We report the case of a patient with a complete form of pachydermoperiostosis with bilateral ptosis as presenting feature. Downloads month by month Downloads Download data is not yet available. [reumatismo.org]

These data show that skin involvement in PDP is a prominent feature, that sometimes these symptoms may also be present in their relatives, and that endothelial and fibroblast activation is present in the skin. [ncbi.nlm.nih.gov]

• Pain

  Bone and joint pains are secondary to periostitis and are usually difficult to control. [doi.org]

  Bisphosphonates inhibit osteoclastic bone resorption and therefore reduce bone remodeling and alleviate painful polyarthritis.In isolated cases, tamoxifen was effective in PDP treatment, especially for bone and joint pain. [en.wikipedia.org]

  Hypertrophic Osteoarthropathy Hypertrophic Pulmonary Osteoarthropathy General Considerations Clinical syndrome consisting of clubbing of the fingers and toes Painful and swollen joints Symmetric periostitis especially involving the tibia and fibula, radius [learningradiology.com]

  […] pachydermoperiostosis is a rare congenital disease characterized by clubbing of the fingers, periostitis of the distal long bones, and hypertrophic skin changes (pachydermia) including thick folds in the skin of the face, forehead, scalp and extremities, and also joint pain [ncbi.nlm.nih.gov]

• Swelling

  Clinical phenotypes of PHO varied, ranging from mild isolated finger clubbing to severe pachydermia and disabling joint swelling, even within families. Circulating PGE 2 metabolism features of PHOAR2 were different from those of PHOAR1. [ncbi.nlm.nih.gov]

  Posthepatic cirrhosis Chronic active hepatitis Bile duct carcinoma Benign bile duct stricture Amyloidosis, liver Abscess Undifferentiated nasopharyngeal carcinoma Pancreatic carcinoma Chronic myelogenous leukemia Clinical Findings Burning pain Painful swelling [learningradiology.com]

  A 40-year-old male presented to the dental department with swelling of the gums. [doi.org]

• Inflammation

  Histological examination of the synovium obtained from his right knee revealed endothelial hyperplasia and vascular thickening without inflammation that was in association with aberrant expression of CD200/CD200R1, which highlighted the importance of [ncbi.nlm.nih.gov]

  These changes are actually caused by the inflammation that occurs at the level of the subungual capillary. The inflammation is because of the excessive collagen deposition, along with the interstitial inflammation and the associated edema. [healthh.com]

  Inflammation 1992, 16 :45–56. PubMed CrossRef Google Scholar 31. Cronstein BN, Weissmann G: The adhesion molecules of inflammation. Arthritis Rheum 1993, 36 :147–157. PubMed Google Scholar 32. [doi.org]

  […] vasodilators which are not metabolized by lung Increased flow through AV shunts Reflex peripheral vasodilation (vagal impulses) Hormones: estrogen, growth hormone, prostaglandin Thoracic Causes Malignant tumor (0.7-12%) Benign tumor Chronic infection / inflammation [learningradiology.com]

• Disability

  This familial disorder can cause diagnostic confusion, as well as significant disability. [ncbi.nlm.nih.gov]

  The pain associated with HOA can be disabling and often refractory to conventional analgesics. We performed a comprehensive review of the literature using the PubMed database on treatment modalities available for HOA. [aianmodena.org]

• Falling

  Ein anatomischer Bericht über den Fall Hagner. Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1891, 10: 1. C. Audry: Pachydermia occipitale vorticalée (cutis verticis gyrata). [whonamedit.com]

  The disease falls into 2 categories: Primary hypertrophic osteoarthropathy Secondary hypertrophic osteoarthropathy. Primary hypertrophic osteoarthropathy is a rare inherited condition. [dermnetnz.org]

  The disease falls into two categories: Primary and secondary HOA. [jiaomr.in]

• Clubbed Finger

  We report a 19-year-old man presenting with arthritis, broadening of the fingers and clubbing of the fingers and toes for the previous 3 years. The ankles and knees were swollen. X-rays showed periosteal apposition. [ncbi.nlm.nih.gov]

  finger 件名（キーワード） Hormone 件名（キーワード） Lung cancer 件名（キーワード） Pulmonary hypertrophic osteoarthropathy (PHO) 件名（キーワード） Bone scintigraphy 件名（キーワード） Clubbed finger 件名（キーワード） Hormone 要約・抄録 症例は75歳男性. [iss.ndl.go.jp]

  Clubbing of the fingers. Edinb Med J. 1949 Mar. 56(3):105-20. [Medline]. Kaditis AG, Nelson AM, Driscoll DJ. Takayasu's arteritis presenting with unilateral digital clubbing. J Rheumatol. 1995 Dec. 22(12):2346-8. [Medline]. [emedicine.medscape.com]

• Heart Disease

  Most cases are associated with malignancy or other conditions such as congenital heart disease, liver cirrhosis, pulmonary fibrosis, biliary atresia, and gastrointestinal polyps. [ncbi.nlm.nih.gov]

  Vitamin D Kinetics and Parathyroid Gland Function in Patients with Congenital Heart Disease. Congenital Heart Disease, Vol. 11, Issue. 6, p. 700. [cambridge.org]

  Diseases associated with clubbing Clubbing can also be seen as an isolated abnormality in patients with lung and/or heart disease, and rarely gastrointestinal disease, hyperthyroidism and other diseases. [dermnetnz.org]

  disease with R-to-L shunt Extrathoracic Causes GI tract Liver disease Biliary + alcoholic cirrhosis Posthepatic cirrhosis Chronic active hepatitis Bile duct carcinoma Benign bile duct stricture Amyloidosis, liver Abscess Undifferentiated nasopharyngeal [learningradiology.com]

  This diagnosis is made after excluding the secondary form of hypertrophic osteoarthropathy that is associated with a variety of disorders, including intrathoracic malignancies, suppurative lung disease, and congenital heart disease. 1 A: Clubbing of fingers [mja.com.au]

• Hypertension

  Primary pulmonary hypertension. Medicine, 65 (1986), pp. 56-72 [7.] S. Rich, D.R. Dantzker, S.M. Ayres, et al. Primary pulmonary hypertension: a nalional prospective study. Ann Intern Med, 107 (1987), pp. 216-223 [8.] G.H. Golsmith, R.G. Baily, D.B. [archbronconeumol.org]

  Hyperactivity of the sympathetic nerve system may cause hypertension and hyperhidrosis of the palms and soles. [69] Other differentials include acromegaly, thyroid acropachy, and scleromyxedema, syphilitic periostitis, osteopetrosis, and Paget disease [emedicine.medscape.com]

  He had stage 1 hypertension. Rest of the cardiovascular examination was normal. There were no other physical findings. Laboratory investigations showed Haemoglobin of 14.6gm/dl, normal differential counts. ESR. [jpma.org.pk]

  The role of sex in the pathophysiology of pulmonary hypertension. Advances in Experimental Medicine and Biology 2018. 1065 511–528. (10.1007/978-3-319-77932-4_31) [ PubMed ] [ CrossRef ] [ Google Scholar ] 55. [ncbi.nlm.nih.gov]

• Blepharoptosis

  Eyelid ptosis which is caused by thickened eyelids (blepharoptosis) is a less common symptom. We report the case of a patient with a complete form of pachydermoperiostosis with bilateral ptosis as presenting feature. [reumatismo.org]

  Keywords: Blepharoptosis, hypertrophic osteoarthropathy, myelolipoma How to cite this article: Dogan AS, Acaroglu G, Dikmetas O. Blepharoptosis and hypertrophic osteoarthropathy: A case report. [ijo.in]

  Keywords: Cornea; Blepharoptosis/physiopathology; Blepharoptosis/surgery; Osteoarthropathy, primary hypertrophic/etiology; Case reports INTRODUCTION Primary hypertrophic osteoarthropathy (PHO) or pachydermoperiostosis is a rare syndrome characterised [sboportal.org.br]

• Thickened Eyelids

  Eyelid ptosis which is caused by thickened eyelids (blepharoptosis) is a less common symptom. We report the case of a patient with a complete form of pachydermoperiostosis with bilateral ptosis as presenting feature. [reumatismo.org]

  Associated clinical features that have been described include clubbing of digits, with or without paronychial thickening; arthralgias; seborrheic dermatitis; mechanical ptosis of thickened eyelids; periodontal disease; and palmoplantar hyperhidrosis. [doi.org]

  The skin of his scalp and face was greasy and thickened and he had deep nasolabial folds, prominent nose, bilateral clubbing of fingers and toes. Ophthalmic examination revealed thickened eyelids with a clear vision. [jiaomr.in]

  On examination, the patient had coarse facial features, thickened eyelids and partial blepharoptosis. [edmcasereports.com]

• Prolapse

  ECHO DONE: Prolapse of AML+. EF 68 %. Intact IAS and IVS. Valves normal. 20. EGD: Hypertrophic gastric mucosal folds. 21. [slideshare.net]

• Arthritis

  Article Sidebar Published: Nov 6, 2014 Case Reports Keywords Idiopathic hypertrophic osteoarthropathy, Periostosis, Ptosis, Arthritis. [reumatismo.org]

  In cases of arthralgia/arthritis together with clubbed fingers, consideration must be given to hypertrophic osteoarthropathy. The primary or idiopathic form is rare and has a good prognosis. [ncbi.nlm.nih.gov]

  Malawista SE (1975) The action of colchicine in acute gouty arthritis. Arthritis Rheum 18 (suppl 6):835–842 Google Scholar 21. [doi.org]

• Arthralgia

  It is a self-limiting condition but arthralgia may be as severe as limiting social life and activity, and it is the main complaint to be treated. We report on two siblings with pachydermoperiostosis (PDP), one of whom had arthralgia. [doi.org]

  Around 70% of the patients treated with NSAIDs had clinical improvement for their symptoms, mostly arthritis or arthralgia symptoms. CONCLUSION: NSAIDs were effective in improving arthralgia or arthritis symptoms in majority of the PHO patients. [ncbi.nlm.nih.gov]

• Bone Pain

  Bone and joint pains are secondary to periostitis and are usually difficult to control. [doi.org]

  Colchicine and retinoids improved skin manifestations, while tamoxifen and bisphosphonates significantly alleviated musculoskeletal symptoms, especially joint and bone pain. [medigoo.com]

  Involvement of long bones may produce severe, incapacitating, or deep bone pain. Areas affected by periostitis are often painful and may be associated with overlying warmth or edema. [rheumaknowledgy.com]

  Digital clubbing appeared, which was associated with arthritis, bone pain and inflammatory syndrome. X-rays and magnetic resonance imaging found periosteal apposition in the long bones; bone scintigraphy confirmed the HOA diagnosis. [karger.com]

• Joint Swelling

  Clinical phenotypes of PHO varied, ranging from mild isolated finger clubbing to severe pachydermia and disabling joint swelling, even within families. Circulating PGE 2 metabolism features of PHOAR2 were different from those of PHOAR1. [ncbi.nlm.nih.gov]

  […] spaces and soft tissue swelling. [edmcasereports.com]

  Hypertrophic osteoarthropathy (a syndrome which also includes chronic proliferative periostitis of the long bones and joint swelling). Pulmonary disease Lung cancer. Tuberculosis. Bronchiectasis. Cystic fibrosis. Interstitial lung disease. [patient.info]

  Otherwise, the weight bearing joints, such as the knees and ankles, are most commonly affected in HOA, and joint swelling is frequently seen in conjunction with the synovial fluid in the rare cases where inflammation occurs.[ 17 ] The clues for the differential [archivesofrheumatology.org]

• Fracture

  There was no fracture, joint effusion, or additional underlying osseous abnormality. A chest radiograph revealed a 5-cm right upper lobe mass ( image B ), which was determined to be a moderately differentiated invasive adenocarcinoma. [jaoa.org]

  85 Tibial Plateau Fracture Fracture Type IV 95 Projectiles 103 Differences between Homocystinuria and Marfan 108 SLAPI 114 Criteria for Diagnosis of Marfan Syndrome 120 Medial Meniscal Tear 122 Mucopolysaccharidoses 128 Differential Signs between PHypoPT [books.google.de]

  The patient initially presented 2 months ago to the orthopedic clinic seeking medical attention for the right knee pain after minor trauma, which was found to be due to patella fracture. [doi.org]

  Last Update: 2012-03-21 Usage Frequency: 5 Quality: English he was clubbed on the back by purportedly unknown assailants, who may be known to the authorities, and as a result he suffered multiple fractures to his arms and head injuries requiring stitches [mymemory.translated.net]

• Hyperhidrosis

  Elevated prostaglandin levels in affected individuals with cytokine-mediated tissue remodelling and vascular stimulation may underlie PHO and associated features as hyperhidrosis, acroosteolysis, pachyderma, periostosis and arthritis. [ncbi.nlm.nih.gov]

  The patient had the typical findings of the complete form of PHO including furrowing of skin of forehead and scalp, digital clubbing, palmoplantar hyperhidrosis, and subperiosteal new bone formation. [scopemed.org]

  Elevated prostaglandin levels in affected individuals with cytokine‐mediated tissue remodelling and vascular stimulation may underlie PHO and associated features as hyperhidrosis, acroosteolysis, pachyderma, periostosis and arthritis. [doi.org]

• Sweating

  A case of pachydermoperiostosis characterised by the presence of finger clubbing, periostosis, sweating of hands and feet is described. Modifications of capillaroscopic pattern and of arteriovenous anastomoses are reported. [ncbi.nlm.nih.gov]

  Furthermore, it blocks cholinergic transmission to the sweat glands and therefore inhibits sweat secretion. However, the exact mechanism for improving leonine faces is unknown and needs to be further investigated. [en.wikipedia.org]

  Joint pain, an abnormal enlargement of the tips of the fingers and toes (clubbing), and excessive sweating of the hands and feet (hyperhidrosis) may also be present. [rarediseases.org]

  Chronic myelogenous leukemia Clinical Findings Burning pain Painful swelling of limbs Stiffness of joints Ankles (88%) Wrists (83%) Knees (75%) Elbows (17%) Shoulders (10%) Fingers (7%) Peripheral neurovascular disorders Local cyanosis Areas of increased sweating [learningradiology.com]

• Eczema

  Hyperhidrosis (92%), arthralgia (65%) and eczema (33%) were variably associated features. Pachydermia (54%) was mild and mostly limited to palms and sole; cutis vertigis gyrata, blepharoptosis and severe skin thickening were never observed. [ncbi.nlm.nih.gov]

  Eczema. Coarse skin. Thick hand and foot skin. Furrowing. Seborrheic hyperplasia. Cutis verticis gyrate. Increased secretion of sebum. Periostosis. Acroosteolysis. Myelofibrosis. Widening of bone formation. Digital clubbing. [medigoo.com]

  […] and of the hands and feet, nose, lips, tongue, and forehead, along with cutis vertices gyrata and corneal leukoma); Currarino idiopathic osteoarthropathy (an incomplete form of PDP seen in children and adolescents and characterized by the presence of eczema [doi.org]

  Currarino idiopathic osteoarthropathy is a juvenile incomplete form of PDP characterized by eczema and wide cranial sutures. [slideshare.net]

  Among the other features that can be present in patients suffering from hypertrophic osteoarthropathy, there are: acne, seborrhea, eczema. Palmoplantar hyperhidrosis can also be present in some patients. [healthh.com]

• Erythema

  Clinical Findings Burning pain Painful swelling of limbs Stiffness of joints Ankles (88%) Wrists (83%) Knees (75%) Elbows (17%) Shoulders (10%) Fingers (7%) Peripheral neurovascular disorders Local cyanosis Areas of increased sweating Parasthesia Chronic erythema [learningradiology.com]

  Within 48 hours of therapy, the patient and family noted a marked improvement in the right shin erythema, finger erythema, and polyarthropathy ( Figure 4 and Figure 5 ). Within 72 hours, the symptoms had completely resolved. [jcadonline.com]

  Other clinical features include, but are not limited to, seborrhea, periungal erythema and oily skin. Diagnosis of pHOA is purely one of exclusion. [doi.org]

  Neurovascular changes of the hands and feet, including chronic erythema, paresthesia, and increased sweating, may occur. In primary HPOA, bone and joint pain tends to be less severe, whereas furrowing of the face and scalp tends to be more severe. [radonc.wikidot.com]

• Increased Sweating

  sweating Parasthesia Chronic erythema Flushing and blanching of skin Clubbing Hypertrophy of extremities (soft-tissue swelling) Location Tibia and fibula (75%) Radius and ulna (80%) Proximal phalanges (60%), femur (50%) Metacarpus and metatarsus (40% [learningradiology.com]

  The presentation at a community clinic with a gradual onset of increased size of hands and feet, facial coarsening, increased sweating and seborrhea led to a referral to a specialist endocrinology department to rule out acromegaly. [edmcasereports.com]

  Neurovascular changes of the hands and feet, including chronic erythema, paresthesia, and increased sweating, may occur. In primary HPOA, bone and joint pain tends to be less severe, whereas furrowing of the face and scalp tends to be more severe. [radonc.wikidot.com]

  Ptosis occurs due to thickening and hypertrophy of the tarsal plates, which in turn is caused by meibomian gland hyperplasia, increased sweat glands, and a thickened dermis. [sboportal.org.br]

Comprehensive diagnostic workup in search of secondary causes was launched. On laboratory examination, CBC exhibited mild anemia with hemoglobin of 12.2 g/dL, metabolic panel including liver enzymes were within normal range. [doi.org]

Thus, a comprehensive workup is required to rule out underlying conditions.[12] In the case of clubbing with periosteal proliferation, however, intrathoracic malignancies account for approximately 90 percent of cases.[11] Etiology. [jcadonline.com]

In combination with NSAIDs and colchicine, treatment with sulfasalazine was started in both cases, and methotrexate was added to the treatment regimen of the female patient at the end of the first year. The patients were found to be typical PHO. [ncbi.nlm.nih.gov]

Neutrophilic chemotaxis, evaluated before starting the treatment and after 15 and 30 days of therapy, showed a progressive decrease of the initial very high index. [doi.org]

Rheumatologic symptoms can be improved by treatment with bisphosphonates, such as pamidronate or risedronate. [en.wikipedia.org]

The primary or idiopathic form is rare and has a good prognosis. [ncbi.nlm.nih.gov]

Prognosis: In secondary forms, the outcome is dictated by the underlying disorder. BIBLIOGRAPHY Martinez-Lavin M, Matucci-Cerinic M, Jajic I, et al. [rheumaknowledgy.com]

All patients need reassurance about good prognosis. Conclusion Primary hypertrophic osteoarthropathy is a rare familial disorder. There are individual case reports in literature with no history of family involvement. [jpma.org.pk]

Nuclear medicine Tc- 99m MDP bone scan symmetric linear increase in tracer accumulation along diaphyseal and metaphyseal surfaces of long bones 4 "tram-track" appearance Treatment and prognosis The success of treatment depends on whether the hypertrophic [radiopaedia.org]

Prognosis Clubbing is potentially reversible if the underlying condition is treated early enough but the changes may be irreversible once collagen deposition has set in. [patient.info]

Osteoarthropathy, Primary Hypertrophic*/drug therapy Osteoarthropathy, Primary Hypertrophic*/metabolism Osteoarthropathy, Primary Hypertrophic*/physiopathology Osteoarthropathy, Secondary Hypertrophic*/diagnosis Osteoarthropathy, Secondary Hypertrophic*/etiology [ncbi.nlm.nih.gov]

Arterial aneurysm, infective arteritis including endocarditis or infected AV fistulas, hemiplegia and cardiac shunts such as patent ductus arteriosus are most common etiologies of secondary HOA. [clinicaladvisor.com]

[…] clubbing of the fingers and toes Painful and swollen joints Symmetric periostitis especially involving the tibia and fibula, radius and ulna Most often secondary to an intrathoracic neoplasm or infection Primary form is called pachydermoperiostitis Etiology [learningradiology.com]

Etiology: The primary form is of unknown etiology, but most likely has a genetic basis. [rheumaknowledgy.com]

Alternatively, neurogenic etiology has been proposed 5. [radiopaedia.org]

Tukamoto K, Riazuddin S, Kabra M, Erdenetungalag R, Radnaabazar J, Khan S, Pandya A, Usami SI, Nance WE, Wilcox ER, Riazuddin S, Griffith AJ (2003) Origins and frequencies of SLC26A4 (PDS) mutations in east and south Asians: global implications for the epidemiology [doi.org]

Epidemiology Pachydermoperiostosis is rare and is characterised by skin thickening of the forehead, eyelids and hands, digital clubbing and periostosis. [ 2 ] The epidemiology of secondary clubbing depends on the cause. [patient.info]

Jaic I (1992) Epidemiology of hypertrophic osteoarthropathy. Clin Exp Rheum 10(suppl 7):13 Google Scholar 3. Hedayati H, Barmada R, Skosey JL (1980) Acrolysis in pachydermoperiostosis, primary or idiopathic hypertrophic osteoarthropathy. [link.springer.com]

[…] diseases generally known to result in secondary hypertrophic osteoarthropathy (Crohn disease, myelofibrosis, and congenital cardiac disease) have been reported to develop in patients with pachydermoperiostosis or primary hypertropic osteoarthropathy. [34] Epidemiology [emedicine.com]

Pathophysiology of HOA remains largely obscure. Several theories have been proposed including autonomic dysfunction with sympathetic bias ( 8 ) and dysfunctional fibroblast proliferation ( 9 ). [doi.org]

Further research is needed to clarify the pathophysiological mechanism of HOA as to appropriately direct therapy. [aianmodena.org]

Clubbing is thought to result from changes to the volume of interstitial fluid and increased blood flow to the area but the exact pathophysiology remains unknown. Primary clubbing may be idiopathic or be a feature of an inherited condition. [patient.info]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

It is able to bind to the ends of microtubules to prevent its elongation. [en.wikipedia.org]

Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public [emedicine.com]