Touraine Solente Gole syndrome: The elephant skin disease

ABSTRACT

Touraine Solente Gole syndrome is a rare hereditary syndrome of primary pachydermoperiostosis, with the characteristic triad of pachydermia (or elephant like skin), periostosis and acropachia. A 27-year-old patient presented with aesthetic deformity of forehead due to deep skin folds and coarsening of facial features due to progressive thickening of skin. Associated palmoplantar hyperkeratosis with broadened of finger and toe tips and digital clubbing were noticed. Dermatologic evaluation revealed cutis verticis gyrata of scalp, seborrhoeic hyperplasia of face and hyperhidrosis. Natural history of the disease and aetiopathogenesis were reviewed. Aesthetic correction of forehead through frontal rhytidectomy was attempted.

KEY WORDS

Clubbing - cutis verticis gyrata - frontal rhytidectomy - hypertrophic osteoarthropathy - pachyderma - periostosis

• 1 Fredreich N. Hyperostose des gesammten skelettes. Virchows Arch Pathol Anat Physiol Klin Med 1868;43:83.
• 2 Touraine A, Solente G, Gole L. Un syndrome osteoderopatique: La pachydermie plicaturee avec pachyeriostostose des extremites. Presse Med 1935;43:1820-4.
• 3 Kerimovic Morina DJ. Mladenovic primary hypertrophic osteoarthropathy in 32 patients. Clin Exp Rheumatol 1992;10 Suppl 7:51-6.
• 4 Saigal R, Kansal A, Mittal M, Singh Y, Ram H. Pachydermoperiostosis with myelofibrosis and empty sella. J Assoc Physicians India 2010;58:253-5.
• 5 Burns T. Rook′s Textbook of Dermatology. 7 th ed. Oxford: Blackwell; 2004. p. 12.72-3.
• 6 Uchida JI. A method of frontal rhytidectomy. Plast Reconstr Surg 1965;35:218-22.