Pseudoxanthoma elasticum-like lesions in association with thalassaemia major.
A 33-year-old woman with a background of thalassaemia major presented with a 3-month history of yellowish plaques on the back of her neck bilaterally and alterations in the texture of the skin in both axillae. Examination of these lesions showed yellowish cobblestone plaques and coalescing papules in both axillae. Pseudoxanthoma elasticum (PXE)-like lesions were confirmed histopathologically. PXE-like lesions in association with thalassaemia major are an uncommon occurrence. Due to the increase in the survival rate of thalassaemia major patients on treatment, the development of these lesions is likely to increase. The histopathological manifestation of these lesions mimics that of hereditary PXE and is associated with severe vascular complications. This case highlights the importance of recognising this rare disorder and the role dermatologists have in establishing the diagnosis and advising on appropriate screening for complications.
Yu
Shirley
S
Department of Dermatology, Prince of Wales Hospital, Sydney, New South Wales, Australia. yu.shirley@yahoo.com.au
Ming
Andrew
A
Wegman
Annette
A
D000328:Adult; D001707:Biopsy, Needle; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008991:Monitoring, Physiologic; D011561:Pseudoxanthoma Elasticum / Q000209:etiology* / Q000473:pathology* / Q000503:physiopathology; D012720:Severity of Illness Index; D016312:Treatment Refusal; D017086:beta-Thalassemia / Q000150:complications* / Q000175:diagnosis