Atrophies of Connective Tissue

Question 1

What is the clinical appearance of mid-dermal elastolysis?

Answer 1

Circumscribed areas of fine wrinkling

Question 2

What areas are most commonly affected by mid-dermal elastolysis?

Answer 2

Symmetric areas on trunk lateral neck, and extremities

Question 3

Histology of mid-dermal elastolysis?

Answer 3

Normal H+E

Elastic tissue stains demonstrate loss of elastic fibers in the mid dermis only

Elastic stains (Verhoeff-van Gieson or Weigert’s stain)

Question 4

Clinical appearance of anetoderma?

Answer 4

1-2 cm areas of flaccid/wrinkled skin, usually elevated (>depressed or flat)

Question 5

Most common areas of involvement for anetoderma?

Answer 5

Neck, trunk, upper extremitites

Question 6

What are the two broad types of anetoderma?

Answer 6

Primary vs Secondary

Question 7

What are the two types of primary anetoderma?

Answer 7

1. Preceding inflammatory lesions (Jadassohn-Pellizzari type)
2. No preceding inflammatory lesions (Schweninger-Buzzi type)

Question 8

In secondary anetoderma, what are some associated skin disorders?

Answer 8

Infection, penicillamine use, inflammatory dermatoses, autoimmune (lupus, Sjogren’s, Graves’), cutaneous tumors

Question 9

Histology of anetoderma?

Answer 9

Normal H+E

Elastic tissue stain shows complete loss of elastic fibers in papillary AND reticular dermis (as compared to mid-dermal elastosis

Fragmented elastic fiber remnants visible

Question 10

Treatment for anetoderma?

Answer 10

Nothing has been proven to work

Hydroxychloroquine has been tried

Surgical excision may be helpful for localized lesions

Question 11

What are the 3 main types of striae?

Answer 11

Striae rubra, striae alba, striae distensa

Question 12

What are striae rubra?

Answer 12

Initially, red-to-violaceous elevated lines that can be mildly pruritic, usually earlier phase

Question 13

What is the clinical of striae alba?

Answer 13

Over time striae rubra fade in color and you get atrophic skin surface w/ fine wrinkled appearance. These are usually permanent

Question 14

What are striae distensae?

Answer 14

Abdomen, breast, thighs of pregnant women, especially during last trimester

Question 15

Clinical of atrophoderma of pasini and pierini?

Answer 15

Large (up to 20cm) brownish-gray hyperpigmented oval, atrophic, well-demarcated plaques w/ sharp sloping borders (“cliff drop”)

Question 16

Most common areas of involvement of atrophoderma of pasini and pierini?

Answer 16

Trunk/upper arms of young females (typically 2nd-3rd decade)

Question 17

Clinical progression of atrophoderma of pasini and pierini?

Answer 17

Starts as a single lesion with more lesions forming over time

Question 18

What is the histology of atrophoderma of Pasini and Pierini?

Answer 18

Decreased dermal thickness compared to normal skin

If concerned you need to bx normal adjacent skin and lesional skin for comparison

Question 19

What is the linear atrophoderma of Moulin?

Answer 19

Linear atrophoderma –> Chronic but non-progressive and benign

• There is less induration and pigmentary changes compared with other forms of morphea
• Tends to follow the lines of Blaschko

Question 20

What is the clinical presentation of follicular atrophoderma?

Answer 20

Dimple-like depressions that are follicular-based

Described as “ice-pick” depressions

Are located on the dorsal hands/feet and cheeks

Question 21

What is the distribution of follicular atrophoderma?

Answer 21

Dorsal hands/feet and cheeks

Question 22

When does follicular atrophoderma present?

Answer 22

Usually present at birth or early in life

Question 23

What is the histology of follicular atrophoderma?

Answer 23

Dilated follicles w/plugging, inflammation and dermal collagen sclerosis

Question 24

What conditions are follicular atrophoderma associated with?

Answer 24

Bazex-Dupre-Christol syndrome, Conradi-Hünermann-Happie Syndrome, Ichthyosis-hypotrichosis syndrome, Rhizomatic chondrodysplasia punctata

Bolded are the main ones, others are lower yield

Question 25

What is atrophia maculosa varioliformis cutis?

Answer 25

Small round “varioliform” and linear facial depressions Spontaneously on cheeks +/- forehead or chin No history of trauma

Question 26

What is the clinical of atrophoderma vermiculatum?

Answer 26

Variant of follicular atrophoderma (more connected/almost serpiginous configuration) - On the face/cheeks ONLY

Question 27

What syndromes have atrophoderma vermiculatum?

Answer 27

Rombo syndrome: atrophoderma vermiculatum, milia, acral erythema, peripheral vasodilation w/ cyanosis, mult. BCC, mult trichoeps Nicolau-Balus syndrome: generalized eruptive syringomas, atrophoderma vermiculatum, milia Tuzun (scrotal tongue) Braun-Falco-Marghescu (PPK and KP)

Question 28

What is KP atorphicans, atrophoderma vermiculatum subtype?

Answer 28

Usually sporadic, AD Erythematous papules w/ follicular plugging, horn cysts, and atrophic cribriform scarring cheek and forehead. Typically presents b/w 5-12yo

Question 29

What are piezogenic pedal papules?

Answer 29

Herniation of fat through fascia of lateral heels Best seen when a patient is standing w/ weight placed on heal

Question 30

What syndromes can piezogenic pedal papules be seen with?

Answer 30

A/w Classical Ehlers-Danlos syndrome + Prader-Willi syndrome

Question 31

How is the infantile variant of piezogenic pedal papules different than the other forms?

Answer 31

Larger nodules on the medial aspect of the heel