A case of malignant atrophic papulosis with cranial nerve and peripheral nerve impairment

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.

Case for diagnosis

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.

Nursing of patients with malignant atrophic papulosis: a case report / 中国实用护理杂志

Objective To understand the etiology and clinical manifestation of malignant atrophic papulosis,to report the psychological care of one case with malignant atrophic papules,and patients to maintain a good psychological state.Methods Nursing measures such as strengthening skin care,reducing discomfort,avoiding aggravating the injury; attention to abdominal signs,detection of changes in disease condition,prevention of intestinal perforation,related medication education to increase medication compliance were given to this patients.Results The abdominal pain disappeared,lower limb muscle force recovered,and the patient was discharged after the disease condition was steady.Conclusions To patients with malignant atrophic papulosis,early detection and early treatment should be given to them.

Malignant atrophic papulosis with neurological involvement / 中华皮肤科杂志

A 48-year-old female presented with a 6-year history of papules and plaques all over the body and with 1-year history of blurred vision in the right eye.Physical examination showed porcelain-white atrophic papules with peripheral erythematous halo and telangiectnsia.She also suffered from exotropia,visual deterioration,visual field defects of the right eye,as well as numbness of the left index finger,thumb and right anterior tibia.Skin biopsies of abdominal lesions revealed dermal necrosis with mucoid degeneration,inflammatory infiltration predominated by lymphocytes around several small blood vessels and occlusion of some blood vessels in deep dermis.Colonoscopy of the whole colon demonstrated scattered patches of hyperemia and erosions with the formation of shallow ulcers.Nerve electromyologram revealed damage to the nerves of right quadriceps femoris muscles.Fecal analysis showed that occult blood was strongly positive.A diagnosis of malignant atrophic papulosis was made based on the characteristic clinical presentation,laboratory and histopathological findings.She was treated with dipyridamole and aspirin for three months,which resulted in no clinical improvement or deterioration.

A Case of Degos' Disease Presenting with Abdominal Angina and Widespread Skin Lesions

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.

A Case of Degos' Disease Presenting with Abdominal Angina and Widespread Skin Lesions

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.

A Case of Systemic Lupus Erythematosus with Degos' Disease / 대한류마티스학회지

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.

Degos' disease:A case report and review of literature / 北京大学学报(医学版)

A 18-year-old male patient's case was diagnosed as Degos'disease with pathognomonic skin lesions,accompanied by small bowel perforation,and reported here. Skin histopothological test show that the typical wedge-shaped necrobiosis and lymphocyte inflammatory infiltration. Vessels showed narrowing and thrombosis,with lymphocyte infiltration. Degos'disease is a systemic necrotizing vasculitis. Skin biopsy can confirm its diagnosis. Severe systemic complication should be prevented. Degos'disease should be considered in the differential diagnosis of skin lesions associated with systemic involvement.

A Case of Malignant Atrophic Papulosis Showed Typical Skin and Intestinal Lesions / 대한피부과학회지

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).

A case of malignant atrophic papulosis (Degos' disease)

No abstract available.

A Case of Malignant Atrophic Papulosis (Degos' Disease) / 대한피부과학회지

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.