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Hyperhidrosis in association with keratoderma and strikingly large hands and feet: a rare case of pachydermoperiostosis

A 20-year-old man presented with excessive sweating of his hands and feet, which had been present since the age of 6 years. From around that time, he had also been aware of thickened skin involving his palms and soles. He reported that other people had often commented on the large size of his hands. No other family members had similar symptoms, and the patient s personal medical history was unremarkable other than having undergone a right knee arthroscopy and washout for recurrent bouts of unexplained knee swelling that was not due to infection. Physical examination revealed no resting tremor or prognathism, although the patient was noted to have large hands and feet with clubbing (Fig. 1a). Visual field assessment was normal. There was no joint tenderness. Nontransgradient keratoderma of the palms and soles was present (Fig. 1b). No other abnormalities were found, although the patient s forehead skin appeared oily and markedly furrowed and thickened for his age (Fig. 1c). All biochemical investigations including tests for growth hormone, insulin-like growth factor and thyroid function gave normal results. Radiological imaging of the hands and feet showed no evidence of a periostial reaction. A clinical diagnosis of pachydermoperiostosis (PDP) was made. The keratoderma was treated with emollients, and iontophoresis was performed for the hyperhidrosis, with good results. PDP (OMIM 167100), is a primary form of hypertrophic osteoarthropathy that was first described in 1868. It is a condition characterized by thickening of the skin of the head and distal extremities; deep folds and furrows in the skin of the forehead, cheeks and scalp (cutis verticis gyrata); seborrhoea; hyperhidrosis; subperiosteal new bone formation associated with pain; polyarthritis; and digital clubbing and spade-like enlargement of the hands and feet. It is more prevalent in males and is usually first evident during adolescence. Its inheritance has been attributed to an autosomal dominant gene of variable expressivity. Secondary PDP occurs predominantly in men between the ages of 30 and 70 years. It is important that the condition is diagnosed correctly because of its association with pulmonary disease including nonsmall-cell carcinoma of the lung, mesothelioma and lung abscesses. Other associations of secondary PDP include primary biliary cirrhosis, ulcerative colitis and Hodgkin s disease. A detailed history and clinical findings will therefore guide the need for further investigations. From a dermatological viewpoint, the cutaneous findings in secondary PDP are relatively mild and may even be absent, in contrast to the more obvious bony changes, which develop rapidly and are usually painful. Touraine et al. described three forms of primary PDP: (i) a complete form with pachydermia (thickening of the facial skin and ⁄ or scalp) and periostitis, (ii) an incomplete form with evidence of bone abnormalities but lacking pachydermia and (iii) a forme fruste with prominent pachydermia and minimal to absent skeletal changes (as seen in our patient). Progression of PDP typically ceases after 10 years, but patients may rarely be left with chronic debilitating complications such as severe kyphosis, restricted motion, and neurological features including carpal and tarsal tunnel syndrome and calcification of the Achilles tendon. Genetic counselling should be offered to patients and their families. Although no chromosomal abnormality has been identified, a radiological survey of relatives may be advisable. CP D