Chapter 147. Dermatologic Findings in Systemic Disease

1. What are the most common cutaneous manifestations of gastrointestinal, renal, rheumatologic, and endocrinologic disease? What are rare but important cutaneous findings which should not be missed?

2. How does systemic amyloidosis manifest in the skin?

3. What key features are necessary to recognize nutritional deficiencies?

4. What is the systemic approach to purpura?

5. When should an inpatient dermatology consultation be obtained?

This chapter will focus on the integument and its unique ability to exhibit disease that lies beneath. Though an all encompassing review of this subject is beyond the scope of this chapter, we will discuss the cutaneous manifestations of systemic disease that are commonly encountered during the daily practice of hospitalist medicine. A helpful approach to this subject is to consider whether a cutaneous lesion represents skin involvement by an internal condition, such as amyloidosis or cutaneous metastases, or a reactive process to underlying disease, such as neutrophilic dermatoses related to inflammatory bowel disease, or cutaneous vasculitis due to a drug hypersensitivity reaction. Additionally, cutaneous lesions can be characterized as specific to an underlying systemic process, or as a nonspecific manifestation that may occur in the setting of multiple disorders. Cutaneous findings may represent the sole clinical manifestation of a systemic disorder. Thus, a basic knowledge of these dermatologic conditions is essential not only for recognizing an underlying association, but also for determining when an inpatient dermatology consult may be useful in the diagnosis and management of an otherwise puzzling patient.

Gastrointestinal Hemorrhage

Disorders in which vascular lesions are found in both the skin and viscera may occasionally manifest with gastrointestinal (GI) hemorrhage. Though rare, this group of disorders should be considered when approaching a patient with a gastrointestinal bleed, particularly in the context of recurrent hemorrhage, familial history of hemorrhage, or multiple vascular lesions noted on the skin.

Telangiectasia in the setting of GI or pulmonary vascular lesions raises suspicion for hereditary hemorrhagic telangiectasia (HHT, also known as Osler-Weber-Rendu syndrome). This is a familial autosomal dominant or spontaneous disorder, characterized by cutaneous telangiectasias and visceral arteriovenous anomalies. Epistaxis in childhood is often the presenting sign, followed by the development of well defined 2–3 mm macular or papular telangiectasias on the face, lips, tongue, ears, and chest during late childhood to young adulthood (Figure 147-1). Telangiectasias represent dilations of the superficial capillary network, which typically blanch upon pressure. GI bleeding from mucosal telangiectasias occurs in about 20–40% of cases, with onset typically after the fourth decade. The stomach and duodenum are often sources of this potentially life-threatening bleeding. Additional visceral manifestations of HHT include hepatic or pulmonary arteriovenous fistulae, which may lead to shunting, dyspnea, hemoptysis, or systemic embolism and infection, and cerebral arteriovenous malformations, which may lead to intracranial hemorrhage.

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