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Clinical and Experimental Dermatology Jan 2024
Topics: Humans; Acro-Osteolysis; Osteolysis
PubMed: 37889144
DOI: 10.1093/ced/llad360 -
The New England Journal of Medicine Sep 1959
Topics: Acro-Osteolysis; Bone Diseases; Disease; Extremities; Humans; Psoriasis
PubMed: 13805591
DOI: 10.1056/NEJM195909102611103 -
The Journal of Bone and Joint Surgery.... Jan 1978Occupational acro-osteolysis is a serious disease definitely related to the inhalation of agents involved in the polymerization of vinyl chloride. After variable periods...
Occupational acro-osteolysis is a serious disease definitely related to the inhalation of agents involved in the polymerization of vinyl chloride. After variable periods of latency, the more or less characteristic clinical picture appears. A Raynaud's phenomenon is usually a premonitory sign indicating that alterations in bone will probably ensue. Scleroderma-like skin lesions and systemic involvement of variable degree occur frequently. The pathophysiology of the disease is unknown, but obstructive lesions of small peripheral arteries are thought to cause osteolysis of the distal phalanges of the fingers.
Topics: Adult; Bone Resorption; Fingers; Humans; Male; Occupational Diseases; Osteolysis; Radiography; Raynaud Disease
PubMed: 624763
DOI: No ID Found -
Annales de Dermatologie Et de... Apr 2016Acro-osteolysis (AO) involves partial or total destruction of the distal phalanges of the fingers or toes. The range of forms of AO is extremely wide. A distinction is... (Review)
Review
BACKGROUND
Acro-osteolysis (AO) involves partial or total destruction of the distal phalanges of the fingers or toes. The range of forms of AO is extremely wide. A distinction is generally made between the acquired forms and the genetic and idiopathic forms. Herein we report a case of idiopathic transverse single-finger AO associated with onychopathy.
PATIENT AND METHODS
A 48-year-old woman consulted for a nail lesion involving brachyonychia in the right index finger alone (pseudo-clubbing appearance of the finger). The remainder of the clinical examination was normal, particularly in terms of neurological and dermatological investigations. Standard x-rays revealed transverse osteolysis of the middle third of the distal phalanx, occurring solely in the right index finger.
DISCUSSION
While certainty regarding diagnosis of AO depends upon radiological findings, the radiological appearance is not specific. Two subgroups suggesting aetiological diagnosis may be distinguished: transverse AO with banding and longitudinal AO. With transverse AO, the diaphysis of the distal phalanx presents osteolysis with banding, but with sparing of the base of the band (pseudo-fracture appearance); this appearance is suggestive of toxic causes, congenital familial conditions or repetitive micro-trauma. Upon x-ray examination, the longitudinal forms exhibit concentric resorption of the band and these forms are more often seen in a setting of neurological, vascular or metabolic disorders. The associated nail involvement frequently present helps clarify the diagnosis, with nail plate shape being dependent on the integrity of the underlying bone.
CONCLUSION
Herein we report the first case of transverse AO in a single finger associated with brachyonychia, and with no discernible cause.
Topics: Female; Finger Phalanges; Humans; Middle Aged; Nail Diseases; Nails, Malformed; Osteolysis; Ultrasonography, Doppler
PubMed: 26944769
DOI: 10.1016/j.annder.2016.01.008 -
Rheumatology (Oxford, England) Dec 2012Acro-osteolysis (bony resorption of the terminal digital tufts) is a well-recognized, but under-researched, manifestation of SSc. Our aim was to investigate the...
OBJECTIVES
Acro-osteolysis (bony resorption of the terminal digital tufts) is a well-recognized, but under-researched, manifestation of SSc. Our aim was to investigate the hypothesis that acro-osteolysis is associated with (i) the severity of digital ischaemia and (ii) the presence of calcinosis.
METHODS
This was a retrospective study of 101 patients with SSc in whom hand radiographs taken between 2001 and May 2008 were available for review. These radiographs were graded for severity of acro-osteolysis on a 0-4-point scale for each finger (0 = normal bone structure, 4 = severe pencilling of the terminal phalanges). From these scores, patients were subdivided into the following two groups: normal/minimal acro-osteolysis and moderate/severe acro-osteolysis. The presence or absence of calcinosis (mild, moderate or severe) was also documented.
RESULTS
Of the 101 patients, 68 were grouped as normal/minimal acro-osteolysis and 33 as moderate/severe acro-osteolysis. Forty-five had severe digital ischaemia: 25 (76%) of the patients with moderate/severe acro-osteolysis compared with 20 (29%) of those with normal/minimal acro-osteolysis (multifactorial analysis: P < 0.001). Patients with moderate/severe acro-osteolysis were more likely to have severe calcinosis (33% vs 13%), but this was not statistically significant after adjustment for potential confounders.
CONCLUSION
Acro-osteolysis was strongly associated with severe digital ischaemia. The potential association with severe calcinosis merits further study. Prospective studies are required to investigate acro-osteolysis as a marker of digital vascular disease progression and of treatment response.
Topics: Acro-Osteolysis; Adult; Aged; Aged, 80 and over; Calcinosis; Female; Fingers; Humans; Ischemia; Male; Middle Aged; Radiography; Retrospective Studies; Scleroderma, Systemic; Young Adult
PubMed: 22923763
DOI: 10.1093/rheumatology/kes214 -
The Journal of Rheumatology Jul 2023To perform a scoping review focusing on osteolysis in systemic sclerosis (SSc). (Review)
Review
OBJECTIVE
To perform a scoping review focusing on osteolysis in systemic sclerosis (SSc).
METHODS
This review was performed in line with the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) recommendations.
RESULTS
From a total of 351 results, 29 articles were included for the final analysis. The publications included proved to be heterogeneous regarding the population and inclusion criteria. The lack of a standardized method of detection of osteolysis further enhanced these inequalities. Most studies reported location/prevalence of osteolysis and associations with other manifestations, with only a minority focusing on topics like predictors of osteolysis and its prognostic value. None of the authors addressed treatment approach. The most frequently analyzed and prevalent location was acro-osteolysis (AO). Diffuse cutaneous subtype and anti-topoisomerase I antibody correlated positively with AO. Disease duration, calcinosis, and digital ischemia were the features more frequently associated with AO, but only the last 2 predicted AO. Ultrasound showed high sensitivity for detection of AO.
CONCLUSION
Despite the effect that osteolysis has on patients with SSc, there is a significant lack of studies on this area. Notably, there are no studies that we know of focused on treatment. Also, there is a lack of longitudinal studies that would allow a reliable assessment of its prognostic value and predictors.
Topics: Humans; Acro-Osteolysis; Osteolysis; Scleroderma, Systemic; Skin
PubMed: 36725053
DOI: 10.3899/jrheum.220626 -
ACR Open Rheumatology May 2023
PubMed: 36988894
DOI: 10.1002/acr2.11541 -
AACE Clinical Case Reports 2020To present a case of pyknodysostosis (PKND), a rare genetic cause of skeletal dysplasia that often goes undiagnosed even in patients with classic features.
OBJECTIVE
To present a case of pyknodysostosis (PKND), a rare genetic cause of skeletal dysplasia that often goes undiagnosed even in patients with classic features.
METHODS
We report a case of PKND that went undiagnosed over many years despite classic features. We performed physical examination, imaging studies, and genetic testing on the patient.
RESULTS
A 21-year-old female presented to endocrinology to establish care. On evaluation, she was noted to have disproportionate short stature and a past medical history notable for bilateral blindness due to optic atrophy secondary to bone enlargement and thickening of the optic nerve canal before age 7 years. She also had a history of foot fractures occurring with ambulation. Her family history was significant for consanguineous parents and relatives with similar clinical features. Physical examination revealed a short, 128-cm tall female with open anterior and mastoid fontanels, mild frontal bossing and micrognathia, evidence of double rows of teeth, and digits of varied length in both hands and feet. Plain radiographs demonstrated diffuse sclerosis and marked cortical thickening of the pelvis, femurs, metacarpals, proximal phalanges, and metatarsals as well as decreased phalangeal length and acro-osteolysis of the hands and feet. Dual energy X-ray absorptiometry demonstrated increased bone mineral density ( scores +2.5 lumbar spine, +3.7 femoral neck, +4.5 total hip). Genetic testing revealed a exon 5-homozygous mutation in the cathepsin K () gene consistent with PKND.
CONCLUSION
Patients with PKND come to medical attention for a variety of reasons but often go undiagnosed even when presenting with classic features due to the rarity of the condition and the overlap with other skeletal dysplasias.
PubMed: 32984533
DOI: 10.4158/ACCR-2020-0169 -
Reumatologia Clinica 2016Systemic sclerosis is a rare disease that predominantly affects women. The Medsger severity scale has been used to assess the severity, but it requires expensive and... (Observational Study)
Observational Study
INTRODUCTION
Systemic sclerosis is a rare disease that predominantly affects women. The Medsger severity scale has been used to assess the severity, but it requires expensive and poorly accessible studies and it does not include complications such acrosteolysis, calcinosis, pericardial disease or hypothyroidism that occur on a relatively frequent basis in this disease. There is no study that considers if comorbidities, such as primary biliary cirrhosis, are related to gravity.
OBJECTIVES
To determine the correlation between severity and the presence of such complications.
METHODS
40 patients with systemic sclerosis, dividing them into tertiles according to severity were studied. Dichotomous variables were described using percentages, while dimensional by averages+SD. Statistical inference was performed using chi square test or Kruskal-Wallis test with Dunn post-test, as appropriate. A significance at P<.05 was set.
RESULTS
Of all the complications studied there were only differences in severity with acrosteolysis. Within comorbidities, primary biliary cirrhosis is not associated with gravity.
Topics: Acro-Osteolysis; Adolescent; Adult; Aged; Cross-Sectional Studies; Female; Humans; Liver Cirrhosis, Biliary; Male; Middle Aged; Scleroderma, Systemic; Severity of Illness Index; Young Adult
PubMed: 26746600
DOI: 10.1016/j.reuma.2015.11.002 -
International Journal of Dermatology Feb 2007
Review
Topics: Acro-Osteolysis; Humans; Male; Middle Aged; Pachyonychia Congenita
PubMed: 17269978
DOI: 10.1111/j.1365-4632.2006.02935.x