complete form pachydermoperiostosis in tunisia – a case series and literature review

Introduction: Pachydermoperiostosis (PDP) or hypertrophic osteoarthropathy (HOA) is a rare disease that has genetic predisposition and defined clinical features. Aim of the work: To study and analyse the clinical features of Tunisian PDP patients and review the literature. Patients and methods: The PDP cases attending the Rheumatology Department of the University Hospital Farhat Hached of Sousse in Tunisia were retrospectively studied over a period of 17 years (2000–2017). Secondary causes were excluded. Results: The PDP cases had a primary and complete form of the disease and included 6 men at a frequency of 0.03% (6/20,000) of the total number of rheumatic diseases cases attending the rheumatology clinic. The mean age was 27 ± 12.3 years [18–46 years]. Five patients had arthralgia and one polyarthritis. Thickening of the skin of the head and distal extremities (pachyderma) and deep folds and furrows of the skin of the forehead was observed in all cases. Digital clubbing of the fingers and toes and spade-like enlargement of the hands and feet was noted in 5 cases. Hyperhidrosis of the hands and feet was observed in 4 cases and seborrhea in 2. Elevated acute phase reactants were found in 4 cases. Hypergammaglobulinemia was present in one case. Periostosis of the long bones was observed in all cases. Treatment consisted of analgesics in all cases, non-steroidal anti-inflammatory drugs in 2 cases and tamoxifen in 2 cases. Conclusions: Complete form of primary PDP is rarely present among rheumatic diseases patients in Tunisia. A multicenter larger number longitudinal study is recommended. Keywords: Pachydermoperiostosis, Clubbing, Hypertrophic osteoarthropathy, Periostosis