Flashcards in Skin and systemic disease Deck (77)
What is Curth's postulate?
Curth's postulates are criteria used to identify a consistent relationship between an internal malignancy and a cutaneous disorder:
- concurrent onset
- parallel course
- uniform site or type of neoplasm
- statistically significant association
- genetic linkage
What dermatological conditions are often seen in cancer patients?
• Carcinoid Syndrome
• Paraneoplastic pemphigus
• Erythema Gyratum Repens
• Acquired hypertrichosis
• Leser Trelat
• Bazex Syndrome
• Ectopic ACTH syndrome
• Pagets Disease: normal and extra-mammary
Describe carcinoid syndrome and how it presents dermatologically
Carcinoid syndrome is a paraneoplastic syndrome comprising the signs and symptoms that occur secondary to carcinoid tumors. The syndrome includes flushing and diarrhea, and less frequently, heart failure, emesis and bronchoconstriction. It is caused by endogenous secretion of mainly serotonin and kallikrein.
– Episodic flushing, mins –hours, no trigger
– Facial telangiectasia (spider veins)
– Usually caused by GI carcinoid, often with liver mets
– If from bronchial or ovarian tumour, no liver mets
Describe paraneoplastic pemphigus and how it presents dermatologically
Paraneoplastic pemphigus (=blistering skin condition) is an autoimmune disorder stemming from an underlying tumor. It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes.
o Erosive stomatitis (ulcers/erosions of oral mucosa and lips)
o Non-hodgkins, Castlemans disease (produces the autoantibody)
Describe Erythema gyratum repens and how it presents dermatologically
Erythema gyratum repens (also known as "Gammel's disease") is a figurate erythema that is rapidly moving and usually a marker of underlying cancer, usually from the lung. Very rarely seen
o Concentric erythematous lesions
o Variable sites & types of malignancy
What is the other name for Gammels disease?
Erythema gyratum repens
Describe acquired hypertrichosis lanuginosa, what conditions its linked to and how it presents dermatological
Acquired hypertrichosis lanugo-type or hypertrichosis lanuginosa acquisita (HLA) is often associated with metabolic and endocrine disorders and use of certain drugs. Colloquially known as “Werewolf syndrome”
• Acute onset of lanugo hairs at face and body
• Lanugo hairs are the ones found on newborns, rare in adults
• Colorectal ca > lung > breast, usually advanced
• Also often seen in those with eating disorders
Describe Leser-Trélat, what conditions its linked to and how it presents dermatological
The Leser-Trélat sign is the explosive onset of multiple seborrheic keratoses (many pigmented skin lesions), often with an inflammatory base. This can be an ominous sign of internal malignancy as part of a paraneoplastic syndrome. In addition to the development of new lesions, preexisting ones frequently increase in size and become symptomatic
Describe Bazex syndrome, what conditions its linked to and how it presents dermatological
o Hyperkeratosis of extremities e.g. hands, feet, ears, nose
o Resembles psoriasis, non-specific fx on skin biopsy
o Gastric, colon, biliary adenocarcinomas, bronchial, oropharyngeal
Describe ectopic ACTH syndrome, and how it presents dermatologically
• Tumour production of ACTH leads to generalised hyperpigmentation
• ACTH -> Inc. MSH -> release of melanin from melanocytes
• Tanned skin and hyperpigmention of buccal mucosa
Describe classic Paget's and extra-mammary Paget's, and how it presents dermatologically
– Eczematous plaque at nipple/areola
– Affects 1-4% of women with breast cancer
– Extension of underlying ductal adenocarcinoma
– Often a primary intraepithelial adenocarcinoma
– Occasionally secondary to underlying visceral tumour: bladder, colon (why its seen near the anus and genitalia)
What are 2 conditions strongly associated with cancer?
• Acanthosis Nigricans
Describe Acanthosis Nigricans, and how it presents dermatologically
Acanthosis nigricans is a skin condition characterized by areas of dark, velvety discoloration in body folds and creases. The affected skin can become thickened. Most often, acanthosis nigricans affects your armpits, groin and neck.
Linked to cancer and diabetes
What skin conditions presents as a dark velvety appearance of skin in body folds and creases?
When should you be concerned about Acanthosis nigricans?
Can preceed malignancy (gastric adenocarcinoma). ?related to by-products. Concern if:
o Older patient, rapid onset
o Other skin signs
o Involves lips
o Weight loss
Describe Dermatomyositis, and how it presents dermatologically
Inflammatory myopathy + rash
– Periorbital heliotrope rash
– Gottrons papules: red maculo-papular lesions over bony prominences e.g. over knuckles
– Shawl sign – seen on chest and across the back
– Photosensitive poikiloderma
– Scalp erythema
What are Gottrons papules?
Gottrons papules: red maculo-papular lesions over bony prominences e.g. over knuckles
Seen in Dermatomyositis
What condition presents dermatologically with periorbital heliotrope rash, gottrons papules, shawl sign rash, photosensitive poikiloderma and scalp erythema, as well as symptoms of weak/painful muscles?
Describe bullous pemphigoid, what conditions its linked to and how it presents dermatologically
AI condition causing annular blister rash
May be associated with cancer
Describe Sweets syndrome, what conditions its linked to and how it presents dermatologically
acute febrile neutrophilic dermatosis, characterized by the sudden onset of fever, an elevated WBC, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination, often after infections
Linked to cancer
Describe pyoderma gangrenosum, what conditions its linked to and how it presents dermatologically
Pyoderma gangrenosum - condition that causes tissue to become necrotic, causing deep ulcers that usually occur on the legs (shins). When they occur, they can lead to chronic wounds. Ulcers usually initially look like small bug bites or papules, and they progress to larger ulcers.
Linked to IBD and some malignancies
Describe acquired ichthyosis, what conditions its linked to and how it presents dermatologically
Acquired ichthyosis - non-hereditary condition, dry and rough skin with scaling (“fish scale” skin), not inherited, linked to deficiencies/tumour
Linked to cancer
Describe acquired angioedema, what conditions its linked to and how it presents dermatologically
Acquired angioedema – AI/idiopathic disease causing swelling of face and lips not caused by allergy. Can be linked to tumours
Linked to cancer
Describe primary systemic amyloidosis, what conditions its linked to and how it presents dermatologically
Primary systemic amyloidosis – build up of amyloid deposits
Linked to cancer
What are some genetic tumour syndromes which can present dermatologically?
Peutz-Jeghers: melanosis, colon hamartomas
Muir-Torre: sebaceous tumours, keratoacanthomas, GI, breast, GU
Neurofibromatosis: Café au lait, Ax freckles, Neurofibromas, nerve sheath, phaeoCCyT, carcinoid
MEN syndromes: angiofibromas, neuromas, lichen or macular amyloid (MEN2), endocrine, P’s
Xeroderma pigmentosum: inability to repair sun damage, sarcoma, leukaemia, GI, lung
Howel-Evans: palmoplantar keratoderma, oesophageal
What is Peutz-Jeghers syndrome?
Peutz–Jeghers syndrome (often abbreviated PJS) is an autosomal dominant genetic disorder characterized by the development of:
o Benign hamartomatous polyps in the gastrointestinal tract - big lumps on GI mucosa
o Hyperpigmented macules on the lips and oral mucosa (melanosis) - small brown spots on lips and mouth
What genetic cancer syndrome can be recognised by benign polyps on the GI mucosa and hyperpigmented macule on the lips and oral mucosa?
How is Peutz–Jeghers syndrome inherited?
Describe Muir-torre syndrome
Muir–Torre syndrome (MTS) is a rare hereditary, autosomal dominant cancer syndrome that is thought to be a subtype of HNPCC. Individuals are prone to develop cancers of the colon, genitourinary tract, and skin lesions, such as:
o Keratoacanthomas - common low-grade skin tumour that is believed to originate from the neck of the hair follicle
o Sebaceous tumors – malignant tumour that arises from sebaceous glands