Paraneoplastic syndromes of the skin Flashcards
Carcinoma erysipelatodes
Erythematous or indurated skin lesion resembling erysipelas, caused by various metastatic cancers
- Inflammatory breast cancer, from blocked lymphatics.
- Mesothelioma causes carcinoma erysipelatodes on chest wall
- Ovarian cancer also
En cuirasse carcinoma
Morphea-like (scleroderma) very hard fibrotic induration of the skin over areas of metastatic nodules.
Usually occuring around breast cancer
cuirasse, like the armor.
Telangiectic metastatic carcinoma
Breast cancer appearing as pin point telangiectasias INSIDE of a lesion of carcinoma erysipelatodes
May also contain violaceous papules or vesicles.
Paget’s disease of the breast
Sharply demarcated patch or plaque of scaling erethema.
Occurs on the nipple or areola and is the contiguous spread of underlying intraductal carcinoma of the breast.
Can occur bilaterally, although it is usually unilateral.
Will NOT respond to glucocorticoids, as Eczematous dermatitis of the nipples WILL.
Simulates chronic dermatitis
Can be asymptomatic or painful and pruritic.
Cancers that can give metastases to nodules on the scalp
What does this cause
Prostate adenocarcinoma
Lung cancer
Breast cancer
Cause Alopecia Neoplastica.
Well demarcated red-pink smooth areas of hair loss.
Extramammary paget disease
Intraepidermal extension of a primary adenocarcinoma of the colon or rectum. Or can be
Resembling the scaly erythema or plaque lesion seen on the niple, but on the anogenital region.
Is treated by excision and can occur on its own witout any other underlying neoplasm though this is uncommon.
Recurrence rates are high even after excision.
Cowden syndrome
Multiple hamartoma syndrome
Autosomal dominant heritable cancer syndrome, from Germ line PTEN mutation (tumor suppressor gene)
Childhood.
skin colored pink/brown papules looking like flat warts on the face, lips, ears.
translucent punctate keratosis; of the palms and soles
Very numerous papules and papillomas on the gingiva, labial mucous membranes, tongue, and palate. ‘cobblestone’ moth.
Patrients need to be daignosed early so that they can be screened regularly to detect thyroid and breast cancer which they get early
In addition to these malignant tumors they get lots of benign hamartomas throughout life
hamartoma is a mostly benign, focal malformation that resembles a neoplasm in the tissue of its origin but in a disorganized fasion.
Peutz-Jeghers syndrome
Autosomeal dominant disorder.
Polyposis of pigmented brown macules on the mucous membranes, nose, palms and soles.
The macules on the face and hands dissapear with time, but the brown macules in the oral mucosa remain forever, which are the main sign.
Mapped to a mutation of 19p13.3 gene.
Also hamartomatous polyps grow in the bowels and stomach, causing GI pain, bleeding, and anemia.
Glucagonoma syndrome
From a glucagonoma, in the skin it causes
Migratory Necrolytic Erythema,
Erosions that appear, crust, and heal with hyperpigmentation.
Inflammatory patches and plaques.
Lesions are located:
Perioral and perigenital regions.
Around flexures, and intertriginal areas.
Fingertips.
Hyperglycemia and elevated glucagon levels in blood.
Severe malabsorption
Hypoaminoacidemia
Erosive and erythematous fingertips
Malignant Acanthosis Nigricans
Acanthosis nigricans: Symmetric, hyperpigmented thickining of the skin, usually in the axilae or on the neck, or in other body folds.
Skin looks thick and dirty.
Caused by tumor cells producing TGF-alpha, inducing keratinocyte proliferation.
Usually in fatties with insulin resistance - armpit hyperpigmentation on diabetics
and
As paraneoplastic sydrome in patients with gastric adenocarcinoma
Malignant AN:
Is more pronounced hyperpigmentation and hyperkeratosis
- Has pronounced mucosal involvement of the mucocutaneous junction.
- Has ‘Tripe hands’ of AN on the hands. This almost always indicates an underlying malignancy.
- Weight loss from malignancy.
Malignant AN can preceed overt carcinoma by up to 5 years, and AN should always indicate for a thorough search for cancer.
Paraneoplastic Pemphigus
Severe mucous membrane involvment.
Rash is a combination of features from Pemphigus Vulgaris and Erythema Multiforme.
Both in gross appearance and histologically.
Severe ORAL and CONJUNCTIVAL erosions, in patient with underlying neoplasia.
The oral lesions are extremely painful and can severely limit food intake.
Non-hodgkin lymphomas, CLL, Castleman disease, THymoma, Sarcoma, Waldenstrom macrogloulinemia.
Usually also have autoantibodies of myasthenia gravis and autoimmune cytopenia.
PNP autoantibodies are against desmosomes in the intercellular lesion. Desmoplakin.
Can sometimes have aa’s against hemidesmosome proteins.
Bazex syndrome, NOT a paraneoplastic syndrome
a rare X-linked dominant inherited disorder of the hair follicle characterised by:
Follicular atrophoderma (breakdown of follicles on the skin) of the extremities.
Multiple basal cell carcinomas of the face
Milia- A milium is a small cyst containing keratin (skin protein); they are usually multiple and are then known as milia. These harmless cysts present as tiny pearly-white bumps just under the surface of the skin.
Localised or generalised hypohidrosis (diminished sweating)
Hypotrichosis (reduced body and scalp hair).
Acrokeratosis neoplastica, also called Bazex syndrome
Hand and fingernails very similar to psoriasis.
It is associated with squamous cell carcinoma of the upper respiratory or gastrointestinal tract.
It is far more common in males than females.
Erythema Gyratum Repens
aka Gammels syndrome.
rapidly growing, erythematous annular lesions.
Concentric, annular rings form a wood-grain pattern.
Zebra-skin-like scaling of the plaques on the torso and extremities
Caused usually by bronchial carcinoma, but also by breast and many GI and hematologic cancers.
Hypertrichosis lanuginosa acquisita
Lanugo hairs are the fine haris found on neonates.
Lanuginosa is the rapid growthy of many long, fine, lanugo hairs.
Usually on the face, around the eyes, ears, and nose.
Usually from a lug, breast, or uterine cancer. Usually occurs at a late stage of cancer.
Erythema necrolyticum migrans
Migrating, annulary, erythematous macules and papules,
perioral and periorbital and perianal erosions
Stomatitis and glossitis
Nail dystrophy
AKA glucagonoma syndrome.
Sweet’s syndrome
aka Acute febrile neutrophilic dermatosis
Painful, erythematous papules, plaques, and nodules
Fever
Neutrophilia
AML, lymphomas, breast, gneital tumors
Pyoderma gangerenosum
Presents as a rapidly enlarging, very painful ulcer, with a livid/purpural border.
It is a neutrophilic dermatosis, with marked neutrophil infiltration into the dermis and epidermal necrosis.
The name pyoderma gangrenosum is historical. The condition is not an infection (pyoderma), nor does it cause gangrene
Caused by AML, multiple myeloma
Leser-Trelat sign
Explosive onset of large numbers of seborrheic keratoses. (large number of pigmented plaques
Caused by tum or TNF-alpha production. 40% caused by neoplasia
Often seen in conjunction with Acanthosis nigricans
Adenocarcinomas of the GI, breast, bronchi
lymphocytic tumors.
Acquired ichthyosis
“Fish scale” skin. severe cornification. Very dry, thickened, skin with extensive, deep fissures.
Polygonal extensive hyperkeratosis, with very thin or missing st. granulosum.
Occurs with Hodkins lymphoma. 70-80% of cases.
Cutanous amyloidosis
From myeloma multiplex
soft, shiny, non-pruritic plaques, nodules, and/or alopecia.
Scleromyxedema
IgG monoclonal gammopathies, Multiple myeloma, NHLs and Hodgkin Lymphomas.
large, shiny, waxy, lichenoid papuiles.
