ABSTRACT
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.
Subject(s)
Adult , Female , Humans , Autoimmune Diseases , Central Nervous System , Diagnosis , Erythema , Fingers , Follow-Up Studies , Infarction , Knee , Lupus Erythematosus, Systemic , Malignant Atrophic Papulosis , Skin , Vasculitis , Vasculitis, Central Nervous SystemABSTRACT
Malignant atrophic papulosisis is a rare, multisystem obliterative vasculopathy of unknown etiology, occasionally involving the cranial nerve. We describe the first case of malignant atrophic papulosisis with cranial nerve and peripheral nerve involvement in China. A 47-year-old woman presented to our hospital with atrophic porcelain white papules over the trunk and extremities, numbness in the right calf, vision decrease and impaired movement of the right eye. She was diagnosed with malignant atrophic papulosisis, based on characteristic symptoms and histopathologic examination. The patient was treated with dipyridamole and aspirin for 9 months, but later died of gastrointestinal hemorrhage. We reviewed currently available case reports on cranial nerve involvement in malignant atrophic papulosisis and emphasized the importance of skin biopsy in diagnosing this disease.
.Subject(s)
Female , Humans , Middle Aged , Cranial Nerve Diseases/pathology , Malignant Atrophic Papulosis/pathology , Peripheral Nervous System Diseases/pathology , Biopsy , Cranial Nerve Diseases/drug therapy , Fatal Outcome , Malignant Atrophic Papulosis/drug therapy , Peripheral Nervous System Diseases/drug therapy , Skin/pathologyABSTRACT
Degos disease, also known as malignant atrophic papulosis, is a rare occlusive vasculopathy of unknown etiology characterized by infarcts in the dermis, gastrointestinal tract, central nervous system, and other organs. It is characterized by papules, which become umbilicated and evolve with a depressed porcelain-white central area, with an erythematous halo with telangiectasias. Histological findings include wedge-shaped dermoepidermal necrosis and blood vessel thrombosis. Approximately 50-60% of patients with systemic symptoms die within 2-3 years, most due to gastrointestinal perforation. We report a typical case, with lethal outcome, in a 45-year-old woman.
Subject(s)
Humans , Female , Middle Aged , Skin/pathology , Malignant Atrophic Papulosis/pathology , Biopsy , Fatal Outcome , Venous Thrombosis/pathology , Malignant Atrophic Papulosis/complications , Intestinal Perforation/complicationsABSTRACT
Presentamos una paciente de sexo femenino, de 53 años de edad, con manifestaciones cutáneas de enfermedad de Degos, sin compromiso sistémico asociado. Se realiza una revisión actualizada de la bibliografía médica mundial, con una descripción clínica, histopatológica, diagnóstica y terapéutica de la enfermedad.
Subject(s)
Humans , Female , Middle Aged , Malignant Atrophic Papulosis/pathology , Malignant Atrophic Papulosis/drug therapy , Skin Diseases/pathology , Extremities/pathology , Thorax/pathologyABSTRACT
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.
Subject(s)
Humans , Middle Aged , Aspirin , Biopsy , Collagen , Dermis , Epidermis , Extremities , Fibrin , Gastrointestinal Tract , Korea , Laparoscopy , Malignant Atrophic Papulosis , Nervous System , Prognosis , SkinABSTRACT
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.
Subject(s)
Humans , Middle Aged , Aspirin , Biopsy , Collagen , Dermis , Epidermis , Extremities , Fibrin , Gastrointestinal Tract , Korea , Laparoscopy , Malignant Atrophic Papulosis , Nervous System , Prognosis , SkinSubject(s)
Adult , Female , Humans , Brain Ischemia/etiology , Malignant Atrophic Papulosis/complications , Aspirin/therapeutic use , Brain Ischemia/diagnosis , Brain Ischemia/drug therapy , Fibrinolytic Agents/therapeutic use , Malignant Atrophic Papulosis/pathology , Malignant Atrophic Papulosis/therapy , Ticlopidine/therapeutic useABSTRACT
Degos disease DD is a rare obstructive vasculopathy characterized by distinctive skin lesions. Involvement of the soles, palms and genitalia is rare. In most cases, disease has an unfavorable course and involves gastrointestinal tract, central nervous system and occasionally other organs. Pleural and pericardial involvements are usually minor manifestations with prolonged course. Death occurs in approximately 50% of the patients usually due to intestinal perforation or central nervous system bleeding. We describe a 48-year-old man of lethal systemic DD. Widespread skin lesions with involvement of palmoplantar surfaces, genitalia and scalp were ignored for 3 years, whereas the disease revealed own malignant nature. The disorder progressed to nervous, gastrointestinal and cardiopulmonary system that led to death after 5 months from onset of systemic involvement as severe restrictive cardio-pulmonary insufficiency. Autopsy showed diffuse fibrotic changes in serosal membranes and internal organs
Subject(s)
Humans , Male , Malignant Atrophic Papulosis/complications , Multiple Organ Failure , Heart Arrest/etiology , Fatal Outcome , Diagnosis, DifferentialABSTRACT
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.
Subject(s)
Antibodies, Antiphospholipid , Central Nervous System , Erythema , Gastrointestinal Tract , Intestine, Small , Kidney , Lupus Erythematosus, Systemic , Malignant Atrophic Papulosis , Pathology , Rare Diseases , Skin , VisceraABSTRACT
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).
Subject(s)
Adult , Humans , Male , Body Surface Area , Cicatrix , Dermis , Dipyridamole , Epidermis , Intestinal Perforation , Intestine, Large , Laparotomy , Malignant Atrophic Papulosis , Mucins , Necrosis , Respiratory Insufficiency , Respiratory System , Salicylic Acid , SkinABSTRACT
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.
Subject(s)
Female , Humans , Middle Aged , Cicatrix , Dichlorodiphenyldichloroethane , Extremities , Foot , Hand , Malignant Atrophic Papulosis , Pigmentation , Skin DiseasesABSTRACT
A 15-year-old female patient had malignant atrophic papulosis(Degos' disease) that affected the skin and probably intestinal tract. She had multiple erythematous papules with porcelain-white atrophic centers on the trunk and extremities. Also she suffered from intermittent nausea, vomiting and abdominal pain. She received appendectomy for abdominal pain 2 months ago. Histopathologic studies showed atrophic epidermis and necrobiotic dermis. There were endothelial thickening, fibrinoid necrosis of bload vessels, perivascular lymphocytic infiltation in the lower dermis. She was treatd with acetyl salicylic acid and dipyridamole, but there was no improvement.
Subject(s)
Adolescent , Female , Humans , Abdominal Pain , Appendectomy , Dermis , Dipyridamole , Epidermis , Extremities , Malignant Atrophic Papulosis , Nausea , Necrosis , Salicylic Acid , Skin , VomitingABSTRACT
A 31-year-old male patient had malignant atrophic papulosis that afected the skin only. He had multiple typical porcelain-white skin lesion on the face and trunk without abdominal symptoms. Histopathologic studies showed moderate inflammatory infiltration in addition to the typical cone-shaped region of necrobiosis, There are no satisfactory treatment modes, and in the present case, the patient's condition improved transiently on oral medication of aspirin, but we lost the contact with him afterwards.
Subject(s)
Adult , Humans , Male , Aspirin , Malignant Atrophic Papulosis , Necrobiotic Disorders , SkinABSTRACT
Reticulated pigrnented anomalies of the flexures is a pigmentary disorder bearing genetic back ground, also known as Dowling Degos disease or dark dot disease. It is characterized reticulated brownish black macules on the flexural areas with the histological features of filiform pigmented epidermal downward proliferation and the presence of intraepidermal keratin containing cysts. We report 34-year-old housewife who showed brawnish black reticulated macules on her axillae, anterior neck and genitocural folds. Pigmented comedones were also found at the marginal areas of the axillae. Biopsy specimen revealed the presence of intraepidermal keratin containing cysts and basilar hyperpigmentation.
Subject(s)
Adult , Humans , Axilla , Biopsy , Hyperpigmentation , Malignant Atrophic Papulosis , Neck , PigmentationABSTRACT
Malignant atrophic papulosis (Degosdisease) was first described in detail by Degos and his co-worker in I942, In this rare disease of unknown etiology, porcehun-white skin lesians develop, and death usually occurs from perforation of the intestine weeks to years after onset, and also but rarely from infarction of central nervous system. Our case of Degos disease in an 18 years old man with typical skin lesions and abdominal symptoms such as pain, nausea and distension due to gastrointestinal involvement is described. He died of peritonitis 9 months after onset. The biopsy specimen of an atrophic papule showed thrombotic occlusion of an arteriole. The authors experienced a case of Degos disease diagnosed by clinical and histopathologieal findings for the first time in Korea.