Progressive osseous heteroplasia

Miller, E. S.

doi:10.1001/archderm.132.7.787

Cited by 11 publications

(4 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In contrast, several dermatologic conditions of humans known as metaplastic ossification, primary or secondary osteoma cutis, plate-like osteoma, and acquired cutaneous osteomatosis are characterized by metaplastic bone formation in the dermis, where osteoid formation occurs with or without collagen mineralization. 2,4,7,10,12,15,18 Some of these syndromes are idiopathic, while others have been associated with a variety of predisposing factors, including acne vulgaris, systemic metabolic illness, dermal surgery, and chronic irritation. 1,12,15,17,18 Reported here are 2 cases of widespread dermal osseous metaplasia in dogs with evidence of iatrogenic hyperglucocorticoidism that share histologic features with human cases of metaplastic ossification.…”

mentioning

confidence: 99%

Multiple Cutaneous Metaplastic Ossification Associated with Iatrogenic Hyperglucocorticoidism

et al. 1998

View full text Add to dashboard Cite

Characteristic dermatologic lesions of naturally occurring and iatrogenic canine hyperglucocorticoidism have been well documented, and include any combination of bilaterally symmetric alopecia, thinning of the epidermis, skin hyperpigmentation or depigmentation, comedones, phlebectasia, and calcinosis cutis. 20 Other generalized signs reported in affected animals include polydipsia, polyuria, muscle weakness, hepatomegaly, and myopathies. Although calcinosis cutis in its broadest definition can encompass all forms of calcium deposition, it is most commonly used to denote the specific pattern of dystrophic calcification associated with hyperadrenocorticism or iatrogenic hyperglucocorticoidism. 8 The mechanism of this process has not been completely elucidated, but it involves phase transformation of calcium and phosphate ions from solution into crystalline aggregates, with deposition upon the matrices of dermal collagen and elastin under the influence of cellular factors. 3,8 The collagen fibers in these cases are variably mineralized, and root sheaths may also be affected. 8,20 The deposition of minerals in calcinosis cutis is often not associated with areas of inflammation and, when observed, typically consists of a histiocytic foreign-body-type reaction. In contrast, several dermatologic conditions of humans known as metaplastic ossification, primary or secondary osteoma cutis, plate-like osteoma, and acquired cutaneous osteomatosis are characterized by metaplastic bone formation in the dermis, where osteoid formation occurs with or without collagen mineralization. 2,4,7,10,12,15,18 Some of these syndromes are idiopathic, while others have been associated with a variety of predisposing factors, including acne vulgaris, systemic metabolic illness, dermal surgery, and chronic irritation. 1,12,15,17,18 Reported here are 2 cases of widespread dermal osseous metaplasia in dogs with evidence of iatrogenic hyperglucocorticoidism that share histologic features with human cases of metaplastic ossification.An 8-year-old male Rottweiler had a 4-year history of chronic skin disease that was only partially responsive to antibiotic and corticosteroid therapy and was becoming progressively worse. The dog was minimally pruritic. On physical examination, moderate multifocal areas of alopecia and lichenification were observed on the dorsal topline with extension to the neck, shoulders, hips, and proximal limbs. Six separate, oozing, variably sized dermal ulcers were present along the back from the shoulder blades to the area near the base of the tail. Numerous pustules, macules, and papules were distributed along the skin of the back, and the skin was noticeably thickened. Multiple skin scrapings and dermatophyte cultures were negative, and complete blood counts were within normal limits. Serum chemistry abnormalities Received for publication May 13, 1997.(normal ranges given in parentheses throughout) included 901 U/liter (Ͻ141 U/liter) alkaline phosphatase and 258 U/ liter (Ͻ85 U/liter) ALT. The urine specific gravity was 1.0...

show abstract

mentioning

confidence: 99%

Multiple Cutaneous Metaplastic Ossification Associated with Iatrogenic Hyperglucocorticoidism

et al. 1998

View full text Add to dashboard Cite

show abstract

“…O diagnóstico diferencial desta entidade é bem limitado, uma vez que seu fenó-tipo, a história clínica e os achados radiográficos praticamente fecham o quadro. Outras causas de ossificação ectópica devem sempre ser lembradas, tais como: osteodistrofia hereditária de Albright, calcificação heterotópica pseudomaligna, heteroplasia óssea progressiva e até mesmo o osteossarcoma (17,18) .…”

Section: Discussionunclassified

Fibrodisplasia ossificante progressiva: relato de caso e achados radiográficos

Júnior¹,

Carvalho²,

Costa³

et al. 2005

Radiol Bras

View full text Add to dashboard Cite

Araújo Júnior CR et al. Radiol Bras 2005;38(1):6973 69A expressão fibrodisplasia ossificante progressiva substitui a antigo nome miosite ossificante (6) , por ser a osteogênese ectópica ocorrendo no tecido conjuntivo, podendo afetar músculos, fáscias, ligamentos, tendões e cápsulas articulares.O doente geralmente apresenta ossificação progressiva do tecido conjuntivo, que causa uma limitação crescente da mobilidade osteoarticular, afetando principalmente a coluna vertebral, ombros, quadril e articulações periféricas. O paciente assume uma postura única e limitada, não podendo sequer sentar. Este aspecto caracteriza a forma avançada da doença, recebendo a denominação de síndrome do "stone man", antes descrita em apenas cerca de 600 doentes . Nesse estágio, o paciente rapidamente evolui para a morte, em conseqüência de problemas respiratórios de origem restritiva.Apresentamos um caso de FOP com 14 anos de evolução em um paciente de 22 anos de idade, do sexo masculino, manifestando grave comprometimento funcional, e discutimos os aspectos radiográficos característicos desta entidade. A fibrodisplasia ossificante progressiva é uma doença genética rara do tecido conjuntivo, caracterizada por ossificação disseminada em tecidos moles e alterações congênitas das extremidades. Sua transmissão é autossômica dominante, com penetrância completa, mas expressão variável. O início ocorre na infância e o envolvimento progressivo axial e da região proximal dos membros leva a uma conseqüente imobilização e deformação articular. Apresentamos um caso de um paciente de 22 anos de idade, do sexo masculino, com quadro clínico característico de fibrodisplasia ossificante progressiva e discutimos os últimos avanços no diagnóstico e na fisiopatogenia desta entidade. Unitermos: Fibrodisplasia ossificante progressiva; Miosite ossificante progressiva; Fisiopatogenia; Ossificação; Raios-X.Fibrodysplasia ossificans progressiva: a case report and radiographic findings. Fibrodysplasia ossificans progressiva is a rare hereditary connective tissue disease characterized by disseminated soft tissue ossification and congenital abnormality of the extremities. It is genetically inherited as a dominant trait with complete penetrance but variable expression. The onset takes place during childhood and the progressive involvement of the spine and proximal extremities leads to immobilization and articular deformity. We report a case of a 22-year-old male patient with typical symptoms of fibrodysplasia ossificans progressiva and discuss the new advances in the diagnosis and pathophysiology.

show abstract

“…Other disorders of ectopic ossification may be considered, such as Albright hereditary osteodystrophy, pseudomalignant heterotopic ossification, progressive osseous heteroplasia and even osteosarcoma [21][22][23] .…”

Section: Discussionmentioning

confidence: 99%

Fibrodysplasia ossificans progressiva: case report

Nucci

Queiróz

Santos

et al. 2000

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

-Fibrodysplasia ossificans progressiva is a rare genetic disease characterized by widespread soft tissue ossification and congenital stigmata of the extremities. We report on a male child followed for ten years since the age of 3 years and 9 months, when the diagnosis was made. He was born with bilateral hypoplasic hallux valgus and ventricular septal defect, corrected by transsternal approach when 32 months old. Restriction of neck mobility followed and foci of ectopic ossification appeared. Four crises of disease exacerbation were treated with oral prednisone and/or other antiinflammatory drugs. Sodium etidronate 5 to 10 mg/kg/day was prescribed intermittently during about six years but was discontinued due to osteopenia. The disease course has been relentless, with severe movement restriction including the chest wall. A review showed few similar case reports in the Brazilian literature. We revisit the criteria for diagnosis and the essentials of management and treatment.KEY WORDS: fibrodysplasia ossificans progressiva, myositis ossificans, muscle biopsy, rigid spine, Tc-MDP scan, etidronate treatment. Fibrodisplasia ossificante progressiva: relato de casoRESUMO -Fibrodisplasia ossificante progressiva é uma doença genética rara, caracterizada por ossificação disseminada em tecidos moles e estigmas congênitos nas extremidades. Relatamos sobre uma criança do sexo masculino, branca, que vem sendo acompanhada desde seus 3 anos e 9 meses quando diagnosticamos a doença. Nascida com halux valgo hipoplásico bilateral e defeito do septo interventricular, submeteu-se a correção cirúrgica através de esternotomia, aos 32 meses de vida. Seguiram-se progressiva restrição de movimentos cervicais e aparecimento de nódulos duros e dolorosos em regiões paravertebrais cérvico-torácicas. Em dez anos, assistimos a quatro episódios bem definidos de exacerbação da doença, com acréscimos de ossificação ectópica. Nessas ocasiões, prescrevíamos prednisona oral e/ou antiinflamatórios não hormonais. Etidronato de sódio, 5 a 10 mg/ kg/dia, foi utilizado em períodos de dois ou três meses, com intervalos de dois meses, durante seis anos. Osteopenia obrigou-nos a interromper o uso do quelante. A evolução tem sido inexorável, com limitação intensa de movimentos torácicos e corpóreos. A revisão da literatura brasileira revelou poucos casos semelhantes. Enfatizamos os critérios para diagnóstico e princípios básicos de tratamento. PALAVRAS-CHAVE: fibrodisplasia ossificante progressiva, miosite ossificante, biópsia muscular, espinha rígida, cintilografia com MDP99m

show abstract

Progressive osseous heteroplasia

Cited by 11 publications

References 0 publications

Multiple Cutaneous Metaplastic Ossification Associated with Iatrogenic Hyperglucocorticoidism

Multiple Cutaneous Metaplastic Ossification Associated with Iatrogenic Hyperglucocorticoidism

Fibrodisplasia ossificante progressiva: relato de caso e achados radiográficos

Fibrodysplasia ossificans progressiva: case report

Contact Info

Product

Resources

About