TOURAINE-SOLENTE-GOLE

TOURAINE-SOLENTE-GOLESYNDROME:THE
SYNDROME:THECOMPLETE
COMPLETEFORM
FORM
AUTHOUR
AUTHOUR:DR.K.SUDHA.M.D.R.D
:DR.K.SUDHA.M.D.R.D
CO
COAUTHOURS:
AUTHOURS:PROF. DR.K.VANITHA, PROF.DR.N.KAILASANATHAN, PROF.DR.MALATHI.R DR.SUNDARESWARAN,DR.KALPANA.SDR.BABU PETER.S,
PROF. DR.K.VANITHA, PROF.DR.N.KAILASANATHAN, PROF.DR.MALATHI.R DR.SUNDARESWARAN,DR.KALPANA.SDR.BABU PETER.S,
DR.RAMESH.D, DR.AMARNATH.C, DR.DEVIMEENAL.J, DR.MANIMEKALA
DR.RAMESH.D, DR.AMARNATH.C, DR.DEVIMEENAL.J, DR.MANIMEKALA

BARNARD
BARNARDINSTITUE
INSTITUEOF
OFRADIOLOGY
RADIOLOGY,MADRAS
,MADRASMEDICAL
MEDICALCOLLEGE,CHENNAI.
COLLEGE,CHENNAI.

INTRODUCTION Primary osteoarthropathy has to be

differentiated from the secondary form .
Primary or familial
hypertrophic osteoarthropathy FEATURES PRIMARY SECONDARY
also referred to as AGE OF ONSET Starts earlier later
pachydermoperiostosis (PDP)
or touraine solente gole
COURSE Remains progressive
static
syndrome.Autosomal dominant
pattern of inheritance. M:F 9:1. COARSE FACIAL FEATURES FURROWED FOREHEAD WITH PTOSIS HYPERTROPHIED THUMB SKIN Prominent Less
COMPARED WITH NORMAL
MANIFESTATION common
CASE SUMMARY JOINT PAIN Minimum Severe
45/M presented with pandigital EPIPHYSEAL Yes No
clubbing,thickened greasy INVOLEMENT
&coarse skin,furrowed forehead
with ptosis, palmoplantar UNDERLYING None Ass. With
hyperhidrosis,generalised CAUSE bronchogenic
DIGITAL CLUBBING
SEBORRHEIC DERMATITIS DIGITAL CLUBBING CA,Bronchiect
seborrhic dermatitis& multiple
asis etc
folliculitis in both lower limbs EPIPHYSEAL INVOLVEMENT OF
& dyspepsia. BOTH TIBIA.

IMAGING DIAGNOSIS
Having excluded the secondary causes &
X-Ray both hands&feet:
Shows soft tissue thickening
with typical clinicoradiological features
noted in all fingers & toes with the diagnosis of TOURAINE-SOLENTE-GOLE
IRREGULAR PERIOSTEAL REACTION
e/o irregular periosteal IRREGULAR PERIOSTEAL REACTION SYNDROME-COMPLETE FORM with
thickening noted in HYPERTROPHIC GASTROPATHY is
metacarpals, metatarsals & made.
proximal phalanges of both feet
& hands. DISCUSSION
X-Ray both forearm & INTERROSEOUS MEMBRANE OSSIFICATION PERIOSTIAL PROLIFERATION
PDP is a rare hereditary disorder being
legs:Irregular periosteal classified into
proliferation with cortical a)Complete form-
thickening of both radius, ulna pachydermia,clubbing,periostosis .
& both tibia &fibula .E/O b)Fruste form-prominent pachydermia with
interosseous membrane minimal skeletal changes.
ossification noted in Left c)Incomplete form-no pachydermia.
GASTRIC FOLD HYPERTROPHY
GASTRIC WALL THICKENING
forearm. NORMAL CHEST XRAY
COMPLICATIONS
99m
Tc-METHYLDIPHOSPHONATE May develop severe kyphosis, restricted
BONE SCAN motion, and neurologic manifestations.
Pericortical linear
accumulation of radiotracer DIFFERENTIAL DIAGNOSIS
along the shaft of long bones & Acromegaly,Thyroid acropachy,Syphilitic
proximal phalanges . periostosis.
UPPER GI ENDOSCOPY PERICORTICAL
TREATMENT&FOLLOW UP
Prominent gastric mucosal folds LINEAR
ACCUMULATION
with nodularity noted in entire OF NSAIDS,Colchicine,Pamidronate,Tamoxifen
RADIOTRACER
stomach. RADIOTRACER UPTAKE ALONG ALONG LONG citrate & Isotretinoin. Progression typically
BONES.
CT ABDOMEN WITH IV & SHAFT OF LONG BONES caeses after 10 years.
NEUTRAL CONTRAST : PATIENT EDUCATION
E/O uniform wall thickening (>1 cm) Genetic councelling to be offered to patients &
noted in the entire stomach & pylorus. REFERENCES their families.
MICROSCOPY 1)Yu YL, Turck WP. Pachydermoperiostosis (idiopathic hypertrophic osteoarthropathy). Postgrad Med
E/O thickening& packing of collagen fibers in dermis J. Aug 1981;57(670):521medline.
2)Vogl A, Goldfischer S. Pachydermo­periostosis: Primary or idiopathic hypertrophic
with hypertrophy of epidermal appendages with osteoarthropathy. Am J Med 1962; 33: 166­87. 
seborrheic hyperplasia-suggestive of pachydermia. 3)Kumar U, Bhatt SP, Misra A. Unusual associations of pachydermoperiostosis: A case
report. Indian J Med Sci. Feb 2008;62(2):65-8. Medline.