Patients seek medical advice for the appearance of small cutaneous lesions that usually are neither pruritic nor painful. In some patients, history and/or review of systems will reveal signs indicative of extracutaneous involvement: abdominal pain, diarrhea, melena, nausea, blurred vision, hemiparesis, paresthesia, or any other sign indicative of an ischemic event. History can also give a clue to previous thromboembolic or obstetrical events suggestive of the antiphospholipid antibody syndrome.
Cutaneous lesions start as crops of 2–10 mm, largely asymptomatic or mildly pruritic, fleshy or rose-colored macules that soon become round, smooth, often dome-shaped firm papules (Fig. 171-2). Some lesions display central umbilication and/or necrosis (Fig. 171-3). These lesions evolve over days or weeks to porcelain-white, atrophic papules with a rim of rosy erythema and/or telangiectases (Fig. 171-4). A fully developed lesion therefore closely resembles lesions of atrophie blanche. In time, the reddish border disappears, and only a varicelliform white scar remains. Usually, the lesions are separated from each other, but they may coalesce, leading to polycyclic atrophic areas or to skin ulcerations.
Numerous typical papules of Degos disease in a young man.
Papule with central ulceration in patient with Degos disease.
Typical pathognomonic papules of Degos disease with a porcelain-white center and an erythematous raised border.
The lesions are localized on the trunk and limbs. Palms, soles, face, scalp, and genitalia are usually spared, although there are exceptions. Exclusive acral localization is suggestive of connective tissue disease. A linear distribution was reported.9 Eye involvement is possible, and the most common manifestation is an avascular patch on conjunctivae, but sclerae, episclera, retina, choroids, and optic nerves may be affected (see eFig. 171-4.1).
Ocular involvement in malignant atrophic papulosis: a typical avascular patch on the conjunctiva surrounded by injected collateral vessels.
In classic Degos disease, the number of cutaneous lesions varies from a few to more than one hundred. Cutaneous findings usually precede the systemic manifestations that may involve the gastrointestinal tract, with bowel perforation and peritonitis, and/or the central nervous system, with hemorrhagic or ischemic stroke. Rarely, cutaneous lesions occur simultaneously or after gastrointestinal or central nervous system involvement. Postmortem studies have revealed small vessel thrombotic involvement of many organs, including kidney, bladder, prostate, liver, pleura, pericardium, lung, and eyes.10 Some patients with classic Degos disease have antiphospholipid antibodies, and this raises the possibility of a relationship with a primary antiphospholipid syndrome.
A benign form of Degos is now widely recognized. In this form, only skin involvement is found, and most of the familial cases are benign.4 Development during pregnancy has been reported in a patient with antiphospholipid antibodies,11 as well as a case occurring in a patient with acquired immunodeficiency syndrome.12 A patient developing Degos disease after interferon injections, a drug known to induce atrophie blanche-like lesions13 and microangiopathy,14 is known to the authors (L. Thomas, personal communication). There is no clear-cut distinction between the classic and the benign form of Degos disease, and there is no way by which one can predict which patients will or will not develop visceral involvement.
Degos Disease-Like Lesions or Secondary Degos Disease
Degos disease-like lesions can occur in patients with known lupus erythematosus, especially those with antiphospholipid antibodies, dermatomyositis, systemic sclerosis, granulomatosis with polyangiitis (Wegener's), Crohn's disease and during parvovirus B19 viremia, a virus with a known affinity for endothelial cells.15,16–18