Departments of Dermatology, Venerology, Allergology and Immunology, Dessau Medical Center, Auenweg 38, Dessau, 06847, Germany.
Orphanet J Rare Dis. 2013 Jan 14;8:10. doi: 10.1186/1750-1172-8-10.
DEFINITION OF THE DISEASE: Malignant atrophic papulosis (MAP), described independently by Köhlmeier and Degos et al., is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and surrounding teleangiectatic rim.
Less than 200 cases have been described in the literature. The first manifestation of MAP usually occurs between the 20th and 50th year of life.
The cutaneous clinical picture is almost pathognomonic. The histology is not consistent but in most cases it shows a wedge-shaped connective tissue necrosis in the deep corium due to a thrombotic occlusion of the small arteries. In the systemic variant, manifestations mostly occur at the intestine and central nervous system.
The etiopathogenesis of the disease remains unknown, a genetic predisposition may occur. Vasculitis, coagulopathy or primary dysfunction of the endothelial cells have been implicated.
Diagnosis is only based on the characteristic skin lesions. DIFFERRENTIAL DIAGNOSIS: It depends on the clinical presentation of MAP, but systemic lupus erythematosus and other connective tissue diseases need to be considered.
No effective treatment exists for the systemic manifestations, while compounds that facilitate blood perfusion have achieved a partial regression of the skin lesions in single cases.
An apparently idiopathic, monosymptomatic, cutaneous, benign variant and a progressive, visceral one with approx. 50% lethality within 2-3 years have been reported. Systemic manifestations can develop years after the occurrence of skin lesions leading to bowel perforation and peritonitis, thrombosis of the cerebral arteries or massive intracerebral hemorrhage, meningitis, encephalitis, radiculopathy, myelitis.
疾病定义：恶性萎缩性丘疹病（MAP），由 Köhlmeier 和 Degos 等人独立描述，是一种罕见的慢性血栓闭塞性血管病，其特征为丘疹性皮肤损害，中央呈瓷白色萎缩，周围有毛细血管扩张边缘。
文献中描述的病例少于 200 例。MAP 的最初表现通常发生在 20 至 50 岁之间。
皮肤临床图像几乎具有特征性。组织学不一致，但在大多数情况下，它显示深部真皮中的楔形结缔组织坏死，由于小动脉的血栓闭塞。在系统性变异中，表现主要发生在肠道和中枢神经系统。
疾病的病因发病机制仍不清楚，可能存在遗传易感性。已经提出了血管炎、凝血功能障碍或内皮细胞的原发性功能障碍。
诊断仅基于特征性皮肤损害。
取决于 MAP 的临床表现，但需要考虑系统性红斑狼疮和其他结缔组织疾病。
系统性表现尚无有效治疗方法，而促进血液灌注的化合物在个别病例中已实现皮肤病变的部分消退。
据报道，有明显特发性、单症状性、皮肤良性变异型和进行性内脏型，约 50%的死亡率在 2-3 年内。皮肤病变发生多年后可出现系统性表现，导致肠穿孔和腹膜炎、脑动脉血栓形成或大量脑出血、脑膜炎、脑炎、神经根病、脊髓炎。
