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Author: Dr Jenny Chung, House Officer, Auckland City Hospital, Auckland, New Zealand; Chief Editor: Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand, August 2016.
Dermatological symptoms and signs sometimes precede or accompany a gastrointestinal disease. It should be noted that the embryonic origin of the gastrointestinal tract and the skin are closely aligned.
Common cutaneous manifestations of liver cirrhosis include:
Malnutrition associated with deficiencies of fat-soluble and water-soluble vitamins often affects the skin.
Acrodermatitis enteropathica is an autosomal recessive inherited form of zinc deficiency that manifests in infancy. It presents with the clinical triad of dermatitis, alopecia and diarrhoea. Skin signs include:
Eruptive xanthomas are:
Dermatitis herpetiformis is associated with:
Skin signs of dermatitis herpetiformis include:
Aphthous ulcers are common in both forms of inflammatory bowel disease.
Erythema nodosum (EN) is the most common skin sign of Crohn disease (4-6%) or ulcerative colitis (3%).
Pyoderma gangrenosum (PG) affects 0.7% of patients with Crohn disease and 2% with ulcerative colitis.
Fissures and fistulae are common in Crohn disease (36%) and do not occur in ulcerative colitis.
Mucosal oedema affects 8–9% of patients with Crohn disease and is absent in ulcerative colitis.
Metastatic Crohn disease may cause cutaneous granulomatous plaques, nodules and ulcers.
Epidermolysis bullosa acquisita (EBA) is a rare immunobullous disorder sometimes associated with Crohn disease.
Degos disease is small vessel angiopathy, thought to be due to dysregulation of interferon-alfa and the membranolytic attack complex. There are two variants: benign atrophic papulosis and malignant atrophic papulosis.
Hereditary haemorrhagic telangiectasia (HHT) is a hereditary, autosomal dominant, bleeding disorder.
Kaposi sarcoma (KS) is a human herpesvirus 8-associated tumour of endothelial cells.
Pseudoxanthoma elasticum (PXE) is an inherited disease in which there are calcification and fragmentation of elastin fibres in the skin and internal organs.
Ehlers-Danlos syndrome (EDS) type IV is an inheritable connective tissue disease due to a defect in collagen synthesis.
Klippel-Trenaunay-Weber syndrome presents in infancy with a cutaneous capillary vascular malformation.
Bowel-associated dermatosis-arthritis syndrome (BADAS, bowel bypass syndrome) presents with recurrent and episodic flu-like symptoms, polyarthralgia and cutaneous lesions.
Gardner syndrome is a variant of familial adenomatous polyposis with numerous adenomatous polyps and mucocutaneous findings, such as:
Hereditary nonpolyposis colorectal cancer, also known as Lynch syndrome, is the most common hereditary cancer.
Peutz-Jeghers syndrome is a hamartomatous polyposis syndrome with autosomal dominant inheritance.
Cowden syndrome, or multiple hamartoma syndrome, is characterised by skin lesions and polyposis coli.
Trichilemmomas are benign hamartomas of the outer sheath of hair follicles. They are:
Other skin lesions described in Cowden syndrome include:
The Bannayan-Riley-Ruvalcaba (BRR) syndrome is a rare germline mutation with hamartomatous polyposis, macrocephaly and mental retardation. Skin manifestations include:
Juvenile polyposis syndrome is a rare autosomal dominant disorder associated with hereditary haemorrhagic telangiectasia.
Neurofibromatosis is a common, autosomal dominant, a neurodermatosis characterised by:
Cronkhite-Canada syndrome is a sporadic syndrome that affects older adults. It presents with:
Acanthosis nigricans is associated with insulin resistance.
Glucagonoma is a rare, glucagon-secreting, pancreatic, alpha cell tumour.
Plummer-Vinson syndrome is a rare triad of dysphagia, iron deficiency anaemia and oesophageal webs.
Extramammary Paget disease is an intraepithelial adenocarcinoma located in and around the anal verge, vulva, or male genitalia.
Carcinoid syndrome is the association of intestinal carcinoid with hepatic metastases.
Dermatomyositis is classified as an autoimmune disease. In older adults with dermatomyositis, 15–50% have an underlying malignancy of the gastrointestinal tract, pancreas, lung, breast, or ovaries, or non-Hodgkin lymphoma.
Paraneoplastic pemphigus (PNP) is a rare acantholytic, mucocutaneous blistering disease.
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