Author
Adjunct Faculty and Preceptor
Physician Assistant Program
University of New England
Physician Assistant
Department of Emergency Medicine
Cambridge Hospital, Cambridge Health Alliance
Cambridge, Massachusetts
Disclosure: Mark P. Brady, PA-C, has disclosed no relevant financial relationships.
Reviewer
Robert A. Schwartz, MD
Professor and Head, Dermatology
Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health
UMDNJ-New Jersey Medical School
Newark, New Jersey
Disclosure: Robert A. Schwartz, MD, has disclosed no relevant financial relationships.
The skin and gastrointestinal (GI) tract may be affected concurrently by the same conditions, which can be primarily systemic diseases involving the skin or dermatologic diseases involving the GI tract and liver simultaneously. The correct diagnosis of such conditions may rely on the clinician's ability to recognize the dermatologic presentations. This patient has necrolytic acral erythema manifesting as plaques on his foot. Necrolytic acral erythema is a rare condition strongly associated with hepatitis. Its exact etiology is unknown, but it is thought to be related to zinc dysregulation, which can occur as a result of metabolic alteration induced by hepatitis C virus.[1]
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is an autosomal dominant disorder characterized by numerous telangiectases on the skin and oral mucosa (arrow). Recurrent epistaxis is the most common presenting manifestation of this syndrome, affecting approximately 85% to 90% of patients. Telangiectases can involve the lungs, liver, brain, eyes, and gastrointestinal tract; hemorrhage can occur at any site.[1] Image courtesy of Wikipedia Commons.
Acrodermatitis enteropathica (shown) is an inherited or acquired condition characterized by pustules, bullae, and scaling in an acral and periorificial distribution with concomitant zinc deficiency. When inherited, acrodermatitis enteropathica results from a mutation in SLC39A, which encodes an intestinal zinc transporter. In infants, deficiency can follow breastfeeding if maternal breast milk contains low levels of zinc. In adults, this disease can occur after total parenteral nutrition without adequate zinc supplementation; with alcoholism, other malabsorptive states, or inflammatory bowel disease; or after bowel surgery. Most patients have diarrhea. Treatment is zinc supplementation.[1]
Acute febrile neutrophilic dermatosis (also called Sweet syndrome) is a reactive process characterized by the abrupt onset of tender, red-to-purple papules and nodules that coalesce to form plaques (shown on the lateral aspect of the hand). The plaques usually occur on the upper extremities, face, or neck and are typically accompanied by fever and peripheral neutrophilia. Vaccination or a gastrointestinal tract infection may precede the eruption. The most commonly associated diseases are Crohn disease and ulcerative colitis.[2]
An aphthous ulcer (shown) typically presents as a small, round, or ovoid ulcer inside the mouth with circumscribed margins, an erythematous halo, and a yellow or gray floor. Aphthous ulcers affect at least 20% of the population. The natural course is eventual remission. Aphthous ulcers are common in patients with Crohn disease or celiac disease; they may also be manifestations of iron, folate, or vitamin B12 deficiency.[3] Image courtesy of The National Center for Biotechnology Information.
Acanthosis nigricans (arrow) is a skin disorder characterized by hyperpigmentation and hyperkeratosis of the skin, occurring mainly in the folds of the skin in the axilla, groin, and back of the neck. Note the papillomatous appearance of the axillary skin. The etiology of acanthosis nigricans is still not clearly defined, but it seems to be most often related to insulin resistance or intra-abdominal malignancy. The malignancies associated with acanthosis nigricans include adenocarcinoma (85% of cases), of which gastric carcinoma is present in 60%.[4,5]
Dermatitis herpetiformis (shown) is a chronic, intensely pruritic blistering disease characterized by symmetric grouped vesicles, papules, and wheals on the elbows, knees, scalp, and buttocks. Biopsy reveals a characteristic neutrophilic infiltrate; direct immunofluorescence demonstrates deposition of immunoglobulin A (IgA) at the dermal-epidermal junction. Most patients have an asymptomatic gluten-sensitive enteropathy or, less commonly, thyroid disease.[1] Image courtesy of Wikipedia Commons.
Henoch-Schonlein purpura (shown) is the most common systemic vasculitis in childhood. The diagnostic criteria include palpable purpura with at least one other manifestation, such as abdominal pain, IgA deposition, arthritis or arthralgia, or renal involvement. Periumbilical and epigastric pain worsens with meals because of bowel angina. Bleeding is usually occult or, less commonly, associated with melena. Intussusception is the most common surgical complication. There are a range of endoscopic findings including gastritis, duodenitis, ulceration, and purpura.[6] Image courtesy of Wikipedia Commons.
Erythema nodosum (shown) is the most common type of panniculitis. It affects subcutaneous fat in the skin and is usually first evident as an outcropping of erythematous nodules that are highly sensitive to touch. Although most often associated with streptococcal pharyngitis, erythema nodosum may be the first sign of a systemic disease such as tuberculosis, bacterial or deep fungal infection, sarcoidosis, cancer, or inflammatory bowel disease.[7] Image courtesy of Wikipedia Commons.
Kaposi sarcoma (shown in a patient with AIDS) is a neoplasm of vascular endothelial and lymphoreticular cells that can involve the skin and numerous visceral organs. Note the characteristic purple hemorrhagic papules coalescing into an irregular plaque. GI involvement occurs in 50-80% of patients with cutaneous Kaposi sarcoma and in almost 100% of those with oral lesions. The GI tract may be involved at any level, although the most frequently affected sites are the small intestine, stomach, and esophagus. Oral lesions are most likely to affect the hard palate, followed in order of frequency by the gingiva and the tongue.[4]
Patients with Plummer-Vinson syndrome (also called Paterson-Brown-Kelly syndrome) may present with koilonychia (arrow), early loss of teeth, development of cheilosis, atrophy of the tongue, and angular stomatitis.[4,8,9] The classic triad of the condition is dysphagia, iron-deficiency anemia, and esophageal webs.
Patients with porphyria cutanea tarda often present with cutaneous fragility and blistering of the hands (shown), forearms, or face. This hepatic porphyria results from a deficiency of the enzyme uroporphyrinogen decarboxylase. The disease becomes active when acquired factors, such as iron, alcohol, hepatitis C virus, human immunodeficiency virus, estrogens (eg, oral contraceptives, prostate cancer treatment) and possibly smoking, combine to cause a deficiency of uroporphyrinogen decarboxylase in the liver. Hemochromatosis, an iron overload disorder, can also predispose individuals to porphyria cutanea tarda.[1,10]
Leukocytoclastic vasculitis (also known as hypersensitivity vasculitis or cutaneous small vessel vasculitis) is associated with circulating type II cryoglobulins and usually yields palpable purpura on the lower extremities, as shown in this patient. Leukocytoclastic vasculitis may be localized to the skin or may manifest internally, most commonly in the joints, the gastrointestinal tract, and the kidneys. Although leukocytoclastic vasculitis has many causes, it is idiopathic in up to 50% of patients. When associated, treatment of hepatitis C infection often leads to resolution of the vasculitis.[1,11] Image courtesy of Wikipedia Commons.
Lichen planus is characterized by violaceous, flat, polygonal papules, often on the flexor aspects of the wrists, trunk, medial thighs, genitalia, and oral mucosa (shown). Lichen planus may occur with primary biliary cirrhosis and hepatitis B virus immunization. Oral erosive lichen planus is the most common expression of lichen planus in patients with hepatitis C virus. Treatment includes topical and intralesional corticosteroids, topical immunomodulators, and phototherapy.[1]
Necrolytic migratory erythema (glucagonoma syndrome) is a rare disease characterized by erythematous, scaly plaques on acral, intertriginous, and periorificial areas, as shown in this patient. The condition occurs in association with an islet cell tumor of the pancreas. Associated signs include hyperglycemia, diarrhea, weight loss, and atrophic glossitis. Necrolytic migratory erythema has been associated with intestinal malabsorption disorders, hepatic cirrhosis, chronic pancreatitis, inflammatory bowel disease, and nonpancreatic malignancies; it may not always be associated with glucagonoma.[1,12]
Pyoderma gangrenosum (shown) is a neutrophilic dermatosis characterized by painful ulcers with boggy, undermined edges and a border of gray or purple pigmentation.[1,4] Note the rolled-up, edematous, and undermined border with the surrounding halo of bright-red erythema. The base of the ulcer contains a fibrinopurulent exudate. The ulcers often follow trauma (pathergy) and begin as pustules or nodules that ulcerate and extend centrifugally. All body areas may be involved, but the legs are the most common site. Pyoderma gangrenosum is found in approximately 5% of patients with ulcerative colitis and about 1% of patients with Crohn disease.
Metastatic cancer of the umbilicus (shown), also known as Sister Mary Joseph nodule, is typically associated with adult cancers of the gastrointestinal tract and ovary. Metastatic tumors of the umbilicus are more common than primary neoplasms and are often adenocarcinomas. Note the shiny, reddish, telangiectatic group of papules in the umbilicus.
More Hidden Clues
Author
Mark P. Brady, PA-C
Adjunct Faculty and Preceptor
Physician Assistant Program
University of New England
Physician Assistant
Department of Emergency Medicine
Cambridge Hospital, Cambridge Health Alliance
Cambridge, Massachusetts
Disclosure: Mark P. Brady, PA-C, has disclosed no relevant financial relationships.
Reviewer
Robert A. Schwartz, MD
Professor and Head, Dermatology
Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health
UMDNJ-New Jersey Medical School
Newark, New Jersey
Disclosure: Robert A. Schwartz, MD, has disclosed no relevant financial relationships.
