No abstract available.
Malignant Atrophic Papulosis*

No abstract available.
Malignant Atrophic Papulosis*

No abstract available.
Malignant Atrophic Papulosis*

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

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

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

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

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

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