Dermatopathology (Part 4 of 4) Flashcards
What is mycosis fungoides?
a cutaneous T-cell lymphoma of CD4 T helper cells in the skin
how does mycosis fungoides present? (age, where, what do they look like)
usually >40 yo with truncal lesions; scaly red-brown patches; raised scaling plaques (can be confused with psoriasis); fungating nodules
eczema like lesions in patients with mycosis fungoides usually means what?
it is in the early stages
multiple tumor nodules in patients with mycosis fungoides usually means what?
systemic spread
what is sezary syndrome?
seen in some cases of mycosis fungoides; erythroderma, diffuse erythema and scaling of entire body surface
what is the morphology of sezary cells?
markedly folded nuclear membrane CEREBRIFORM contour
what is the histological hallmark of cutaneous t cell lymphoma?
the presence of atypical cells that characteristically form band-like aggregates within the superficial dermis and invade the epidermis as single cells and small clusters known as Pautrier microabscesses
what is urticaria pigmentosa?
a cutaneous form of mastocytosis that primarily affects children
how does urticaria pigmentosa present?
multiple, widely distributed lesions, round-oval, red-brown, nonscaling papule and small plaques; around 10% of cases have systemic disease with mast cells infiltrating organs (mostly seen in adults)
what is solitary mastocytoma?
usually seen in young kids; pink-tan-brown nodule with possible blister formation
many of the signs and symptoms of mastocytosis occurs when mast cells degranulate- what happens when mast cells degranulate?
histamine and heparin are released
what are two signs that can be seen with mastocytosis?
darier sign and dermatographism
what is darier sign?
localized area of dermal edema and erythema (wheal) when skin is rubbed
what is dermatographism?
area of dermal edema resembling a hive, result of local stroking skin with pointed instrument
what are the systemic symptoms associated with mastocytosis?
pruritus and flushing or bone pain
why is there bone pain seen in mastocytosis?
mast cell infiltration causes it
what could be a clue that a patient has mastocytosis?
if they are presenting with osteoporosis in premenopausal women or in men –> due to excessive histamine release in the bone marrow
what causes mastocytosis?
a point mutation in KIT receptor tyrosine kinase–> leads to mast cell growth and survival
what is the histological appearance of mastocytosis?
spindle shaped and stellate mast cells, fibrosis, edema, and few eosinophils
what stain can you use to diagnose mastocytosis?
giemsa (shows metachromatic granules)
what is ichthyosis?
impaired epidermal maturation, hyperkeratosis that results in a clinically fish-like scale appearance
what are three examples of acute inflammatory dermatoses?
urticaria, acute eczematous dermatitis, erythema multiforme
generally speaking, what are acute inflammatory dermatoses?
acute lesions that last from days to weeks that are characterized by inflammatory infiltrates (usually lymphocytes and macrophages) NOT NEUTROPHILS
generally speaking, what are chronic inflammatory dermatoses?
chronic lesions persist for months to years and are often associated with changes in epidermal growth or dermal fibrosis
what causes urticaria?
localized mast cell degranulation; it is an antigen induced release of vasoactive mediators from mast cells
what can urticaria cause?
wheals or angioedema
what is the common presentation of urticaria?
20-40 yo, less than 24 hours, sites exposed to pressure
how does urticaria look on a macro level?
small, pruritic papules to large edematous plaques; individual lesions may coalesce to form annular, linear, or arciform configurations
how does urticaria look on a micro level?
sparse superficial perivenular infiltrate of mononuclear cells (so around the veins)
which antibody is associated with urticaria?
IgE
what is one of the most common skin disorders?
acute eczematous dermatitis
what causes acute eczematous dermatitis?
external application of Ag or ingested food or drug
what are the 5 main causes of acute eczematous dermatitis?
allergic contact, atopic dermatitis, drug-related, photoeczematous dermatitis, primary irritant dermatitis
what is the official name of poison ivy/oak and what in it causes acute eczematous dermatitis?
Rhus toxicodendron- Urushiol
what is treatment of acute eczematous dermatitis?
it is palliative, no cure- just treat symptoms
what type of reaction is acute eczematous dermatitis?
T-cell mediated inflammatory reaction (type IV hypersensitivity)
how does acute eczematous dermatitis look on a macro level?
red, papulovesicular, oozing, and crusted (impetiginization) lesions
what do you get if acute eczematous dermatitis persists? (2 things leading to something)
you develop acanthosis and hyperkeratosis–> raised scaling plaques
what is spongiosis?
acute eczematous dermatitis, edema in the intracellular spaces, splaying them apart, particularly in the stratum spinosum
if eczematous dermatitis was set off by drugs, how might you tell?
there might be a lot more eosinophils present
what is erythema multiforme?
it is uncommon, self-limited hypersensitivity reaction to certain infections and drugs
How is erythema multiform characterized?
by keratinocyte injury mediated by CD8+ T lymphocytes
what is located in the center of the erythema multiform lesions? and the periphery?
CD8 cytotoxic T cells are in the center; CD4 T cells and Langerhans cells are in the periphery
what is the classic morphology of erythema multiforme?
targetoid lesions
what is interface dermatitis seen in erythema multiforme?
dermal edema, lymphocyte infiltration along the dermoepidermal junction, associated with dermal edema and degenerating keratinocytes
what are two important variants of erythema multiform to know about?
stevens-johnson and toxic epidermal necrolysis
what is stevens-johnson?
seen in children; extensive skin involvement plus oral mucosa, conjunctiva, urethra, genital, and perianal areas; secondary infections can lead to life-threatening sepsis
what is toxic epidermal necrolysis?
diffuse necrosis and sloughing of cutaneous and mucosal epithelia; resembles extensive burns- also life threatening
what are three examples of chronic inflammatory dermatoses?
psoriasis, seborrheic dermatitis, and lichen planus
what is psoriasis?
a chronic inflammatory dermatosis with an autoimmune basis; may also be asosciated with myopathy, and AIDS
what is the morphology of psoriasis?
pink to salmon colored plaque covered by loosely adherent silver scale
what is the Koebner phenomenon?
induce psoriatic lesions in susceptible patients by trauma, starts a self-perpetuating local inflammatory response
besides the skin what else can psoriasis affect and how?
the nails: pitting, dimpling, yellow-brown discoloration (oil slick); oncolysis (separation of the nail plate from the underlying bed)
what are the histological features of psoriasis?
marked acanthosis, regular downward elongation of rete ridges “test tubes in a rack”
what is munro microabscesses?
seen in psoriasis; when small PMN aggregates in parakeratotis stratum corneum