Skin signs of gastrointestinal disease

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Chapter 37 Skin signs of gastrointestinal disease

Christina S. O’Hara, MSIII, 2LT MC , Paul M. Benson, MD, COL (Ret), MC

1. List some of the hallmark skin signs seen with diseases of the digestive tract.

• Cirrhosis: Jaundice, ascites, purpura, spider angiomas

• Peutz-Jeghers syndrome (PJS): Lip lentigo

• Inflammatory bowel disease: Pyoderma gangrenosaum on legs

• Gardner’s syndrome: Osteomas, epidermoid inclusion cysts

It is logical that many diseases of the skin also involve the oral and anal mucosa because, embryologically, the foregut (forming the oral epithelium) and the hindgut (creating the anal mucosa) share a common ectodermal component in the first few weeks of fetal development. Therefore, the skin becomes a “mirror” of underlying pathology, both obvious and occult, in the gastrointestinal (GI) system.

Fitzpatrick JE: Cutaneous manifestations of gastrointestinal disease. In McNally PR, editor: Gastroenterology secrets, ed 3, Philadelphia, 2006, Elsevier Mosby, pp 559–566.

2. What is jaundice (icterus) and when is it apparent in the skin?

On average, 250 to 350 mg of bilirubin is normally generated daily, with 70% to 80% arising from senescent red blood cells and the remainder coming from heme proteins in the bone marrow and liver. Jaundice is the overaccumulation of bilirubin and various bile pigments in the skin and other organs. It is first seen in the sclera of the eye, skin (especially the face), and hard palate. Jaundice may result from stone obstruction, hemolysis with overproduction of bilirubin, ineffective erythropoiesis, or intrinsic liver disease. Jaundice is best seen in bright daylight and may be overlooked indoors. Jaundice is not clinically apparent until serum bilirubin exceeds 2.0 to 2.5 mg/dL in the adult and 5 mg/dL in the neonate. Jaundice may be the first and sole sign of hepatic dysfunction.

Kliegman R, Nelson WE: Nelson textbook of pediatrics. ed 18. Philadelphia, 2007, Saunders Elsevier, p 3147.

3. What can a jaundice color spectrum tell me about the types of liver disease in a patient?

Yellow discoloration of the skin is caused by bilirubin, while orange shades come from xanthorubin (intrahepatic jaundice). A deep green skin color is due to marked biliverdinemia and is characteristic of obstructive jaundice, as seen with pancreatic cancer. Patients with hepatobiliary disease, especially obstructive jaundice, often have severe pruritis; constant scratching results in inflammation of the skin follow by postinflammatory hyperpigmentation. The combination of postinflammatory hyperpigmentation and bile pigments imparts a bronze color to the skin. “Bronzing” is also encountered in hemochromatosis and primary Addison’s disease.

Do not forget about exogenous plant pigments, metabolic diseases, and trematode ingestion when examining a patient who appears jaundiced. The differential diagnosis of jaundice includes carotenemia (excessive ingestion of carotenoids), lycopenia (via tomato juice), Clonorchis sinensis (travel to Asia) or Fasciola hepatica (ingesting watercress) infection, and the sallow skin of myxedema.

4. List the top ten skin findings suggestive of hepatic and biliary tract disease.

Jaundice	Purpura
Pigment changes	Loss of body hair
Spider angioma	Gynecomastia
Palmar erythema	Peripheral edema
Dilated abdominal wall veins	Non-palpable gallbladder

Hepatobiliary diseases are associated with alterations of the vasculature, including spider angiomas, palmar erythema, and cutaneous varices. Spider angiomas are classically associated with chronic liver disease, yet may also be seen in pregnancy, oral contraceptive use, and in normal persons, especially children. The vascular spider consists of a coiled central arteriole with smaller vessels radiating outward like the legs of a spider. In chronic liver disease, they are numerous and are found on the face, neck, upper chest, hands, and forearms. “Liver palms” refers to the mottled erythema and increased warmth of the palms (and sometimes the soles of the feet) in chronic liver disease. Palmar erythema also may be seen in pregnancy, lupus erythematous, pulmonary disease, and hyperthyroidism.

Portal venous hypertension due to chronic liver disease leads to the development of collateral circulation, with esophageal varices as an example. In the skin, this is observed as dilation of the abdominal wall veins (Fig. 37-1). Caput medusa refers to the dilated periumbilical veins and has been known for centuries as a marker of advanced liver disease. In men with chronic liver disease, induction of a “hyperestrogen state” (due to a decreased efficacy of estrogen breakdown in the liver) leads to gynecomastia, testicular atrophy; loss of axillary, truncal, and pubic hair; and a female pattern of pubic hair. Purpura, ecchymoses, and gingival bleeding reflect impaired hepatic production of various clotting factors, especially the vitamin K–dependent factors. Peripheral edema and ascites indicate hypoalbuminemia and/or portal venous hypertension.

5. What is the most common skin symptom associated with liver disease?

Pruritis is common and it may be severe. It is particularly intense in primary biliary cirrhosis, diseases caused by biliary tract obstruction, and cholestatic jaundice. Constant scratching may lead to excoriations, pigment disturbances, and thickening of the skin (lichenification).

6. What diseases associated with intestinal bleeding may also leave clues in the skin?

See Table 37-1.

7. What is pyoderma gangrenosum?

Pyoderma gangrenosum (PG) is a severe ulcerative condition that affects 1 in 100,000 United States citizens per year and, in over 70% of cases, affects the lower legs (Fig. 37-2). It is one of the skin lesions associated with the abdominal pain and bleeding of inflammatory bowel disease. PG originates as a small, tender pustule that breaks down to form a painful, rapidly expanding necrotic ulcer with a cyanotic, raised and undermined edge. Lesions may develop at sites of minor trauma, a phenomenon known as pathergy. The ulcers of PG may become quite large. They frequently heal with a thin, atrophic scar.

Figure 37-1. Profile view showing dilation of the umbilical vein in the epigastrium. Enlarged abdominal wall veins reflect portal venous hypertension. The patient also had esophageal varices.

Table 37-1. Conditions Associated with GI Bleeding and Skin Lesions

INFLAMMATORY CONDITIONS	VASCULAR MALFORMATIONS AND TUMORS
Ulcerative colitis Crohn’s disease Henoch-Schönlein purpura Polyarteritis nodosa	Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu) Kaposi’s sarcoma Blue rubber bleb nevus syndrome
HEREDITARY POLYPOSIS SYNDROMES	MISCELLANEOUS
Gardner’s syndrome Peutz-Jeghers syndrome Multiple hamartoma syndrome (Cowden’s syndrome)	Ehlers-Danlos syndrome Pseudoxanthoma elastica

Figure 37-2. Pyoderma gangrenosum. Large necrotic undermined ulcer of the lower leg with exposed tendon in a patient with ulcerative colitis.

(Courtesy of the Fitzsimons Army Medical Center teaching files.)

The exact cause of PG is unknown, but immune complex–mediated neutrophilic vascular reactions in the skin have been postulated. After the diagnosis of PG is made, the next step should be to look for an underlying cause. Important conditions to search for include chronic infectious hepatitis, inflammatory bowel disease (ulcerative colitis or Crohn’s disease), rheumatoid arthritis, lupus erythematosus, HIV infection, and leukemia. About 2% of patients with ulcerative colitis have PG, and the course of both illnesses maybe parallel. Some patients may have PG for several years before developing inflammatory bowel disease. In general, firstline treatment is corticosteroids and cyclosporine. Infliximab (TNF-α antibody) is the treatment of choice for PG with underlying inflammatory bowel disease or rheumatoid arthritis.

Brooklyn T, Dunnill G, Probert C: Diagnosis and treatment of pyoderma gangrenosum, BMJ 333(7560):181–184, 2006.

8. A patient presents with anemia, blood in the stool, and red macules on his lips/tongue. What diagnosis should I first consider?

The most likely cause is hereditary hemorrhagic telangiectasia (HHT), also known as Olser-Weber-Rendu syndrome. This is an autosomal dominant genetic disorder with highest prevalence in the Dutch Antilles (1:1330). Two subtypes exist: HHT-1 and HHT-2, due to ENG (9q33-34) and ALK-1 (2q13) TGFB-1 receptor mutations, respectively. Affected individuals develop linear, punctuate, and papular red lesions on the lips, face, mucous membranes, fingers (Fig. 37-3

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