Flashcards in Chapter 50 - Calcifying Ossifying Disorders Deck (15):
What are the 4 types of cutaneous calcification?
1) dystrophic, 2) metastatic, 3) iatrogenic, 4) idiopathic
What are some examples of AI CTDs that exhibit dystrophic calcification?
Scleroderma (CREST syndrome), dermatomyositis
What are some types of panniculitis that may be associated with dystrophic calcification?
Pancreatitis/carcinoma, SC fat necrosis of newborn, lupus profundus
What are some genetic disorders that may be associated with dystrophic calcification?
Pseudoxanthoma elasticum, Ehlers Danlos syndrome, porphyria cutanea tarda, Werner syndrome, Rothmund Thompson syndrome
What are some neoplasms that may be associated with dystrophic calcification?
Pilomatricoma, pilar cysts, BCC, chondroid syringomas, melanocytic nevi, AFX, pyogenic granuloma, trichoepitheliomas, SKs
What are some causes of metastatic calcification?
Chronic renal failure (most common), milk-alkali syndrome, hypervitaminosis D, hyperparathyroidism, neoplasms
What are the clinical features of calciphylaxis?
Violaceous reticulated patches, +/- bullae, ulcer, extremely painful, death due to gangrene and sepsis
What are the 4 genetic d/o with ossification as a regular feature?
1) fibrodysplasia ossificans progressiva, 2) progressive osseous heteroplasia, 3) plate like osteoma cutis, 4) Albright hereditary osteodystrophy
What is miliary osteoma?
Common foci of cutaneous ossifcation on the face, may be associated with crhonic acne vulgaris, multiple small firm papules (skin, white, blue color)
What lab work would you consider ordering in a patient with a possible calcification/ossification disorder?
Serum calcium, phosphate, PTH, and vitamin D3, in addition to 24 hour urinary calcium excretion. Also, consider ordering protein C activity and function, if calciphylaxis is suspected.
True or false: the treatment of military osteoma cutis is primarily surgical.
True; laser resurfacing with curettage of exposed osseous fragments has also be described, as has tretinoin gel
Patients with progressive osseous heteroplasia, plate-like osteoma cutis, and Albright hereditary osteodystrophy all have mutations in which gene?
GNAS1, which encodes the alpha subunit of the stimulatory G protein which regulates adenyl cyclase activity (adenyl cyclase is believed to be a regulator of bone formation)
What are the two main mechanisms of bone formation that can be seen in disorders of cutaneous ossification?
Endochondral and intramembranous
There are four genetic disorders that feature cutaneous or subcutaneous ossification as a regular feature. What are they?
Fibrodysplasia ossificans progressiva, progressive osseous heteroplasia, plate-like osteoma cutis, and Albright hereditary osteodystrophy