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Dermatopathology: Practical & Conceptual October - December 2001
Hypothesis: Degos’ Disease Is a Distinctive Pattern, Chiefly of Lupus Erythematosus, And Not a Specific Disease
Elízabeth Ball, M.D.
Amy Newburger, M.D.
A. Bernard Ackerman, M.D.
The Patient Who Sparked Our Interest
Histopathologic Findings In Chronologic Sequence
Course and Prognosis
Cause and Mechanism
The Association of Degos’ Disease and “Collagen Vascular Disorders”
Our Concept of Degos’ Disease
The Association of Degos' Disease and "Collagen Vascular Disorders"
Until 2000, 16 patients with typical lesions of Degos' disease seemed to have other diseases concurrent with it, especially collagen vascular disorders. The first report about that phenomenon was by Durie who, in 1969, recorded the occurrence in a 34-year-old man of both Degos' disease and progressive systemic sclerosis.
The patient presented himself with numerous skin lesions typical of Degos' disease and, simultaneously, developed diffuse swelling and stiffness of his hands and feet, ulceration on the tip of several fingers, Raynaud's phenomenon, and difficulty opening his mouth. Sections of tissue of a biopsy specimen from skin of a finger showed characteristic changes of scleroderma. Sections of tissue of a biopsy specimen of a papule showed findings of Degos' disease. Months later the patient died of renal and heart failure believed to be a manifestation of fulminant progressive systemic sclerosis, not of Degos' disease. The authors were aware that the nature of the vascular changes in both diseases is similar, namely, intimal proliferation in small vessels that may progress to obstruction of a lumen in conjunction with a perivascular lymphocytic infiltrate of variable density. Autopsy of kidneys in Degos' disease and in progressive systemic sclerosis may reveal multiple subcortical infarcts.
Three reports in the literature concern patients with cutaneous lesions like those of Degos' disease who subsequently developed lupus erythematosus.
In 1974, Dubin
told of a 29-year-old man who had skin lesions that resembled those of Degos' disease, clinically and histopathologically. The patient went on to develop disease of the central nervous system. Biopsy of the skin lesions produced a specimen the sections of which exhibited fibrosis of the dermis, atrophy of the epidermis, and a slight "chronic vasculitis" within a zone of dermal fibrosis. LE preparations were positive. Both affected and normal skin showed immunofluorescent findings at the dermal-epidermal junction consonant with lupus erythematosus. The authors concluded their article as follows:
"our case illustrates that there can be nothing specific or pathognomonic about cutaneous infarcts associated with endovasculitis or other changes in the walls of blood vessels. Depressed, atrophic lesions with porcelain-like centers can be secondary to microscopic infarcts associated with vasculitis and obliterative vascular changes, which can in turn be associated with several diseases, including lupus erythematosus and other collagen diseases. Malignant atrophic papulosis appears to be a specific entity with multisystemic involvement but this must be an exclusion diagnosis after other conditions known to manifest similar vascular changes, especially the collagen diseases, are ruled out."
In 1976, Black and Hudson offered their opinions about two patients with systemic lupus erythematosus, who, in the course of the disease, developed atrophic papules that looked just like those of Degos' disease. The first patient, a 39-year-old woman, developed erythematous infiltrated lesions typical of discoid lupus erythematosus on her cheeks, nose, forehead, and dorsa of hands. Sections from a biopsy specimen taken from one of the lesions showed findings diagnostic of lupus erythematosus. Later, those lesions, and others on the trunk, acquired a porcelain-white center and enveloping telangiectasias similar to lesions of Degos' disease. Sections from a specimen of an early papule showed abundant mucin in the center of the lesion. In an older lesion, the epidermis was atrophic and beneath it were hyalinization and relative acellularity of collagen in the upper part of the dermis. At the edge of the papule was a moderately dense perivascular infiltrate of lymphocytes. Direct immunofluorescence demonstrated the presence of granular deposits of IgM, IgG, and C3 in the basement membrane zone. One year later, painful lesions like those of atrophie blanche appeared on her heels, toes, and fingers. Those lesions later resolved, but many lesions like those of Degos' disease continued to appear in the skin of her trunk and limbs.
The second patient, a 37-year-old woman, presented herself with typical lesions of discoid lupus erythematosus and livedo reticularis over the arms, some lesions sporting a white atrophic zone reminiscent of lesions of Degos' disease. Sections from a specimen of a skin biopsy of one of these lesions displayed findings diagnostic of lupus erythematosus. Direct immunofluorescence showed thready BMZ deposits of IgM and IgG. These changes indicate how similar are the lesions in the skin of Degos' disease and lupus erythematosus. Black and Hudson agreed with the conclusion of Dubin
that diagnosis of Degos' disease should be made only after collagen diseases, in particular lupus erythematosus, have been excluded from consideration.
in 1987, recounted the history of a 25-year-old woman who had skin lesions of Degos' disease, confirmed by assessment histopathologically and by immunological anomalies consonant with systemic lupus erythematosus. The latter disease became obvious clinically three years after the appearance of signs of Degos' disease and during her first pregnancy, at which time she developed extra-membranous glomerulonephritis.
in 1984, commented on a patient with Degos' disease, high titers of anticardiolipin antibodies, and high activity of lupus anticoagulant. The latter two aberrations are known to be markers of a tendency to thrombosis in systemic lupus erythematosus and in other diseases.
In this patient, antinuclear antibody was negative and there was a false positive VDRL.
Other authors have noted the presence of lupus anticoagulant and/or anticardiolipin antibodies in patients with Degos's disease.
studied, prospectively, 33 patients with systemic lupus erythematosus who had lupus anticoagulant and in whom a variety of skin manifestations developed. The skin changes differed from those seen in a control group of 37 patients with systemic lupus erythematosus but without lupus anticoagulant. The feature most characteristic was the presence of vascular lesions, especially necrotic ulcers, in two thirds of the patients. Clinically, four different types of ulcers could be distinguished, to wit, small painful ulcers of livedoid vasculitis on the leg, pyoderma gangrenosum-like ulcers, pseudosarcomatous Kaposi-like lesions, and small painful ulcers on the trunk and palms that left as residua porcelain white scars similar to those in Degos' disease. The latter lesions occurred in three of 33 patients and showed typical histopathologic findings of Degos' disease.
told of their experience with a patient who had 10 erythematous papules, 38 mm in size, on the trunk and extremities. The lesions developed into atrophic porcelain-white scars surrounded by an erythematous, telangiectatic border. Examination by conventional microscopy of tissue sections from such a lesion showed histopathologic findings typical of Degos' disease. Later, the patient developed lesions of leukocytoclastic vasculitis on his legs. Colonoscopy was performed and revealed numerous aphthous ulcers like those of Crohns' disease.
in 1997, published their observations about a 29-year-old woman with dermatomyositis who developed approximately 30 atrophic porcelain-white papules rimmed by a zone of erythema. Sections of tissue from a biopsy specimen of one of these lesions showed a slight interface dermatitis with epidermal atrophy, "incontinence" of melanin, abundant dermal mucin, telangiectasias in foci, and fibrin within small to medium-sized vessels in the reticular dermis. There was no vasculitis. Titers of ANA (speckled and cytoplasmic) were high. Anticardiolipin antibodies, lupus anticoagulant, cryoglobulin, and cryofibrinogen were negative.
Skin lesions in another patient with dermatomyositis and "Degos' disease like lesions" are shown in
Figs. 5A and 5B
Figs. 5A, B Skin lesions like those of Degos' disease in a patient with dermatomyositis. Similar lesions may be observed in patients with lupus erythematosus and other collagen vascular diseases. According to Degos, lesions of malignant atrophic papulosis could "form irregular patches, linear, whose size never exceeded 10 to 3 mm." (From Braverman IM.
Skin Signs of Systemic Disease.
Philadelphia: WB Saunders Company; 1998: 40912. With permission).
In the same year, 1997, Demitsu
published their thoughts in regard to a 53-year-old Japanese woman who had approximately 250 skin lesions of Degos' disease and evidence of rheumatoid arthritis. Sections of tissue from a biopsy specimen of one of these lesions showed slight "liquefaction degeneration at the dermo-epidermal junction," edema in the upper part of the dermis in the center of the lesion, a perivascular infiltrate of lymphocytes at the periphery of the lesion, marked swelling of endothelial cells, and thrombi in the lumen of small vessels.
A patient with acquired immunodeficiency syndrome and lesions of Degos' disease, but without visceral involvement, and who responded to therapy with dipyridamol was reported on recently by Requena
wrote of a woman with cutaneous lesions of Degos' disease, without visceral involvement, but with a family history of Degos' disease. Lesions worsened after immunosuppresion for renal transplantation. As the dose of her immunosuppressive therapy was reduced, low-dose aspirin was added to the regimen and the frequency with which she seemed to develop new lesions decreased.
The findings in these two patients are contradictory, some of them indicative that immunosuppression leads to development of more skin lesions and others of them suggestive that it protects against development of lesions in internal organs. Reports regarding treatment of Degos' disease with immunosuppressant agents convey no evidence of benefit and even possible worsening of the disease.
followed a patient with typical lesions of Degos' disease in the skin, the intestinal tract, and the central nervous system. Sections of tissue of one of the skin lesions showed findings indistinguishable from those of lupus erythematosus, including sclerosing panniculitis identical to that of lupus profundus.
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