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Congenital plate-like osteoma cutis of the forehead: an atypical presentation form
L Tresserra1, F Tresserra, P J Grases
1Oral and Maxillo Facial Surgery Service, Instituto Universitario Dexeus, Barcelona, Spain.
Insights
Primary osteoma cutis, a rare condition of bone formation in the skin, can manifest as congenital plate-like osteoma cutis. This case highlights a 7-year-old girl with non-progressive cutaneous ossifications.
Area of Science:
- Dermatology
- Genetics
- Pathology
Background:
- Osteoma cutis, or cutaneous ossification, is associated with various syndromes and can be primary or secondary.
- Primary osteoma cutis, appearing in infancy without metabolic issues or trauma, includes congenital plate-like osteoma cutis and progressive osseous heteroplasia.
- Progressive osseous heteroplasia is characterized by deep connective tissue ossifications alongside osteoma cutis.
Observation:
- A 7-year-old female presented with a plate-like cutaneous ossification on her forehead causing aesthetic concerns.
- Additional dermal ossifications were noted in the inguinal, preauricular, and left hand regions.
- A solitary focus within the extraocular muscles of the left orbit was also identified.
Findings:
- The observed cutaneous ossifications were non-progressive, with most present since birth.
- The forehead lesion was surgically addressed.
- The lack of progression and solitary orbital lesion supported a diagnosis of plate-like osteoma cutis.
Implications:
- Plate-like osteoma cutis and progressive osseous heteroplasia may represent a spectrum of the same underlying disorder.
- Accurate diagnosis is crucial for understanding prognosis and potential management strategies.
- Further research into the genetic and molecular mechanisms of these ossification disorders is warranted.
Abstract:
Cutaneous ossifications or osteoma cutis can be found in many syndromes. Primary osteoma cutis, present since birth or the first months of life, in the absence of metabolic disorders or trauma, is found in congenital plate-like osteoma cutis and progressive osseous heteroplasia, coexisting in the latter with deep connective tissue ossifications. This report documents the case of a 7-year-old female with a plate-like cutaneous ossification of the forehead causing aesthetic deformities. Other dermal ossifications in the inguinal and preauricular region, as well as the left hand and a small focus in the extraocular muscles of the left orbit, were also present. These lesions did not show progression, and most of them were present since birth. The lesion of forehead was treated surgically. The lack of progression and the fact that the orbital lesion was solitary still fits the criteria for the diagnosis of plate-like osteoma cutis. Plate-like osteoma cutis and progressive osseous heteroplasia may represent varieties of the same disorder.