skin and mucous membranes. Patients with LP are prone to recurrent skin infections and episodic swelling of the submandibular or parotid salivary glands. Hepatosplenomegaly has also been described, although without evidence of organ dysfunction. The baseline data of extracutaneous http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Clinical & Experimental Dermatology Wiley

Lipoid proteinosis: towards predictive clinical clues

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Publisher
Wiley
Copyright
Copyright © 2018 British Association of Dermatologists
ISSN
0307-6938
eISSN
1365-2230
D.O.I.
10.1111/ced.13312
Publisher site
See Article on Publisher Site

Abstract

I read with great interest a clinical follow‐up letter of lipoid proteoniosis (LP) by Hamie et al. published by Clinical and Experimental Dermatology. I thank the authors for providing the opportunity to discuss these points of a rare genodermatosis.The authors presented 17 cases of LP in 10 Lebanese families, and proposed a number of clinical clues to predict the clinical course of the disease. However, only a few of the presented patients had undergone genetic study. Further, as a general rule, it would be expected that the older the patient, the more prominent the lesions; however, in the study, most of the patients at time of examination were in the paediatric age group (5–16 years), except for one (22 years), and no details were given about the period between onset of the lesions and clinical presentation. In addition, there was no discussion about the length of time for sufficient follow‐up.It is noteworthy that LP is a generalized disorder involving many organs besides the skin and mucous membranes. Patients with LP are prone to recurrent skin infections and episodic swelling of the submandibular or parotid salivary glands. Hepatosplenomegaly has also been described, although without evidence of organ dysfunction. The baseline data of extracutaneous

Journal

Clinical & Experimental DermatologyWiley

Published: Jan 1, 2018

References

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