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Background
This rare disease is important because a skin biopsy may be the first clue to the diagnosis. Patients classically develop multiple skin papules that evolve to have a dimpled appearance with a porcelain white center. This is the clue to the underlying disease which can affect the gastrointestinal tract and central nervous system and may lead to death. The skin biopsy places the pathologist at the center of the diagnostic evaluation.
OUTLINE
DISEASE ASSOCIATIONS CHARACTERIZATION PROGRESSIVE SYSTEMIC SCLEROSIS
- Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis.
Liu CM, Harris RM, Hansen CD.
Department of Dermatology, University of Utah, Salt Lake City, UT 84101, USA.
Cutis. 2005 Feb;75(2):101-4. Abstract quote
Malignant atrophic papulosis (MAP), or Degos syndrome, is a rare disorder of unknown etiology. It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules.
We report the case of a 42-year-old woman with a history of progressive systemic sclerosis who presented with painful subcutaneous nodules on her abdomen along with chronic atrophic papules on her upper and lower limbs. Biopsy results of both types of lesions revealed vascular thrombi without surrounding inflammation.
We briefly review the literature on MAP and its association with various connective tissue diseases. To our knowledge, there have been no previous reports of a patient with the clinical and histologic presentations described here. Although the histologic appearance of the subcutaneous nodules was very similar to that of the atrophic papules, the clinical characteristics of the 2 types of lesions were strikingly different. It is fair to theorize that Degos lesions do not start as atrophic porcelain-white papules but rather evolve from a primary lesion.
We hypothesize that these lesions start as painful red nodules and may represent part of the disease spectrum in the evolution of MAP.
PATHOGENESIS CHARACTERIZATION Unknown-has been considered a vasculitis, mucinosis, or thrombotic disorder No circulating immune complexes, anti-endothelial cell antibodies, or anticardiolipin antibodies identified
ENDOVASCULITIS Some consider a primary endothelial cell defect with secondary thrombosis leading to infarctive changes LUPUS VARIANT
Degos' Disease: A Distinctive Pattern of Disease, Chiefly of Lupus Erythematosus, And Not a Specific Disease per se.Ball E, Newburger A, Ackerman AB.
Am J Dermatopathol. 2003 Aug;25(4):308-20. Abstract quote Degos' disease, known confusingly as malignant strophic papularis, is an uncommon condition of unknown cause characterized by distinctive infarctive lesions in the skin, gastrointestinal tract, and central nervous system; the lesions at the two latter sites often result in death.
We deem Degos' disease to be analogous to lupus erythematosus in the sense that each is fundamentally a systemic pathologic process involving several organs, among them the skin, but, moreover, we regard Degos' disease, in most instances, to be an actual manifestation of lupus erythematosus. Histopathologically, the findings in sections of tissue of skin lesions of Degos' disease are indistinguishable from those of one expression of cutaneous lupus erythematosus; immunopathologically, some patients with morphologic findings stereotypical of Degos' disease display signs characteristic of lupus erythematosus.
For these reasons, we consider Degos' disease to be a distinctive pattern of disease, rather than a specific disease per se, just as are erythema multiforme, erythema nodosum, leukocytoclastic vasculitis, Sweet's syndrome, and pyoderma gangrenosum, to name but five of scores of them.
The singular pattern that is designated Degos' disease usually is an expression of lupus erythematosus, but, episodically, of conditions like dermatomyositis and rheumatoid arthritis.
LABORATORY/
RADIOLOGIC/
OTHER TESTSCHARACTERIZATION LABORATORY Impaired fibrinolytic activity Alterations in platelet function MRI
- Nervous system involvement in Degos disease.
Amato C, Ferri R, Elia M, Cosentino F, Schepis C, Siragusa M, Moschini M.
Department of Neuroradiology, Oasi Institute for Research on Mental Retardation and Brain Aging, Troina, Italy.
AJNR Am J Neuroradiol. 2005 Mar;26(3):646-9. Abstract quote
Degos disease, or malignant atrophic papulosis, is a rare obstructive vasculopathy of unknown origin, characterized by distinctive skin lesions, visceral involvement, and an unfavorable outcome. The gastrointestinal tract and the central nervous system are most frequently affected, but cases limited to benign skin lesions have also been described. Neuroradiologic reports of this condition are exceptionally rare.
We report the case of a 29-year-old woman with central and peripheral nervous system involvement who presented with progressive clinical deterioration and a meningovascular pattern at cerebral MR imaging; other organs were spared in this patient.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
- A case of systemic malignant atrophic papulosis (Kohlmeier-Degos' disease).
Fernandez-Perez ER, Grabscheid E, Scheinfeld NS.
Department of Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA.
J Natl Med Assoc. 2005 Mar;97(3):421-5. Abstract quote
Malignant atrophic papulosis (MAP) is a rare and clinically distinctive vasculopathy. It is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction in the involved organ systems.
Its rarity and rapidly fatal course make the disease a difficult diagnostic and therapeutic challenge.VARIANTS CENTRAL NERVOUS SYSTEM Infarction EYE
- Malignant atrophic papulosis. Report of a case with multiple ophthalmic findings.
Thomas RK, Nithyanandam S, Rawoof BA, Rajendran SC.
Department of Ophthalmology, St. John's National Academy of Health Sciences, Johnnagar, Bangalore, India.
Indian J Ophthalmol. 2003 Sep;51(3):260-3. Abstract quote
Malignant atrophic papulosis is a rare and fatal condition with multiple organ involvement.
We describe a patient with progressive ocular and dermatological findings.
GASTROINTESTINAL TRACT Infarction SKIN Crops of papules ranging from 0.5-1 cm which develop an umbilication with a porcelain white center with a telangiectatic ring
Evolves to leave an atrophic scar
10-40 lesions all in different stages of evolution
SMALL INTESTINE
- Presentation of Degos syndrome as acute small-bowel perforation.
Gonzalez Valverde FM, Menarguez Pina F, Ruiz JA, Gomez Ramos MJ, Mauri Barbera F, Luri Prieto P, Garcia Marcilla JA, Vazquez Rojas JL.
Department of Surgery, Hospital General Vega Baja, Servicio de Cirugia General, Murcia, Spain.
Arch Surg. 2003 Jan;138(1):57-8 Abstract quote.
Malignant atrophic papulosis is an uncommon vasculopathy affecting multiple systems and frequently associated with a poor prognosis.
We describe a 56-year-old patient with this rare disorder whose death was the result of intestinal involvement.
Clinical diagnostic features of malignant atrophic papulosis as well as the main therapeutic approaches suggested in the literature are presented herein.Small bowel fistulae in Degos' disease: a case report and literature review.
Atchabahian A, Laisne MJ, Riche F, Briard C, Nemeth J, Valleur P.
Washington University Plastic Surgery, St. Louis, Missouri, USA.
Am J Gastroenterol 1996 Oct;91(10):2208-11 Abstract quote
Degos' disease (malignant atrophic papulosis) is a rare, progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. We report a case with bowel involvement followed by enterocutaneous fistulae. Diagnostic laparoscopy with jejunal biopsy was followed by jejunal perforations, peritonitis, and fistulae leading to death after a 4-month course in the intensive care unit. The usual treatment of enterocutaneous fistula by somatostatin and parenteral nutrition was ineffective in this case. The course of the disease in our patient was not usual, as can be seen in a literature review underlining the specific features of Degos' disease. Laparoscopy and bowel biopsy should be avoided in this context. Degos' disease should be considered in the differential diagnosis of a primary ulceration of the small intestine.
HISTOLOGICAL TYPES CHARACTERIZATION SKIN Well developed lesions have epidermal atrophy with hyperkeratosis overlying a wedge shaped area of cutaneous ischemia, extending into the deep dermis
Superficial and deep perivascular lymphocytic infiltrate is present at the edge of the ischemia
Marked endothelial swelling and occasional platelet-fibrin thrombi
Epidermis may show infarctive changes or scattered necrotic keratinocytes
Abundant acid mucopolysaccharides in the dermis giving an appearance of a dermal mucinosis
Is Degos' disease a clinical and histological end point rather than a specific disease?
High WA, Aranda J, Patel SB, Cockerell CJ, Costner MI.
J Am Acad Dermatol. 2004 Jun;50(6):895-9. Abstract quote
Degos' disease is described as a rare disorder, with approximately 100 cases detailed in the literature. Nearly all are characterized by the near "pathognomonic" appearance of porcelain-white, atrophic papules with peripheral erythema and telangiectases. Many Degos' disease variants have been described including benign cutaneous Degos' disease, familial Degos' disease, atrophie blanche with Degos'-like features, and connective tissue diseases with similar findings. The course, prognosis, and treatment have substantially varied.
We present four patients: the first carries a diagnosis compatible with classic Degos' disease, the second and third demonstrate cutaneous and histological findings of Degos' disease but laboratory evidence suggestive of lupus erythematosus, while the fourth has dermatomyositis with Degos'-like lesions.
Because of broad overlap in clinical and histological findings, we contend that Degos' disease may not be a specific entity, but rather, may represent a common end point to a variety of vascular insults, many of which have not been fully elucidated.Panniculitis mimicking lupus erythematosus profundus: a new histopathologic finding in malignant atrophic papulosis (Degos disease).
Grilli R, Soriano ML, Izquierdo MJ, Farina MC, Martin L, Manzarbeitia F, Requena L.
Department of Dermatology, Fundacion Jiminez- Diaz, Universidad Autonoma, Madrid, Spain.
Am J Dermatopathol 1999 Aug;21(4):365-8 Abstract quote
We present the case of a 57-year-old woman with a 1-year history of a cutaneous eruption clinically and histopathologically characteristic of Degos disease.
In one of the two cutaneous biopsy specimens taken from the trunk lesions, the histopathology consisted of necrosis and sclerosis of the subcutaneous lobules, a finding that to our knowledge has not been previously described in the cutaneous lesions of Degos disease.
Two months after cutaneous biopsies were taken, the patient developed ptosis and an episode of acute abdominal pain; intestinal perforation and many characteristic lesions of visceral Degos disease involving the entire small bowel were noted at laparotomy.
Benign Cutaneous Degos' Disease A Case Report With Emphasis on Histopathology As Papules Chronologically Evolve
Jeff D. Harvell, etal.
Am J Dermatopathol 2001;23:116-123
The following case report details a 53-year-old man with a 6-year history of the benign cutaneous or skin-limited form of Degos' disease.
Clinically, the patient demonstrated a diffuse eruption of papules on the upper trunk and arms. Many papules demonstrated the classic porcelain-white centers characteristic of Degos' disease, but others exhibited different clinical morphologies that corresponded to the evolutionary stages of papules originally described by Degos. Over the course of several clinic visits, the patient underwent a total of 5 punch biopsies, the histologies of which were correlated with their clinical morphologies.
Early papules were skin-colored and demonstrated a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition. The overlying epidermis showed a mild vacuolar interface reaction and the histologic appearances at this early stage resembled tumid lupus erythematosus.
Fully developed papules were raised with umbilicated porcelain-white centers and a surrounding erythematous rim. Histologically these exhibited a prominent interface reaction with squamatization of the dermo-epidermal junction, melanin incontinence, epidermal atrophy, and a developing zone of papillary dermal sclerosis that resembled the early stages of lichen sclerosus et atrophicus in miniature. These interface reactions were invariably confined to the central portion of the punch biopsy specimen, corresponding to the central porcelain-white area seen clinically. Additional features of fully developed papules included a prominent lymphocytic vasculitis affecting venules, a mild periadnexal infiltrate of neutrophils and/or eosinophils, and interstitial mucin deposition. In late-stage papules, the porcelain-white areas were better developed and the lesion flattened.
Histologically, the degree of inflammation was generally sparse and the overall picture mirrored the classic histologic description of Degos' disease with a central roughly wedge-shaped zone of sclerosis surmounted by an atrophic epidermis and hyperkeratotic compact stratum corneum.
These late-stage papules closely resembled a miniaturized version of fully developed lichen sclerosus et atrophicus confined to the center of the punch biopsy specimen.
SPECIAL STAINS/
IMMUNOPEROXIDASECHARACTERIZATION Direct immunofluorescence (DIF) Conflicting results with perivascular fibrin and complement in some cases
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Dermal mucinosis DERMATOMYOSITIS
- Lesions resembling malignant atrophic papulosis in a patient with dermatomyositis.
Tsao H, Busam K, Barnhill RL, Haynes HA.
Department of Dermatology, Harvard Medical School, Brookline, MA, USA.
J Am Acad Dermatol. 1997 Feb;36(2 Pt 2):317-9. Abstract quote
Many consider porcelain white atrophic papules as pathognomonic for malignant atrophic papulosis (MAP), or Degos' disease.
During the past three decades, five patients with a collagen vascular disease have been reported to have MAP-like lesions as a manifestation of their underlying illness.
We describe a patient with dermatomyositis who had porcelain-white atrophic papules resembling malignant atrophic papulosis.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS Gastrointestinal involvement may occur in up to 60% of cases-death may occur
Patients with skin lesions only may have a good prognosis
Approximately 15% of Degos' disease exhibit long-term survival with disease often limited to the skin and with no history of catastrophic bowel or central nervous system involvement TREATMENT ANTI-PLATELET THERAPY
- Malignant atrophic papulosis: response to antiplatelet therapy.
Drucker CR.
Baylor College of Medicine, Houston, Tex.
Dermatologica. 1990;180(2):90-2 Abstract quote.
A patient with malignant atrophic papulosis (Degos' disease) is studied. Platelet function studies showed abnormalities of increased adhesiveness and aggregation. Treatment with dipyridamole and aspirin correlated with reversal of these abnormalities and cessation of new lesion development. When treatment was stopped, platelet function abnormalities and new skin lesions developed within 2 months. With reinstitution of therapy, the patient developed no new lesions in the 5 months he was followed.
This is the second report of platelet function abnormality and apparent successful treatment with antiplatelet therapy in a patient with Degos' disease.Arch Pathol Lab Med 1989;113:354-362.
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Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
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Last Updated June 15, 2005
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