No abstract available.
Malignant Atrophic Papulosis*

No abstract available.
Malignant Atrophic Papulosis*

No abstract available.
Malignant Atrophic Papulosis*

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.
Adult ; Axilla ; Biopsy ; Humans ; Hyperpigmentation ; Malignant Atrophic Papulosis ; Neck ; Pigmentation

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.
Adult ; Aspirin ; Humans ; Male ; Malignant Atrophic Papulosis* ; Necrobiotic Disorders ; 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

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

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.
Adolescent ; Arterioles ; Biopsy ; Central Nervous System ; Humans ; Infarction ; Intestines ; Korea ; Malignant Atrophic Papulosis* ; Nausea ; Peritonitis ; Rare Diseases ; Skin

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.
Abdominal Pain ; Adolescent ; Appendectomy ; Dermis ; Dipyridamole ; Epidermis ; Extremities ; Female ; Humans ; Malignant Atrophic Papulosis* ; Nausea ; Necrosis ; Salicylic Acid ; Skin ; Vomiting

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