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SURGICAL PATHOLOGY: DISEASES OF THE SKIN

Dr. Christine Ayochok | August 19, 2015

[5] TUMORS OF CELLULAR MIGRANTS TO THE SKIN


Lymphocytes and Mast cells
T-cells

MYCOSIS FUNGOIDES/CUTANEOUS T-CELL LYMPHOMA


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Primary T-cell lymphoma of the skin


Tumor cells: clonal populations of CD4 subset (T helper)
-progresses through 3 stages
Marker: CLA (common lymphocytes antigen: CD45)
Specific abnormalities
1. patch

Aberrant cell surface Ags

Clonal T-cell receptor rearrangement


2. plaque
Usually localized to the skin
3. nodular- full blown stage
Occasionally: systemic involvement
Prognostic factors
*Sezary = erythroderma

% of body surface involved

Degree of progression from patch to plaque to nodular forms


Some patients: seeding of blood by tumor cells, with erythema and scaling (diffused)

Sezary syndrome: disseminated mycosis fungoides

SEZARY-LUTZNER CELLS
o Hallmark of CTCL
o CD4+ T-cells in band-like aggregates
o Superficial dermis
o Invade epidermis singly or in clusters (Pautrier microabscesses): initially

Misnomer: since the microabscess is not made of neutrophils but tumor cells
o Advanced disease: into the dermis

First: Patch stage


Probably patch stage

Second: Plaque stage


Third: Nodular/ Tumor
stage
Fourth: Seeding into the
blood, erythematous
scales [Sezary syndrome]

Start of microabscess formation

Plaque stage (stage 2)

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Pautrier microabscess

SURGICAL PATHOLOGY: DISEASES OF THE SKIN


Dr. Christine Ayochok | August 19, 2015

Mast cells

Normal epidermis

MASTOCYTOSIS
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Increase # of mast cells in skin


Acquired activating point mutations of tyrosine
kinase (c-Kit receptor)

Promotes mast cell growth, survival


Solitary mastocytosis

Urticaria pigmentosa
o Localized form
o Typically in children
o Usually multiple lesions: papules, plaques
o Maybe single (solitary mastocytoma): nodules 1 to few nodular skin lesons
o Occasionally: systemic disease - liver, spleen, BM, LN

Bone marrow:
systemic mastocytosis

Urticaria pigmentosa
presenting as multiple
plaques

Symptoms

Related to effects of substances


released from degranulation of mast
cells

Granules: histamine, heparin

Darier sign: edema and erythema after skin


lesion is rubbed

Nonspecific

Dermatographism

Dermal edema in normal skin after


stroking with a pointed instrument

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Increase # of mast cells in the dermis


Use of special stains: toluidine blue,
Giemsa
Mast cell tryptase: helpful when mast
cells are already degranulated
Epidermis is normal

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cytoplasm- has granules


nucleus-eccentric

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Upper Left: Special stain with toluidine blue


Arrows pointing to granules
Upper right: BM toluidine blue (systemic mastocytosis)
Lower left: Liver toluidine blue (systemic mastocytosis)

SURGICAL PATHOLOGY: DISEASES OF THE SKIN


Dr. Christine Ayochok | August 19, 2015

[6] ACUTE INFLAMMATORY DERMATOSES

SKIN DISEASES
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o Last from days to weeks


choles sulfate

Vs. chronic: months to years


prevent sloughing off of
Chronic, excessive keratin buildup (hyperkeratosis): fish-like scales keratinocytes
o Lymphocytes and macrophages
proliferating cells, not neutrophils

Some cases: due to defective desquamation


o Edema
X-linked form: deficiency of steroid sulfatase - accumulation of cholesterol sulfate

Promote compaction and aggregation of S. corneum cells


URTICARIA
Autosomal dominant or recessive
o Localized mast cell degranulation leading to dermal microvascular hyperpermeability
Compacted, thickened stratum corneum (hyperkeratosis) mostly genetic,
(edema)
may be acquired
Loss of normal basket-weave pattern
o Forms
increased permeability of blood vessels

IgE-dependent: Ag exposure

IgE-dependent: drugs, chemical *IgE-independent

Complement-mediated: Hereditary angioneurotic edema (HAE): C1 deficiency


o Basic lesions: wheals

Papules to plaques

ICHTHYOSIS

Papules

Wheal

Can be compounded by bacterial infection

HISTOLOGY
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Widely spaced collagen bundles due to dermal edema


Dilated lymphatic channels: lymphangiectasia
Epidermis is normal

SURGICAL PATHOLOGY: DISEASES OF THE SKIN


Dr. Christine Ayochok | August 19, 20154

ACUTE ECZEMATOUS DERMATITIS


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wet inflammatory skin lesion

Eczema: blistering disease


Papulovesicular, oozing, red, crusted lesions
Progress to scaling plaques due to acanthosis and hyperkeratosis (chronic)
Categories based on initiating factors

Allergic contact dermatitis

Atopic dermatitis -high IgE

Drug-related

Photoeczematous
light

Primary irritant
ex. radioactive dyes
Ag taken by Langerhans cells
Presented to CD4+ T-cells: effector and memory T cells
Re-exposure to Ag: memory T-cells go to site - release cytokines and chemokines
Hallmark: spongiosis - edema in the epidermis

Spongiotic dermatitis
When edema is severe may lead to intraepidermal vesicles

ERYTHEMA MULTIFORME
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noted fungal infections:


HSR to infections or drugs
cystoplasmosis
Associated with:
coccidiomycosis

Infections: herpes, typhoid, leprosy

Drugs: PCN, sulfonamides

Malignancy: carcinoma, lymphoma

Collagen vascular disease: lupus and polyarteritis


nodosa (PAN)
Immune-mediated epidermal cell injury

Cell death by CD8+ T-cells (central part of lesion) and


CD4+ T-cells and Langerhans cells at the erythematous
periphery
Multiform lesions: macules, papules, bullae, vesicles
Target lesion: red macule/papule with pale or eroded
center

Target lesions

VARIANTS:
STEVENS JOHNSON SYNDROME
o Erosions, hemorrhagic crusts
o Confined to lips and oral mucosa
o Secondary bacterial infections: sepsis DANGER!

Oozing stage (multiple blisters)

Erythematous blistering lesions

Plaque stage

lichenification

TOXIC EPIDERMAL NECROLYSIS (TEN)


burnt-like
o Diffuse necrosis
o Sloughing of cutaneous/mucosal epithelial surfaces
o Hallmark: interface dermatitis

Dermal edema

Lymphocytes at the DEJ

Dying/dead keratinocytes
o Progression: lymphocytes go up to epidermis
o Blister formation in the epidermis due to necrosis
o With necrosis of keratinocytes: formation of intraepidermal vesicles
Left:
Stevens Johnson Syndrome
Right: TEN
DDx: Burn

Green: Inflammatory response


Blue: spongiosis
Red: Dean keratinocyte
Green: Inflammatory infiltrate
Blue: DEJ

Arrows: intraepidermal vesicles

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Formation of intraepidermal vesicles


because of necrosis