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[Generalized pseudoxanthoma elasticum combined with vitamin K dependent clotting factors deficiency].

Abstract

INTRODUCTION

Pseudoxanthoma elasticum is a connective tissue disease currently classed in 4 forms. Two forms are inherited via dominant autosomal transmission and the other two via recessive autosomal transmission. The generalized form of pseudoxanthoma elasticum is the most uncommon form and corresponds to recessive type II. Clinical manifestations include the typical generalized "peau d'orange" skin associated with hyperlaxity of the skin. Usually, there is no systemic manifestation.

CASE REPORT

We report a case of a patient with generalized pseudoxanthoma elasticum associated with deficiency of vitamin-K dependent factors II, VII, IX, and X. A search for other causes of vitamin-K dependent factor deficiency was negative.

DISCUSSION

The association of generalized pseudoxanthoma elasticum with deficiency of vitamin-K dependent clotting factors has been reported previously in very rare cases and is probably not fortuitous. It could led to the definition of a sub-group of recessive autosomal pseudoxanthoma elasticum.

Authors+Show Affiliations

,

Clinique Dermatologique, Hôpital Charles-Nicolle, Rouen.

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Source

MeSH

Adult
Blood Coagulation Disorders
Factor VII Deficiency
Factor X Deficiency
Humans
Hypoprothrombinemias
Male
Prothrombin Time
Pseudoxanthoma Elasticum
Vitamin K Deficiency

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

fre

PubMed ID

9615107

Citation

Le Corvaisier-Pieto, C, et al. "[Generalized Pseudoxanthoma Elasticum Combined With Vitamin K Dependent Clotting Factors Deficiency]." Annales De Dermatologie Et De Venereologie, vol. 123, no. 9, 1996, pp. 555-8.
Le Corvaisier-Pieto C, Joly P, Thomine E, et al. [Generalized pseudoxanthoma elasticum combined with vitamin K dependent clotting factors deficiency]. Ann Dermatol Venereol. 1996;123(9):555-8.
Le Corvaisier-Pieto, C., Joly, P., Thomine, E., Lair, G., & Lauret, P. (1996). [Generalized pseudoxanthoma elasticum combined with vitamin K dependent clotting factors deficiency]. Annales De Dermatologie Et De Venereologie, 123(9), pp. 555-8.
Le Corvaisier-Pieto C, et al. [Generalized Pseudoxanthoma Elasticum Combined With Vitamin K Dependent Clotting Factors Deficiency]. Ann Dermatol Venereol. 1996;123(9):555-8. PubMed PMID: 9615107.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Generalized pseudoxanthoma elasticum combined with vitamin K dependent clotting factors deficiency]. AU - Le Corvaisier-Pieto,C, AU - Joly,P, AU - Thomine,E, AU - Lair,G, AU - Lauret,P, PY - 1996/1/1/pubmed PY - 1998/6/6/medline PY - 1996/1/1/entrez SP - 555 EP - 8 JF - Annales de dermatologie et de venereologie JO - Ann Dermatol Venereol VL - 123 IS - 9 N2 - INTRODUCTION: Pseudoxanthoma elasticum is a connective tissue disease currently classed in 4 forms. Two forms are inherited via dominant autosomal transmission and the other two via recessive autosomal transmission. The generalized form of pseudoxanthoma elasticum is the most uncommon form and corresponds to recessive type II. Clinical manifestations include the typical generalized "peau d'orange" skin associated with hyperlaxity of the skin. Usually, there is no systemic manifestation. CASE REPORT: We report a case of a patient with generalized pseudoxanthoma elasticum associated with deficiency of vitamin-K dependent factors II, VII, IX, and X. A search for other causes of vitamin-K dependent factor deficiency was negative. DISCUSSION: The association of generalized pseudoxanthoma elasticum with deficiency of vitamin-K dependent clotting factors has been reported previously in very rare cases and is probably not fortuitous. It could led to the definition of a sub-group of recessive autosomal pseudoxanthoma elasticum. SN - 0151-9638 UR - https://www.unboundmedicine.com/medline/citation/9615107/[Generalized_pseudoxanthoma_elasticum_combined_with_vitamin_K_dependent_clotting_factors_deficiency]_ L2 - http://www.diseaseinfosearch.org/result/6053 DB - PRIME DP - Unbound Medicine ER -