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 rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Approximately 15% of Degos disease have a benign course limited to the skin and no history of gastrointestinal or central nervous system (CNS) involvement. A 29-year-old female with history of systemic lupus erythematosus (SLE) presented with a 2-year history of asymptomatic lesions on the dorsum of all fingers and both knees. The patient had only skin lesions and no gastrointestinal or CNS vasculitis symptoms. Her skin lesions were umbilicated, atrophic porcelain-white lesions with a rim of erythema. On the basis of clinical, histologic, and laboratory findings, a diagnosis of Degos-like lesions associated with SLE was made. The patient had been treated for SLE for 7 years. Her treatment regimen was maintained over a 2 month follow-up period, and the skin lesions improved slightly with no development of new lesions.
Adult ; Autoimmune Diseases ; Central Nervous System ; Diagnosis ; Erythema ; Female ; Fingers ; Follow-Up Studies ; Humans ; Infarction ; Knee ; Lupus Erythematosus, Systemic* ; Malignant Atrophic Papulosis ; Skin ; Vasculitis ; Vasculitis, Central Nervous System

No abstract available.
Malignant Atrophic Papulosis*

Malignant atrophic papulosis (MAP), also known as Degos' disease, is a rare and often fatal occlusive thrombotic vasculopathy, with fewer than seven cases reported in Korea. MAP is characterized by porcelain-white, atrophic, papular skin lesions and multi-organ system involvement, especially the gastrointestinal (GI) tract and nervous system. Involvement of the GI tract is usually associated with a poor prognosis. To date, no treatment has been shown to be effective in the treatment of MAP. We describe a 52-year-old man who presented with a 5-month history of abdominal angina and a 2-year history of multiple skin lesions on the trunk and extremities. The skin lesions were papules, 4-6 mm in diameter, with a porcelain-white center and a slightly raised erythematous telangiectatic rim. A biopsy of a skin lesion showed a wedge-shaped degeneration of collagen in the dermis and atrophic epidermis. An explorative laparoscopy revealed multiple, yellow-white plaques scattered throughout the small bowel. A biopsy of the small bowel showed sclerotic vascular alterations containing intravascular fibrin thrombi. He was started on aspirin (100 mg daily) and has survived for 24 months since the onset of gastrointestinal symptoms.
Aspirin ; Biopsy ; Collagen ; Dermis ; Epidermis ; Extremities ; Fibrin ; Gastrointestinal Tract ; Humans ; Korea ; Laparoscopy ; Malignant Atrophic Papulosis ; Middle Aged ; Nervous System ; Prognosis ; Skin

Malignant atrophic papulosis (MAP), also known as Degos' disease, is a rare and often fatal occlusive thrombotic vasculopathy, with fewer than seven cases reported in Korea. MAP is characterized by porcelain-white, atrophic, papular skin lesions and multi-organ system involvement, especially the gastrointestinal (GI) tract and nervous system. Involvement of the GI tract is usually associated with a poor prognosis. To date, no treatment has been shown to be effective in the treatment of MAP. We describe a 52-year-old man who presented with a 5-month history of abdominal angina and a 2-year history of multiple skin lesions on the trunk and extremities. The skin lesions were papules, 4-6 mm in diameter, with a porcelain-white center and a slightly raised erythematous telangiectatic rim. A biopsy of a skin lesion showed a wedge-shaped degeneration of collagen in the dermis and atrophic epidermis. An explorative laparoscopy revealed multiple, yellow-white plaques scattered throughout the small bowel. A biopsy of the small bowel showed sclerotic vascular alterations containing intravascular fibrin thrombi. He was started on aspirin (100 mg daily) and has survived for 24 months since the onset of gastrointestinal symptoms.
Aspirin ; Biopsy ; Collagen ; Dermis ; Epidermis ; Extremities ; Fibrin ; Gastrointestinal Tract ; Humans ; Korea ; Laparoscopy ; Malignant Atrophic Papulosis ; Middle Aged ; Nervous System ; Prognosis ; Skin

Adult ; Female ; Humans ; Intestinal Perforation ; etiology ; pathology ; surgery ; Malignant Atrophic Papulosis ; complications ; pathology ; surgery ; Young Adult

Degos' disease, malignant atrophic papulosis, is a rare disease characterized by a pathognomonic appearance of porcelain-white, atrophic papules with peripheral erythema and multiple occlusive thrombotic vascular pathology. It sometimes involves internal viscera such as the gastrointestinal tract, the central nervous system and kidney which may bring to death. Although several reports showed the associations between Degos' disease and antiphospholipid antibody or systemic lupus erythematosus, its pathogenetic significance remains elusive. The previous 4 Korean cases of Degos's disease involved only skin and/or the gastrointestinal tract. We report a case of systemic lupus erythematosus with Degos' disease involving skin, kidney and small intestine.
Antibodies, Antiphospholipid ; Central Nervous System ; Erythema ; Gastrointestinal Tract ; Intestine, Small ; Kidney ; Lupus Erythematosus, Systemic* ; Malignant Atrophic Papulosis ; Pathology ; Rare Diseases ; Skin ; Viscera

A 33-year-old male patient had malignant atrophic papulosis that affected the skin and intestinal tract and probably respiratory system. He had multiple typical skin lesion with porcelain-white atrophic scars on the whole body surface area except on face, palm and sole. And he had been operated two times due to intestinal perforation after onset. Multiple whitish necrotic patches were found in small and large intestine during laparotomy. Also he had a plueral effusion. Histopathologic studies showed atrophic epidermis and dermal necrosis, vascular change, mucin deposition in lower dermis. He was treated with acetyl salicylic acid and dipyridamole, but there was no improve-ment. He died of intestinal perforation and respiratory failure 67 months after onset(6 months after onset of abdominal symptom).
Adult ; Body Surface Area ; Cicatrix ; Dermis ; Dipyridamole ; Epidermis ; Humans ; Intestinal Perforation ; Intestine, Large ; Laparotomy ; Male ; Malignant Atrophic Papulosis* ; Mucins ; Necrosis ; Respiratory Insufficiency ; Respiratory System ; Salicylic Acid ; Skin*

Reticulate acropigmentation of Kitamura (RAPK) is an autosomal dominant dermatosis comprising of reticulate slightly clepessed pigmentation of the extensor surfaces of the hands and feet with palmar pits. It has been suggested that this may be the same disease as Dawling Degos disease (DDD), an autosomal dorninant condition which is characterized by a reticulate pigmentation of flexures, comedo-like lesicns and pitted scars. We present a case of RAPK in a 49-year-old female who had reticulate, brownish, slightly depressed pigmentation on the extremities with t,he involvement of flexures, the predilection sites of DDD.
Cicatrix ; Dichlorodiphenyldichloroethane ; Extremities ; Female ; Foot ; Hand ; Humans ; Malignant Atrophic Papulosis ; Middle Aged ; Pigmentation ; Skin Diseases

No abstract available.
Malignant Atrophic Papulosis*

No abstract available.
Malignant Atrophic Papulosis*