Osteoma Cutis Clinical Presentation
- Author: Luke Lennox, MD; Chief Editor: William D James, MD more...
Patients may report having hard areas in the skin. A familial occurrence of Albright hereditary osteodystrophy may be present.
The presentations of osteoma cutis can be highly variable, with clinical entities that are defined by the number, the form, and the location of the lesions. There are four clinical types: isolated, widespread, multiple miliary facial, and platelike osteomas. Hence, they may present as single or multiple hard nodules, miliary tumors, or plaques.[5, 6] The face, scalp, extremities, digits, and subungual areas are most commonly affected.
See the images below.
Osteoma cutis can be a feature of several groups of patients. In can be secondary to inflammatory skin diseases, trauma/scars, and cutaneous tumors. It can also occur with genetic syndromes or in isolation.
Albright hereditary osteodystrophy is due to an autosomal dominant defect in the alpha subunit of intracellular G proteins. The characteristic phenotype includes short stature, round facies, defective teeth, mental retardation, brachydactyly (fourth and fifth metacarpals/"knuckle knuckle dimple dimple" of the Archibald sign), and osteomas of the soft tissue and the skin. Classically, it presents with pseudohypoparathyroidism (elevated parathyroid hormone [PTH], hypocalcemia, hyperphosphatemia secondary to renal PTH resistance). Tetany is often the presenting sign, secondary to the low calcium level. Albright hereditary osteodystrophy can cause osteoma cutis without endocrine abnormalities in the pseudo-pseudohypoparathyroidism variant.
Progressive osseous heteroplasia is also, like Albright hereditary osteodystrophy, associated with a defect in the alpha subunit of G proteins (GNAS1 gene). It is characterized by ossification of the dermis in infancy, with progression to the subcutaneous and deeper connective tissues throughout childhood. It is not associated with endocrine changes, but it can have a severe affect on growth and joint mobility.
Congenital platelike osteomatosis, a type of primary osteoma cutis, meets the following criteria:
Lesion present at birth (or within first year of life)
No evidence of abnormal calcium or phosphorous metabolism
No evidence of trauma or infection
Presence of at least one bony plate
Congenital platelike osteomatosis is most commonly found on the scalp. It should be monitored, as its diagnosis could be representative of a slow-evolving progressive osseous heteroplasia (something with much more severe consequences).
Fibrodysplasia ossificans heteroplasia and fibrodysplasia ossificans progressiva (stone man syndrome) are possible causes.
Osteoma cutis can be found in patients with Gardner syndrome, which includes colonic polyposis, retinal hyperplasia, and other osseous and soft tissue growths.
Multiple miliary osteomas of the face often present in patients with a history of severe acne, sunburn, neurotic excoriation, or dermabrasion. These small, hard papules can have a bluish hue, especially in patients who have been exposed to tetracycline treatment.[9, 4]
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