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Dermatology Board Prep > DERM (13) Metabolic and Systemic Disease > Depositional disorders and Calcification Disorders > Flashcards

Depositional disorders and Calcification Disorders Flashcards

1
Q

What is the histopathology of gout?

A

Amorphous (Pink cotton candy) w/ needle-like clefts in the dermis. Giant cells around material

  • Negative birefringence
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2
Q

What are the clinical cutaneous findings of gout?

A

Monosodium urate crystal deposition in the tissue

  • Gouty arthritis is the most common, can also get nephrolithiasis/renal impairment, and in the skin you get gouty tophi (firm white/yellow subcutaneous nodules
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3
Q

Risk factors for gout?

A

Obesity, EtOH, renal issues, diuretic meds

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4
Q

What are the most common areas to get gouty tophi?

A

Most common non the helix of the ear and skin overlying small joints (fingers, toes)

  • <10% of pts get gouty tophi
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5
Q

What is the best preservative for samples thought to be gout?

A

Ethanol

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6
Q

What are the treatment options for gout?

A

NSAIDs, colchicine, allopurinol, febuxostat

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7
Q

What is pseudogout?

A

It is on the ddx for gout. More commonly affects the knees or other bigger joints and can be associated with osteoarthritis.

  • IT is from the deposition of calcium pyrophosphate crystals (rhomboidal, positive birefringence)
  • Can cause cutaneous tophi too
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8
Q

What is the histology of colloid milium?

A

Amorphous homogenous pink nodular material in superficial dermis +/- clefts

  • Grenz zone
  • Solar elastosis is seen deep to nodules in adult type
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9
Q

What are the two broad types of colloid milium?

A

Adult type and other types (includes juvenile, pigmented (in setting of hydroquinone), nodular colloid degeneration)

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10
Q

What are the clinical presentations of colloid milium?

A

Adult type (most common): pts usually middle-aged or older and severely photodamaged, M>F; multiple translucent yellowish papules in photo-exposed areas

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11
Q

What disease can colloid milium stain like?

A

Can stain like amyloid (congo red, thioflavin T, crystal violet positive) PAS+ as well

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12
Q

What 5 situations is dystrophic calcification most commonly seen in?

A
  • Autoimmune connective tissue disease
  • Panniculitis
  • Genodermatoses
  • Infections
  • Neoplasms
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13
Q

In what autoimmune disorders are dystrophic calcification seen in?

A

CREST and childhood DM (in DM can progress to calcinosis Universalis which is the severe form)

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14
Q

What types of panniculitis is dystrophic calcification seen in?

A

Pancreatic panniculitis is most common, but lobular panniculitis can. Also subcutaneous fat necrosis of the newborn and lupus profundus

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15
Q

In what genoderms can dystrophic calcification be seen in?

A

Pseudoxanthoma elastic (calcification of dermal elastic fibers), Ehlers-Danlos syndrome, PCT (longstanding)

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16
Q

What infections are associated with dystrophic calcium?

A

Onchocera volvulus and Taenia solium

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17
Q

What neoplasms can dystrophic calcium be seen in?

A

Pilomatricoma (75%), BCC, epidermal/pilar cysts

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18
Q

What is the histology of metastatic calcification?

A

Think calciphylaxis: perivascular and intravascular calcium in vessels within the subcutaneous fat –> thrombotic vasculopathy

19
Q

What is the clinical appearance of metastatic calcification?

A

Violaceous reticulated patches and livedo + subcutaneous nodules

  • these evolve into painful necrotic, purpuric ulcers/bullae
20
Q

What is the treatment for malignant calcification/calciphylaxis?

A

Low calcium dialysis, phosphate binders, sodium thiosulfate (IV or intralesional), parathyroidectomy (check parathyroid hormone), surgical debridement, management of tissue infections

21
Q

What is the biggest risk factor for calciphylaxis/metastatic calcification?

A

Chronic renal failure (decreased phosphate clearance, decreased 1-alpha hydroxylation D3, decreased calcium absorption, secondary hyperparathyroidism can also play a role, as well as milk-alkali syndrome and hypervitaminosis D

22
Q

What are the two most common forms of idiopathic calcification?

A

idiopathic calcified nodules of the scrotum and tumor calcinosis

23
Q

What is the histology of idiopathic calcification?

A

Calcified nodules of the scrotum; may see calcification within an epidermal cyst too

24
Q

What is tumor calcinosis?

A

Large painful calcium-phosphate subcutaneous nodules around joints leading to ulceration

25
Q

What types of idiopathic calcification can be seen in Down syndrome?

A

Milia-like calcinosis that is often seen on the dorsal hands/face

26
Q

In what settings can metastatic calcinosis be seen?

A

Chronic renal failure (calciphylaxis, benign nodulear calcification of renal disease)

  • Hypervitaminosis D
  • Milk-alkali syndrome
  • Sarcoidosis
  • Tumoral calcinosis (familial; hyper-or normophosphatemic)
  • Hyperparathyroidism
  • Neoplasms (multiple myeloma, adult t-cell leukemia/lymphoma, SCC of the lung or head and neck)
27
Q

How can steroids contribute to dystrophic calcification?

A

Steroids decrease calcium absorption in the gut which increases PTH

28
Q

What is the most common cause of death in calciphylaxis?

A

Sepsis

29
Q

What is the most specific stain for calcium?

A

Most specific is alizarin red

30
Q

What is the histology of osteoma cutis?

A

Bone formation within the dermis or subcutis

(hydroxyapatite (calcium/phophate) and protein matrix)

31
Q

What 4 genetic conditions are associated with osteoma cutis?

A

Fibrodysplasia ossificans progressiva, Progressive osseous heteroplasia, Albright hereditary osteodystrophy, and Plate-like osteoma cutis

32
Q

What gene mutation causes the 4 types genetic osteoma cutis?

A

GNAS1 mutations

33
Q

What do mutations in GNAS1 lead to osteoma cutis?

A

GNAS is thought to regulate adenyl cyclase activity which is thought to be a negative regulator of bone formation –> clinical phenotypes of the 4 condition thought to be 2/2 imprinting

34
Q

What is an important feature of fibrodysplasia ossificans progressiva?

A

Malformed great toes

35
Q

What genetic forms of osteoma cutis are progressive?

A

Fibrodysplasia ossificans progressiva and progressive osseous heteroplasia

36
Q

What forms of genetic osteoma cutis are limited?

A

Albright hereditary osteodystrophy and Plate-like osteoma cutis

37
Q

What other features are seen in Albright hereditary osteodystrophy?

A

Pseudohypoparathyroidism, pseudo-pseudohypoparathyroidism, obesity, brachydactyly, short stature

38
Q

Where do miliary osteomas occur?

A

Occur on the face

39
Q

What is the presentation of miliary osteomas?

A

White-skin colored tiny papules on faces of adults

40
Q

What is miliary osteomas associated with?

A

Prior acne treatment and tetracyclines

41
Q

What is the knuckle knuckle dimple dimple sign?

A

It is seen in Albright hereditary osteodystrophy from the pseudohypoparathyroidism (type 1A)

Type 1a Pseudohypoparathyroidism is clinically manifest by bone resorption with blunting of the fourth and fifth knuckles of the hand, most notable when the dorsum of the hand is viewed in closed fist position. This presentation is known as ‘knuckle knuckle dimple dimple’ sign (Archibald’s sign)

42
Q

What is lipoid proteinosis?

A

AR dz due to loss-of-function of ECM1 (encodes extracellular matrix protein 1) –> deposition of hyaline-like material in multiple sites including the brain (seizures) larynx (hoarnessness), as well as the skin and oral mucosa

43
Q

What is the clinical presentation of lipoid proteinosis?

A

Skin-colored to yellowish waxy papules and nodules that favor the face, including the eyelid margin and the tongue

  • Can also see atrophic scarring, verrucous changes especially on the elbows and knees, and diffuse waxy appearance

DERM (13) Metabolic and Systemic Disease (7 decks)

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