malignant atrophic papulosis/eritema

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Is Degos' disease a clinical and histological end point rather than a specific disease?

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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

Erythrokeratoderma en cocardes.

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The erythrokeratodermas are a distinct but clinically variable group of rare geno-dermatoses, characterized by circumscribed erythematous and hyperkeratotic lesions. All attempts to establish a valid classification have been based on purely clinical and morphologic criteria. Erythrokeratoderma en

Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se.

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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'

Laparoscopy shows superiority over endoscopy for early detection of malignant atrophic papulosis gastrointestinal complications: a case report and review of literature.

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BACKGROUND The malignant form of atrophic papulosis (Köhlmeier-Degos disease) is a rare thrombo-occlusive vasculopathy that can affect multiple organ systems. Patients typically present with distinctive skin lesions reflective of vascular drop out. The small bowel is the most common internal organ

Erythrokeratodermia variabilis with erythema gyratum repens-like lesions.

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A large pedigree with erythrokeratodermia variabilis (EKV) and erythema gyratum repens-like lesions is described. Clinical, laboratory, and histologic findings of this family are presented. The differential diagnoses of the following dermatoses with an erythematous and a hyperkeratotic component are

Prominent Degos-like skin lesions in a patient with chronic cutaneous lupus erythematosus.

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Malignant atrophic papulosis, commonly known as Degos disease, is a rare vasculopathy encompassing both benign, cutaneous and lethal systemic variants. We report a case of chronic cutaneous lupus erythematosus in a 41-year-old male presenting with prominent Degos-like skin lesions. Multiple

Degos-Like Lesions Associated with Systemic Lupus Erythematosus.

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Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic

Degos cutaneous disease with features of connective tissue disease.

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A 30-year-old woman was referred on April 2002 for a plaque that involved the internal aspect of the right leg, an erythema nodosum-like lesion on the lower extremities, and periarthritis on her left ankle. Subsequently, the patient developed anular, atrophic, growing, porcelain-white papules, with

Diseases associated with hidranitis suppurativa: part 2 of a series on hidradenitis.

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Hidradenitis suppurativa (HS), a pathologic follicular disease, impacts patients' lives profoundly and usually occurs in isolation. The diseases with the strongest association are obesity, depression, and pain. HS is associated with many diseases including acne conglobata (AC), dissecting

Diagnosis and treatment of cutaneous paraneoplastic disorders.

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The skin plays a critical role in the detection of internal malignances. Cutaneous signs of these disorders afford clinicians opportunities for early diagnosis and treatment. We aim to succinctly review the recognition, diagnosis, and treatment of selected cutaneous paraneoplastic diseases. Skin

Cutaneous manifestations of gastrointestinal disease: part II.

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The gastrointestinal (GI) and cutaneous organ systems are closely linked. In part I of this continuing medical education article, the intricacies of this relationship were explored as they pertained to hereditary polyposis disorders, hamartomatous disorders, and paraneoplastic disease. Part II

Degos-Like Lesions In Association With Connective Tissue Diseases: A Report Of Three Cases And Literature Review.

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Degos disease is characterized by atrophic porcelain-white papules with peripheral erythema. Degos-like lesions have been reported in association with several connective tissue diseases (CTDs), mostly systemic lupus erythematosus (SLE), and rarely in dermatomyositis and systemic sclerosis. Herein,

Dermoscopy in General Dermatology: A Practical Overview.

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Over the last few years, dermoscopy has been shown to be a useful tool in assisting the noninvasive diagnosis of various general dermatological disorders. In this article, we sought to provide an up-to-date practical overview on the use of dermoscopy in general dermatology by analysing the

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