Contributor Information
Authors
Carly A. Elston
Medical Student
The Commonwealth Medical College
Scranton, Pennsylvania
Disclosure: Carly A. Elston has disclosed no relevant financial relationships.
Dirk M. Elston, MD
Director
Ackerman Academy of Dermatopathology
New York, New York
Disclosure: Dirk M. Elston, MD, has disclosed no relevant financial relationships.
Nazanin Saedi, MD
Dermatology Fellow
SkinCare Physicians
Chestnut Hill, Massachusetts
Disclosure: Nazanin Saedi, MD, has disclosed no relevant financial relationships.
Contributor Information
Editor
Lars Grimm, MD, MHS
House Staff
Department of Diagnostic Radiology
Duke University Medical Center
Durham, North Carolina
Disclosure: Lars Grimm, MD, MHS, has disclosed no relevant financial relationships.
Reviewer
Rashid M. Rashid, MD
Resident Physician
Department of Dermatology
University of Texas
MD Anderson Cancer Center
Morzak Research Initiative
Houston, Texas
Disclosure: Rashid M. Rashid, MD, has disclosed no relevant financial relationships.
Suggested Reading
Suggested Reading
Lesions on the dorsal aspect of the hand, demonstrating the photodistribution of Dermatomyositis, are shown. Note the sparing of the interdigital web spaces.
Dermatologic manifestations of systemic disease have long been known and span a range from the subtle to the conspicuous. Of these, a number feature prominently on the hands.
Another example of Gottron papules is shown.
Dermatologic manifestations are only one of a set of features of dermatomyositis, a systemic disorder that also frequently affects the joints, the esophagus, the lungs, and, less commonly, the heart. Patients with cutaneous manifestations of dermatomyositis may also have an associated systemic malignancy, and age-appropriate cancer screening is recommended.
A hand with Raynaud phenomenon is shown. Notice the discoloration of the fingers.
Raynaud phenomenon manifests as recurrent vasospasm of the fingers and toes, usually in response to stress or exposure to cold. Secondary Raynaud phenomenon should be distinguished from primary Raynaud phenomenon (Raynaud disease). They are distinct disorders that share a similar name. Raynaud disease is characterized by the occurrence of the vasospasm alone, with no association with another illness. Secondary Raynaud phenomenon is a designation usually used in the context of vasospasm associated with another illness, most commonly an autoimmune disease. Image courtesy of Wikipedia Commons.
Secondary Raynaud phenomenon in a patient with systemic sclerosis is shown.
Raynaud phenomenon is a common finding in this systemic connective tissue disease. There is symmetrical acral vasospasm, with characteristic pallor, cyanosis, and suffusion. Patients with Raynaud phenomenon also often note a sense of fullness and tautness.
Sclerosis of the hands, a common presentation in progressive systemic sclerosis is shown.
Characteristics of systemic sclerosis include essential vasomotor disturbances; fibrosis; subsequent atrophy of the skin, subcutaneous tissue, muscles, and internal organs (eg, gastrointestinal tract, lungs, heart, kidney, central nervous system); and immunologic disturbances. Generally, renal and lung changes are responsible for death in patients with systemic sclerosis. Image courtesy of Wikimedia Commons.
Although not in the hand, other classic findings of systemic sclerosis include thickened and taut skin with numerous telangiectasias. Radical furrowing around the mouth is characteristic in the later stage of the disease. Patients with systemic sclerosis may be at increased risk for malignancy, including breast carcinoma; multiple myeloma; lymphoma; and cancer of the ovary, esophagus, colon, or rectum.
Osler nodes (arrow), another finding related to subacute infective endocarditis, are smallish, tender, subcutaneous nodules that range from red to purple and are found primarily in the pulp spaces of the terminal phalanges of the fingers and toes, soles of the feet, and the thenar and hypothenar eminences of the hands. Their appearance is often preceded by neuropathic pain. They last from hours to several days. The underlying mechanism is probably the circulating immunocomplexes of subacute infective endocarditis.
Thick, doughy skin with blisters and milia are characteristic of porphyria cutanea tarda, a term encompassing a group of familial (20%) and acquired (80%) disorders in which activity of the heme synthetic enzyme uroporphyrinogen decarboxylase is deficient.
Porphyria cutanea tarda is commonly associated with hepatitis C infection and may be seen in heterozygotes for the hemochromatosis gene.
The most common presenting sign of porphyria cutanea tarda is fragility of sun-exposed skin after mechanical trauma, leading to erosions and bullae, typically on hands and forearms and occasionally on face or feet. Healing of crusted erosions and blisters leaves milia, hyperpigmented patches, and hypopigmented atrophic scars. Ethanol intake, estrogen therapies, and HIV infection are among other factors that may contribute to the development of porphyria cutanea tarda.
This slide demonstrates palmar erythema in a patient with hepatitis C virus (HCV) infection.
Palmar erythema may be seen in patients with liver dysfunction, such as chronic hepatitis. Cutaneous findings relevant to HCV infection manifest in 20%-40% of patients presenting to dermatologists and in a significant percentage (15%-20%) of general patients. HCV must be considered in the differential diagnosis of these patients. Other cutaneous manifestations of HCV include mixed cryoglobulinemia, leukocytoclastic vasculitis, lichen planus, and sicca syndrome, all of which should be regarded as early markers of a potentially fatal chronic liver disease. Image courtesy of Walter Reed Army Medical Center Dermatology.
Xanthomas over joints (tuberous xanthomas) are common in type 2 hypercholesterolemia, but tendinous xanthomas are more specific for the homozygous familial hypercholesterolemia (FH). Patients with homozygous FH may have cutaneous xanthomas at birth or by early childhood. Tuberous xanthomas may also appear on elbows or knees.
Herpetic whitlow (vesicular outbreaks on the hands and the digits) in a young child is shown.
Herpetic whitlow is most commonly due to infection by herpes simplex virus (HSV)-1. It usually occurs in children who suck their thumbs. Herpetic whitlow due to HSV-2 is increasingly recognized and is probably due to digital-genital contact. Image courtesy of Wikimedia Commons.
A transverse palmar crease (simian crease) in a child with Down syndrome is shown. This finding is seen in 40%-50% of patients with Down syndrome.
Other common cutaneous findings in Down syndrome include soft and velvety skin in early childhood, dry skin in late childhood (xerosis, atopic dermatitis, palmoplantar hyperkeratosis, and seborrheic dermatitis), premature wrinkling of the skin, cutis marmorata, and acrocyanosis
Contributor Information
Authors
Carly A. Elston
Medical Student
The Commonwealth Medical College
Scranton, Pennsylvania
Disclosure: Carly A. Elston has disclosed no relevant financial relationships.
Dirk M. Elston, MD
Director
Ackerman Academy of Dermatopathology
New York, New York
Disclosure: Dirk M. Elston, MD, has disclosed no relevant financial relationships.
Nazanin Saedi, MD
Dermatology Fellow
SkinCare Physicians
Chestnut Hill, Massachusetts
Disclosure: Nazanin Saedi, MD, has disclosed no relevant financial relationships.
Contributor Information
Editor
Lars Grimm, MD, MHS
House Staff
Department of Diagnostic Radiology
Duke University Medical Center
Durham, North Carolina
Disclosure: Lars Grimm, MD, MHS, has disclosed no relevant financial relationships.
Reviewer
Rashid M. Rashid, MD
Resident Physician
Department of Dermatology
University of Texas
MD Anderson Cancer Center
Morzak Research Initiative
Houston, Texas
Disclosure: Rashid M. Rashid, MD, has disclosed no relevant financial relationships.
Suggested Reading
Suggested Reading