Atrophies of Connective Tissue Flashcards
What is the clinical appearance of mid-dermal elastolysis?
Circumscribed areas of fine wrinkling
What areas are most commonly affected by mid-dermal elastolysis?
Symmetric areas on trunk lateral neck, and extremities
Histology of mid-dermal elastolysis?
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)
Clinical appearance of anetoderma?
1-2 cm areas of flaccid/wrinkled skin, usually elevated (>depressed or flat)
Most common areas of involvement for anetoderma?
Neck, trunk, upper extremitites
What are the two broad types of anetoderma?
Primary vs Secondary
What are the two types of primary anetoderma?
- Preceding inflammatory lesions (Jadassohn-Pellizzari type)
- No preceding inflammatory lesions (Schweninger-Buzzi type)
In secondary anetoderma, what are some associated skin disorders?
Infection, penicillamine use, inflammatory dermatoses, autoimmune (lupus, Sjogren’s, Graves’), cutaneous tumors
Histology of anetoderma?
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
Treatment for anetoderma?
Nothing has been proven to work
Hydroxychloroquine has been tried
Surgical excision may be helpful for localized lesions
What are the 3 main types of striae?
Striae rubra, striae alba, striae distensa
What are striae rubra?
Initially, red-to-violaceous elevated lines that can be mildly pruritic, usually earlier phase
What is the clinical of striae alba?
Over time striae rubra fade in color and you get atrophic skin surface w/ fine wrinkled appearance. These are usually permanent
What are striae distensae?
Abdomen, breast, thighs of pregnant women, especially during last trimester
Clinical of atrophoderma of pasini and pierini?
Large (up to 20cm) brownish-gray hyperpigmented oval, atrophic, well-demarcated plaques w/ sharp sloping borders (“cliff drop”)
Most common areas of involvement of atrophoderma of pasini and pierini?
Trunk/upper arms of young females (typically 2nd-3rd decade)
Clinical progression of atrophoderma of pasini and pierini?
Starts as a single lesion with more lesions forming over time
What is the histology of atrophoderma of Pasini and Pierini?
Decreased dermal thickness compared to normal skin
If concerned you need to bx normal adjacent skin and lesional skin for comparison
What is the linear atrophoderma of Moulin?
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
What is the clinical presentation of follicular atrophoderma?
Dimple-like depressions that are follicular-based
Described as “ice-pick” depressions
Are located on the dorsal hands/feet and cheeks
What is the distribution of follicular atrophoderma?
Dorsal hands/feet and cheeks
When does follicular atrophoderma present?
Usually present at birth or early in life
What is the histology of follicular atrophoderma?
Dilated follicles w/plugging, inflammation and dermal collagen sclerosis
What conditions are follicular atrophoderma associated with?
Bazex-Dupre-Christol syndrome, Conradi-Hünermann-Happie Syndrome, Ichthyosis-hypotrichosis syndrome, Rhizomatic chondrodysplasia punctata
Bolded are the main ones, others are lower yield
What is atrophia maculosa varioliformis cutis?
Small round “varioliform” and linear facial depressions
Spontaneously on cheeks +/- forehead or chin
No history of trauma
What is the clinical of atrophoderma vermiculatum?
Variant of follicular atrophoderma (more connected/almost serpiginous configuration)
- On the face/cheeks ONLY
What syndromes have atrophoderma vermiculatum?
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)
What is KP atorphicans, atrophoderma vermiculatum subtype?
Usually sporadic, AD
Erythematous papules w/ follicular plugging, horn cysts, and atrophic cribriform scarring cheek and forehead.
Typically presents b/w 5-12yo
What are piezogenic pedal papules?
Herniation of fat through fascia of lateral heels
Best seen when a patient is standing w/ weight placed on heal
What syndromes can piezogenic pedal papules be seen with?
A/w Classical Ehlers-Danlos syndrome + Prader-Willi syndrome
How is the infantile variant of piezogenic pedal papules different than the other forms?
Larger nodules on the medial aspect of the heel
