Saturday, 28 September 2024

CASE 784: SKIN ULTRASOUND for PACHYDERMOPERIOSTOSIS [PDP], Dr PHAN THANH HẢI, Dr TRƯƠNG THỊ NGỌC TIẾN, Dr HỒ CHÍ TRUNG, MEDIC MEDICAL CENTER, HCMC, VIETNAM

 A 26 year-old man with lion- like face [dermatological face]  and drumstick- like fingers [digital clubbing](hypertrophic osteoarthropathy) for 6 years.



X-rays films show thickening of the periosteum of ribs, upper and lower limb bones.



  

Skin ultrasound with 23 MHz probe reveales thickening of subcutanous layer and skin of forehead # 5mm, neck #1mm, cheek #3 mm. It exists  distorted structures as hyperechoic and hypoechoic layers in appearance without vascularisation.



             Cheek (L) and  neck (R) skin.
 


      
        Forehead skin # 5 mm of the patient (L) and                evidence # 2mm (R).

Skin biopsy result is related to Tourain-Solente-Gole syndrome (pachydermoperiostosis) which is a rare hereditary disorder affecting to bone and skin.



Pachydermoperiostosis (PDP) is a form of primary hypertrophic osteoarthropathy (see this term), a rare hereditary disorder, and is characterized by digital clubbing, pachydermia and subperiosteal new bone formation associated with pain, polyarthritis, cutis verticis gyrata, seborrhea and hyperhidrosis (from Orphanet).

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