<abstract xmlns="http://www.w3.org/1999/xhtml"> The authors present a rare case of congenital pachydermoperiostosis associated with striate palmoplantar keratoderma in a 55-year-old female. Pachydermoperiostosis (PDP) is a heterogeneous syndrome characterized by hypertrophic changes involving predominantly the skin and bones of the extremities: pachydermia, clubbing of the fingers and toes, and hypertrophic osteoarthropathy. Primary pachydermoperiostosis (Touraine-Solente-Gole syndrome) (PPDP) or primary hypertrophic osteoarthropathy (PHO) is a rare congenital disorder and is one of two types of hypertrophic osteoarthropathy. In addition to the three main criteria, which are confirmed clinically, histologically, and by X-ray, there may be other additional clinical features. Hyperhidrosis of the hands and feet may be troublesome. The skin of the face, forehead and scalp becomes grossly thickened and thrown into folds. The folding of the scalp produces a form of cutis verticis gyrata. Additional clinical features include hypohidrosis, seborrhea, sebaceous gland hyperplasia and folliculitis, carpal and tarsal tunnel syndrome, chronic leg ulcers and calcification in the Achilles tendon. Our patient presented with most of these additional clinical features, such as acro-osteolysis of the fingers and toes, which generally occurs occasionally. In regard to palmoplantar keratoderma, we have not found reports of its association with PPDP in the available literature. Unlike PPDP, secondary pachydermoperiostosis (secondary hypertrophic osteoarthropathy -SHO) occurs in association with severe pulmonary disease such as bronchiectasis, abscess, bronchial carcinoma, pleural mesothelioma, or thymic, esophageal or stomach cancer, which were all excluded in our patient. In conclusion, this paper presents a congenital form of pachydermoperiostosis in a female also suffering from striate keratoderma. According to the available literature, this is the first case report of comorbidity between these two dermatoses.</abstract>

The authors present a rare case of congenital pachydermoperiostosis associated with striate palmoplantar keratoderma in a 55-year-old female. Pachydermoperiostosis (PDP) is a heterogeneous syndrome characterized by hypertrophic changes involving predominantly the skin and bones of the extremities: pachydermia, clubbing of the fingers and toes, and hypertrophic osteoarthropathy. Primary pachydermoperiostosis (Touraine-Solente-Gole syndrome) (PPDP) or primary hypertrophic osteoarthropathy (PHO) is a rare congenital disorder and is one of two types of hypertrophic osteoarthropathy. In addition to the three main criteria, which are confirmed clinically, histologically, and by X-ray, there may be other additional clinical features. Hyperhidrosis of the hands and feet may be troublesome. The skin of the face, forehead and scalp becomes grossly thickened and thrown into folds. The folding of the scalp produces a form of cutis verticis gyrata. Additional clinical features include hypohidrosis, seborrhea, sebaceous gland hyperplasia and folliculitis, carpal and tarsal tunnel syndrome, chronic leg ulcers and calcification in the Achilles tendon. Our patient presented with most of these additional clinical features, such as acro-osteolysis of the fingers and toes, which generally occurs occasionally. In regard to palmoplantar keratoderma, we have not found reports of its association with PPDP in the available literature. Unlike PPDP, secondary pachydermoperiostosis (secondary hypertrophic osteoarthropathy -SHO) occurs in association with severe pulmonary disease such as bronchiectasis, abscess, bronchial carcinoma, pleural mesothelioma, or thymic, esophageal or stomach cancer, which were all excluded in our patient. In conclusion, this paper presents a congenital form of pachydermoperiostosis in a female also suffering from striate keratoderma. According to the available literature, this is the first case report of comorbidity between these two dermatoses.

Congenital Primary Pachydermoperiostosis and Striate Palmoplantar Keratoderma - a Case Report

Clinic of Dermatovenereology Diseases, Clinical Center of Vojvodina, Faculty of Medicine, University of Novi Sad

Center of Pathology and Histology, Clinical Center of Vojvodina, Faculty of Medicine, University of Novi Sad

Center of Radiology, Clinical Center of Vojvodina, Faculty of Medicine, University of Novi Sad

Serbian Journal of Dermatology and Venereology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

The authors present a rare case of congenital pachydermoperiostosis associated with striate palmoplantar keratoderma in a 55-year-old female....

Congenital Primary Pachydermoperiostosis and Striate Palmoplantar Keratoderma - a Case Report

Pubblicato online: 11 dic 2014

Pagine: 81 - 92

DOI: https://doi.org/10.2478/sjdv-2014-0008

Parole chiave
Osteoarthropathy, Primary Hypertrophic, Keratoderma, Palmoplantar, Diagnosis, Signs and Symptoms, Comorbidity

© 2014

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Congenital Primary Pachydermoperiostosis and Striate Palmoplantar Keratoderma - a Case Report

Pubblicato online: 11 dic 2014

Pagine: 81 - 92

DOI: https://doi.org/10.2478/sjdv-2014-0008

Parole chiaveOsteoarthropathy, Primary Hypertrophic, Keratoderma, Palmoplantar, Diagnosis, Signs and Symptoms, Comorbidity

© 2014

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Parole chiave
Osteoarthropathy, Primary Hypertrophic, Keratoderma, Palmoplantar, Diagnosis, Signs and Symptoms, Comorbidity