Skin and Systemic Disease

Question 1

What cancer syndromes affect the skin?

Answer 1

Carcinoid Syndrome

Paraneoplastic pemphigus

Erythema Gyratum Repens

Acquired hypertrichosis

Leser Trelat

Bazex Syndrome

Ectopic ACTH

Pagets Disease

Question 2

What are the skin features of carcinoid syndrome?

Answer 2

Episodic flushing, mins - hours (no sweating)

Facial telangiectasia

GI carcinoid = liver mets

Bronchial, ovarian = can occur with no liver mets

Question 3

What are the skin signs of paraneoplastic pemphigus?

Answer 3

Erosive stomatitis

Rash

—Non-hodgkins, Castlemans disease (produces the autoantibody)

Question 4

What is erythema graytum repens?

Answer 4

—Concentric erythematous lesions

—Variable sites & types of malignancy

Question 5

What is acquired hypertrichosis lanuginosa?

Answer 5

Acute onset of lanugo hairs at face and body

•Colorectal ca > lung > breast, usually advanced

Question 6

What is leser-trelat?

Answer 6

—Eruptive seborrhoeic keratoses

—GI adenocarcinomas

Question 7

What is Bazex syndrome?

Answer 7

• Hyperkeratosis of extremities
• Resembles psoriasis, non-specific fx on skin biopsy

Question 8

What cancers are associated with Bazex syndrome?

Answer 8

• SCC: bronchial, oropharyngeal, GI
• Gastric, colon, biliary adenocarcinomas

Question 9

What condition leads to generalised hyperpigmentation?

Answer 9

Tumour production of ACTH (ectopic ACTH syndrome)

Pigmentation also occurs in the mouth

•ACTH -> Inc. MSH -> release of melanin from melanocytes

Question 10

What is pagets disease?

Answer 10

–Eczematous plaque at nipple / areola

–Extension of underlying ductal adenocarcinoma

Question 11

The previous conditions until now have an underlying cancer in almost all cases

Question 12

What is acanthosis nigricans?

Answer 12

Velvety hyperpigmentation of the skin of the skin

Question 13

What is acanthosis nigricans associated with?

Answer 13

Strongly assocaited with gastric adenocarcinoma

—Concern if:

Older patient, rapid onset

—Other skin signs

—Involves lips

—Weight loss

Question 14

What is dermatomyositis?

Answer 14

It is one of three inflammatory myopathies (can include muscle weakness)

•Inflammatory myopathy + rash

–Periorbital heliotrope rash

–Gottrons papules: red maculo-papular lesions over bony prominences

–Shawl sign

–Photosensitive poikiloderma

–Scalp erythema

Question 15

What conditions may be asociated with malignancy?

Answer 15

Bullous pemphgoid

Sweets Syndrome

Pyoderma gangrenosum - this can be related to IBD or malignancy

Acquired ichthyosis

Acquired angioedema

Primary systemic amyloid

Cryoglobulinaemia - increase in the thickness of blood - blockage of small blood vesses

Question 16

What are the genetic tumour syndromes with skin features?

Answer 16

—Peutz-Jeghers: melanosis, colon hamartomas, colon ca

—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

Question 17

What are skin signs of genetic GI disease?

Answer 17

—Hereditary haemorrhagic telangiectasia - abnormal blood vessel formation that leads to epistaxis and GI haemorrhage

—Ehlers danlos (collagen) (connective tissue disroder, also presents with hypermobile joints)

—Pseudo xanthoma elasticum (fragmentation and mineralisation of elastic fibres)

—Blue rubber bleb naevus (vascular blue lesions

Question 18

What are the inflammatory / autoimmune diseases of the GI tract associated with skin signs?

Answer 18

•Malignant atrophic papulosis (degos disease) (occlusive small vessel vasculopathy)

–> GI haemorrhage

• Erythema nodosum
• Pyoderma gangrenosum

–> IBD

Question 19

What are the skin signs of diabetes?

Answer 19

• Acanthosis nigricans
• Acral erythema (reddening, swelling, numbness and desquamation of the palms of the hands and the souls of the feet)
• Carotenemia (yellow colouration of the skin and increased levels of beta-carotenes in the blood)
• Diabetic bullae (non-inflammatory blisters in the acral areas of the body - peripheral body parts: hand, feet ears, nose)
• Diabetic dermopathy - light brown scaly pathces often found on the shins of the front of the thighs
• Disseminated granuloma annulare - raised red skin lesions that form ring patterns
• Eruptive xanthomas - A condition affecting individuals with severe hyperlipemia in which groups of waxy yellow or yellowish-brown lesions appear suddenly, especially over extensors of the elbows and knees and on the back and buttocks
• Necrobiosis lipoidica - yellowish brown patch on the lower leg, the centre of the patch becomes pale and shiny, telangiectasia is also seen - the necrobiosis lipoidica is susceptibe to bleeding, ulcer formation and secondary infection
• Rubeosis - new abnormal blood vessels are found on the surface of the iris
• Scleredema - progressive thickening and hardening of the skin, usually on the areas of the upper back, neck, shoulders and face

Question 20

What are the skin signs of thyroid disorders?

Answer 20

Hyperthyroid: Telogen effluvium (thinning or shedding of hair), moist skin, and hyperhidrosis (excessive sweating)

Hypothyroidism: dry skin (keratoderma), dry nails, and hair loss with madarosis (loss of eyelashes) are hallmark cutaneous features of the disease

Question 21

What are the autoimmune conditions that present with skin changes?

Answer 21

Cutaneous lupus (acute: butterfly shaped malar rash, subacute: red-raised scaly non-scarring rash on sun exposed areas, discoid lupus is the most common chronic form of cutansous lupus) - not necessarily a systemic disease

Cutaneous vasculitis - inflamed blood vessels in the skin - IgA deposition on direct immunoflourescence, post infective, drug induced, idiopathic

Alopecia - T - lymphocyte, cytokine rejection of hair. (totalis involves loss of skull and facial hair, universalis is the loss of all hair) can be scarring

Vitiligo - segmental/generalised - destruction of melanocytes - associated with other immune conditions