Journal of Postgraduate Medicine
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Year : 2014  |  Volume : 60  |  Issue : 2  |  Page : 205-206  

Learning difficulty and pachydermoperiostosis

MP Gajre, RP Khubchandani, K Mahathi, R Mendadkar 
 Department of Pediatrics, Division of Pediatric Neurology and Epilepsy, Learning Disability Clinic, Lokmanya Tilak Municipal Medical College and Lokmanya Tilak Municipal Medical College, Sion, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India

Correspondence Address:
Dr. M P Gajre
Department of Pediatrics, Division of Pediatric Neurology and Epilepsy, Learning Disability Clinic, Lokmanya Tilak Municipal Medical College and Lokmanya Tilak Municipal Medical College, Sion, Jaslok Hospital and Research Centre, Mumbai, Maharashtra
India




How to cite this article:
Gajre M P, Khubchandani R P, Mahathi K, Mendadkar R. Learning difficulty and pachydermoperiostosis.J Postgrad Med 2014;60:205-206


How to cite this URL:
Gajre M P, Khubchandani R P, Mahathi K, Mendadkar R. Learning difficulty and pachydermoperiostosis. J Postgrad Med [serial online] 2014 [cited 2023 Jan 31 ];60:205-206
Available from: https://www.jpgmonline.com/text.asp?2014/60/2/205/132351


Full Text

 Introduction



A 14-year-old adolescent boy was referred to the learning disability clinic with academic issues secondary to profuse palmoplantar hyperhidrosis of insidious onset. This socially embarrassing handicap interfering with activities like writing, eating, and shaking hands was aggravated during stressful situations like exams and taking a tremendous toll on his self-confidence leading to poor peer interaction, refusal to participate in outdoor play and avoidance of situations involving physical contact with others. He gave a history of acyanotic congenital heart disease which was operated in childhood but no other prolonged systemic complaints. Physical exam revealed a marfanoid habitus with erythema and hyperkeratosis of the palms with grade four digital clubbing. There was no focal neurological deficit. Swelling with restriction of movement was observed in the knees and fingers. There was no family history suggesting similar complaints.

 Diagnosis and Differential Diagnosis



Investigations were carried out for possible noncardiac causes of clubbing since his postoperative echocardiogram was normal. Investigations for a pulmonary and gastrointestinal cause were put on hold because of prominent skin and joint involvement and absence of systemic complaints. However, endocrine work up to rule out thyroid and parathyroid abnormalities was negative and blood counts were normal ruling out secondary causes of hyperhidrosis. The diagnosis of pachydermoperiostosis was confirmed after radiological examination and by a pediatric rheumatologist.

 Discussion



Pachydermoperiostosis is a rare syndrome of unknown incidence, with a male preponderance. ed (9:1). [1] Chromosomal abnormalities have been reported, but no definite locus had been identified. It is characterised by hyperhidrosis [Figure 1], digital clubbing, subperiosteal new bone formation [Figure 2], polyarthritis, seborrhoea, and cutis verticis gyrata. [2] The osteoarthropathy begins during childhood, and progresses gradually over years before stabilizing. Skin changes lead to coarse facial features with thickening, furrowing, and excessive oiliness of the skin of the face and forehead. Bone and joint involvement includes arthritis, arthralgia, periosteal new bone formation, subperiosteal ossification, acroosteolysis (seen in our patient), and osteoporosis. [2] Gastric hypertrophy, gastric ulcers, Crohn's disease, [3] malignancies, [4] and other endocrine abnormalities [5] have been described as associations. The associated hyperhidrosis can have physiological consequences such as cold and clammy hands, dehydration, and skin infections secondary to maceration of the skin. Diagnosis is essentially clinical and radiological. Osteocalcin [6] blood levels could be important to determine the degree of activity of the disease. Treatment of the rheumatologic component includes nonsteroidal anti-inflammatory drugs, colchicine, and bisphosphonates. Isotretinoin is used to address the skin symptoms. Plastic surgery may be beneficial for certain deformities. Considering the limitations, ; it was recommended that he be allowed additional time to complete his examinations.{Figure 1}{Figure 2}

 Acknowledgments



Dr. Mamta Manglani, Professsor and Head, Department of Pediatrics, Lokmanya Tilak Municipal Medical College and Lokmanya Tilak Municipal Medical College, Sion, Mumbai.Dean, Dr. Sandhya Kamath- Lokmanya Tilak Municipal Medical College and Lokmanya Tilak Municipal Medical College, Sion, Mumbai.

References

1Matsui Y, Nishii Y, Maeda M, Okada N, Yoshikawa K. Pachydermoperiostosis--report of a case and review of 121 Japanese cases. Nihon Hifuka Gakkai Zasshi 1991;101:461-7.
2Cooper CJ, Freemont AJ, Riley M, Holt PJ, Anderson DC, Jayson MI. Bone abnormalities and severe arthritis in pachydermoperiostosis. Ann Rheum Dis 1992;51:416-9.
3Shim YW, Suh JS. Primary hypertrophic osteoarthropathy accompanied by Crohn's disease: A case report. Yonsei Med J 1997;38:319-22.
4Thomas RH, Kirby JD. Pachydermoperiostosis with multiple basal cell carcinomata. J R Soc Med 1985;78:335-7.
5Shimizu C, Kubo M, Kijima H, Uematsu R, Sawamura Y, Ishizu A, et al. A rare case of acromegaly associated with pachydermoperiostosis. J Endocrinol Invest 1999;22:386-9.
6Bianchi L, Lubrano C, Carrozzo AM, Iraci S, Tomassoli M, Spera G, et al. Pachydermoperiostosis, study of epidermal growth factor and steroid receptors. Br J Dermatol 1995;132:128-33.

 
Tuesday, January 31, 2023
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