CM- Approach to Scaling Disorders Flashcards

1
Q

Define the terms guttate, nummular, pityriasis.

A

Guttate - drop shaped
Nummular- shaped like a coin
Pityriasis- bran-like flakes of scale

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2
Q

What is a scale?

A

piece of stratum corneum. Usually they are so small that they go undetected, but in disorders, abnormal stratum corneum accumulates and forms visible flakes

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3
Q

What are the 3 main functions of skin?

A
  1. mechanical protection
  2. prevent water loss
  3. immunological defense
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4
Q

Describe the normal development of the skin [keratinization].
What 3 disruptions to keratinization can lead to scaling?

A

Pool of stem cells in basal cells mature as they rise to the stratum corneum. The end result takes 2 weeks and is an anucleate, non-viable corneocyte that populates the most superficial layer of the epidermis [80% protein, 20% lipids]
Shedding takes 2 weeks after degradation of the lipids and cell adhesion proteins.

Scaling can be caused by:

  1. increased proliferation of basal keratinocytes
  2. abnormal maturation
  3. increased retention of corneocytes
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5
Q

What is the effect of inflammation on scaling?

A

Inflammatory conditions –> released cytokines–> increased proliferation of basal keratinocytes

This results in excess, incompletely matured keratinocytes–> nucleated stratum corneum [parakeratosis]

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6
Q

What is parakeratosis?

What is the cause?

A

It is nucleated stratum corneum cells. It is caused by increased proliferation of basal keratinocytes leading to excess of incompletely matured cells

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7
Q

What is the pathogenesis of psoriasis?

A
  1. genetic predisposition [HLA-B27]

2. immune triggers that inappropriately activate the immune response

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8
Q

What is the histology of psoriasis?

A
  1. inflammatory cells in the epidermis and dermis
  2. accumulation of neutrophils [munro’s microabsesses]
  3. increased mitoses in keratinocytes, fibroblasts, endothelial cells
  4. thickened epidermis [acanthosis]
  5. retention of nuclei in the horny layer with absence of granular layer
  6. parakeratotic hyperkeratosis [nucleated stratum corneum]
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9
Q

What are the 5 clinical patterns of psoriasis?

A
  1. plaque type [classic and most common]
  2. guttate type [drop-shaped]
  3. Inverse [ axilla, gluteal cleft] macerated/less obvious
  4. erythrodermic -diffuse over whole body
  5. pustular - localized to palms or soles
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10
Q

What 3 diagnostic clues point to psoriasis?

A
  1. Koebner phenomenon - primary lesion induced by trauma to the skin
  2. Nail involvement - pits, oil-drop changes, onycholysis [detachment of nail from nail bed]
  3. arthritis [asymmetric]
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11
Q

What areas of the body tend to be affected by psoriasis?

A
  1. scalp
  2. nails
  3. sacral area
  4. extensor surfaces
  5. shins & knees
  6. elbows
  7. palms and soles
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12
Q

What is the pathogenesis of secondary stage syphilis?

A

Cutaneous dissemination of treponema pallidum

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13
Q

Describe the histology of secondary syphilis.

A
  1. epidermal hyperkeratosis [thickening of stratum corneum]
  2. perivascular monocytes, lymphocytes and plasma cells in the dermal layer
  3. dilation and proliferation of capillaries and lymphatics in the dermis
  4. silver stain shows spirochetes
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14
Q

What is the “classic” presentation of secondary syphilis?

A
  1. red/brown macules or papules on trunk AND extremities [freq. palms and soles]
  2. diffuse hair loss/ lateral 1/3 of eyebrow loss
  3. “great imitator” - can mimic:
    - ptyriasis rosea
    - guttate psoriasis
    - lichen planus
    - sarcoidosis
  4. condyloma lata- vesicles/pustules or mucus patches
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15
Q

What is the histology of seborrheic dermatitis?

A
  1. parakeratosis focally around the edges of follicular ostia [opening where hair emerges from the follicle]
  2. spongiosis [intracellular edema] with neutrophilic infiltrate
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16
Q

What are common locations for seborrheic dermatitis?

A
  1. scalp
  2. eyebrows
  3. eyelids
  4. nasolabial folds
  5. ears
  6. sternum
  7. axillae
  8. submammory folds
  9. umbilicus
  10. groin
  11. gluteal creases
17
Q

What are the 5 types of seborrheic dermatitis?

A
  1. Classic - “greasy” yellowish scales on face, dandruff on scalp
  2. infantile - prominent scalp involvement
  3. “sebopsoriasis”- mixed psoriasis and seborrheic dermatitis
  4. Erythroderma
  5. immunosuppressed have it florid and treatment resistant
18
Q

What is the histology of pityriasis rosea?

A
  1. parakeratosis that is patchy or diffuse and contains serum
  2. spongiotic dermatitis with perivascular lymphocytic infiltrate
19
Q

A patient presents with a herald patch [open ring] on the trunk with crops of smaller round to oval salmon-color patches following lines of cleavage [“christmas tree pattern”]. There are peripherally attached thin scales.
What does this pattern describe?

A

Pityriasis rosea

20
Q

Describe the histology of pityriasis lichenoides.

A
  1. parakeratosis and spongiosis in the epidermis
  2. keratinocyte necrosis
  3. erythrocytes in the epidermis
  4. wedge-shaped inflammatory cell infiltrate in the dermis
21
Q

Describe the acute and chronic presentation of pityriasis lichenoides.

A

Acute - bright red, erythematous papules that can erode/ulcerate

Chronic:

  1. red-brown lesion
  2. micaceous [aluminum silicate chalky residue] scale
  3. post inflammatory hyperpigmentation
22
Q

You are looking at a slide and see:

  1. irregular acanthosis with hypergranulosis
  2. hyperkeratosis
  3. Civatte/colloid bodies
  4. band like infiltrate at dermal-epidermal junction
  5. pigment incontinence - loss of melanin from basal cells with accumulation in upper dermis melanophages

What is the likely diagnosis?

A

Lichen planus

23
Q

A patient presents with flat-topped, polygonal violaceous papules that coalesce into plaques. The surface has a fine scale with a network of white lines [Wickham striae] going through.
The lesions are primarily on the wrist, pretibial area and mucus membranes including oral lesions - buccal mucosa with white papules in reticular pattern. The patients nails are rough with longitudinal ridging, thinning and pterygium formation.

What does the patient have?

A

Lichen planus

24
Q

What is the histology of lichen simplex chronicus?

A
  1. Hyperplasia of the epidermis
  2. hyperkeratosis
  3. hypergranulosis
  4. acanthosis
  5. elongated and irregular rete ridges
  6. spongiosis
25
Q

A patient present with a thickened and hyperpigmented plaque. Skin lines are visible and the area is sharply demarcated. There is some excoriations and pruritus is a prominent feature. What is the likely diagnosis?

A

Lichen simplex chronicus

26
Q

Most ichthyoses have non-specific histology but share what 2 features?
Which 2 types of ichthyoses have unique features and what are they?

A
  1. compact hyperkeratosis
  2. normal or thickened granular layer

Ichthyosis vulgaris - decreased or absent granular layer

Epidermolytic hyperkeratosis-

  1. hypergranulosis with giant keratohyalin granules
  2. keratinocyte lysis resulting in subcutaneous vacuolization and vesicles
27
Q

You look at histology of a skin lesion and see:

  1. hypergranulosis with giant keratohyalin granules
  2. keratinocyte lysis with subcutaneous vacuolization and vesicles

What is the diagnosis?

A

Epidermolytic hyperkeratosis

28
Q

What is the inheritance pattern of ichthyosis vulgaris?
What is the mutation in?
What is the presentation?

A

It is an autosomal dominant mutation in filaggrin.

Patient will present with non-inflamed scaling that can be fine or thick. Patients can also present with:

  1. atopic dermatitis
  2. keratosis pilaris
  3. hyperlinearity
  4. thickening of palms and soles
29
Q

What is the mutation in X-linked ichtyosis? What area of the body is most affected? What areas are spared?

A

It is a mutation in steroid sulfatase.
Neck is almost always involved
Palms, soles, and face are spared

30
Q

What is the inheritance pattern and mutation in lamellar ichtyosis?
How do newborns present?
How do adults present?

A

autosomal recessive mutation in transglutaminase 1, ABCA12.

Newborns have colloidion membrane
Adults have thick, plate-like scale

31
Q

What is the inheritance pattern and mutation in epidermolytic hyperkeratosis?

A

Autosomal recessive mutation in Keratin 1 or 10
Newborns present with blisters and erosions
Later in life, erosions are replaced by hyperkeratosis

32
Q

What do you do if there is a possibility of fungal origin for a scaling disorder?

A

KOH scraping for microscopic analysis

33
Q

How does scaling of papulosquamous lesions present?

A

predominant papules/plaques with prominent scaling

34
Q

How does scaling of erythrodermic lesions present?

A

widespread erythema with fine/subtle scaling

35
Q

Differentiate acute, subacute and chronic dermatitis.

A

Acute -very inflamed/exudative [oozing, weeping, blisters]
Subacute - intermediate
Chronic- dry, scaly, lichenification