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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
Pautrier microabscess
Mast cells
Normal epidermis
MASTOCYTOSIS
o
o
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
Nonspecific
Dermatographism
o
o
o
o
SKIN DISEASES
o
o
o
o
o
IgE-dependent: Ag exposure
Papules to plaques
ICHTHYOSIS
Papules
Wheal
HISTOLOGY
o
o
o
o
o
o
o
o
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
o
o
o
o
Target lesions
VARIANTS:
STEVENS JOHNSON SYNDROME
o Erosions, hemorrhagic crusts
o Confined to lips and oral mucosa
o Secondary bacterial infections: sepsis DANGER!
Plaque stage
lichenification
Dermal edema
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