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

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

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 (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

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