恶性萎缩性丘疹病一例

报道1例恶性萎缩性丘疹病。患者男,58岁。因全身皮损5年余,急腹症剖腹探查术后11 d入院。躯干、四肢散在分布圆形、椭圆形淡红色丘疹,黄豆大小,周边呈环状隆起,境界清楚,中央破溃、萎缩。皮损组织病理示真皮深部小血管周围淋巴细胞浸润,部分小血管管腔闭塞。临床表现及组织病理改变符合恶性萎缩性丘疹病。该病临床罕见,累及系统,死亡率高。     

婴儿恶性萎缩性丘疹病一例

报道1例婴儿恶性萎缩性丘疹病.患儿女,8个月,出生后4天,躯干四肢反复发生红色丘疹,缓慢愈合后遗留瓷白色瘢痕,表面脱屑,周围绕以水肿性红晕.组织病理检查见表皮明显萎缩变薄,真皮中上部轻度水肿,少许黏蛋白沉积,局部附属器减少,未见明显血管病变及炎细胞浸润.因活动皮损少未予治疗,随访16个月仍有少量新发小皮损,无系统受累表现,是否属良性皮肤型仍需进一步随访.     

Papulosis maligna atrophicans Degos Nicht immer maligne!

A patient developed malignant atrophic papulosis with only cutaneous manifestation. Repeated coloscopy uncovered no malignant papules in the colon. Referring to the literature, the value of permanent anticoagulant therapy is discussed. In contrast to the term "malignant" atrophic papulosis, there also seems to be a variant with a benign clinical course.     

婴儿恶性萎缩性丘疹病一例

报道1例婴儿恶性萎缩性丘疹病.患儿女,8个月,出生后4天,躯干四肢反复发生红色丘疹,缓慢愈合后遗留瓷白色瘢痕,表面脱屑,周围绕以水肿性红晕.组织病理检查见表皮明显萎缩变薄,真皮中上部轻度水肿,少许黏蛋白沉积,局部附属器减少,未见明显血管病变及炎细胞浸润.因活动皮损少未予治疗,随访16个月仍有少量新发小皮损,无系统受累表现,是否属良性皮肤型仍需进一步随访.     

婴儿恶性萎缩性丘疹病一例

报道1例婴儿恶性萎缩性丘疹病.患儿女,8个月,出生后4天,躯干四肢反复发生红色丘疹,缓慢愈合后遗留瓷白色瘢痕,表面脱屑,周围绕以水肿性红晕.组织病理检查见表皮明显萎缩变薄,真皮中上部轻度水肿,少许黏蛋白沉积,局部附属器减少,未见明显血管病变及炎细胞浸润.因活动皮损少未予治疗,随访16个月仍有少量新发小皮损,无系统受累表现,是否属良性皮肤型仍需进一步随访.     

恶性萎缩性丘疹病一例

患者，女，58岁。躯干四肢散在红斑丘疹4个月余。皮肤科检查：躯干四肢散在红斑丘疹，丘疹中央可见瓷白色萎缩性斑片，边缘隆起伴红晕，伴腹痛。皮损组织病理示：表皮萎缩变薄，真皮浅中层胶原变性，血管周围散在小片状的淋巴细胞及组织细胞浸润。诊断：恶性萎缩性丘疹病。给予阿司匹林、双嘧达莫、雷贝拉唑钠、美沙拉嗪、阿嗪米特口服治疗1个月后腹痛症状缓解，全身皮疹颜色变淡。2个月后患者腹痛加重诊断为肠穿孔，术后无明显改善。4个月后去世。     

白色纤维性丘疹病

报告1例白色纤维性丘疹病。患者女，44岁。躯干四肢丘疹3年，无自觉症状。丘疹直径3-5mm，象牙白色或肤色，部分皮损萎缩。皮损组织病理检查显示真皮乳头层及网层上方胶原纤维增生，呈均质化改变。弹性纤维染色显示真皮浅层带状弱弹性纤维消失。     

Wegener's granulomatosis: a new entity in the growing differential diagnosis of Degos' disease

Wegener's granulomatosis (WG) is a multisystemic vasculitis, with skin involvement in 14% of cases and with palpable purpura, subcutaneous nodules and necrotic papules as the common features. ¹ We present a patient diagnosed with WG who had multiple whitish papules similar to those of malignant atrophic papulosis (Degos' disease), which appeared during a flare of his disease. Lesions of malignant atrophic papulosis are said to be pathognomonic; nevertheless, various diseases with similar clinical lesions have been described. To our knowledge, this is the first reported case of such lesions in a patient with WG, and we suggest WG should be included in the differential diagnosis of Degos' disease.     

累及神经系统的恶性萎缩性丘疹病

患者女,48岁,全身丘疹、斑块6年伴右眼视物不清1年就诊。体检：躯干、四肢可见瓷白色萎缩性丘疹,外周伴红晕及毛细血管扩张。右眼右侧斜视受限,视力减退,视野缺损。左手食指、拇指麻木,右侧胫骨前麻木。腹部皮损组织病理检查：真皮层坏死伴黏液样变性,深部数个小血管周围见淋巴细胞为主的炎细胞浸润,数个小血管腔闭塞。肠镜检查：全结肠散在片状充血、糜烂、浅溃疡形成;肌电图：股四头肌源性损害;粪隐血+++。诊断：恶性萎缩性丘疹病。治疗：口服阿司匹林、双嘧达莫治疗3个月,病情无进展。     

Maligne atrophische Papulose (Morbus Köhlmeier-Degos) Kein Ansprechen auf Interferon α-2a,Pentoxifyllin und Azetylsalizylsäure

A 45 year old female patient presented with the cutaneous manifestations of malignant atrophic papulosis (Köhlmeier-Degos disease) for two years. The typical papules with central porcelain-white atrophy correspond histologically to wedge-shaped necrosis of the connective tissue due to thrombotic occlusion of small vessels in the corium. The pathogenesis of malignant atrophic papulosis and effective treatment modalities are unknown. A slow virus infection has been suggested by some authors. Therefore, we attempted an immune therapy with interferon α-2a over a period of 11 months, but failed to cause a significant effect on the appearance and progression of the skin lesions. Furthermore, we could not confirm the effectiveness of a recently reported treatment modality with pentoxifylline and aspirin administered to our patient over a period of 5 months.     

The role of dermal blood vessels in the pathogenesis of malignant atrophic papulosis (Degos'' disease)

The role of dermal blood vessels in the histopathogenesis of malignant atrophic papulosis has been investigated in two patients using enzyme histochemical, tissue fibrinolysis and electron-microscopic techniques. In the earliest papules the normal enzymatic reactions of the endothelium and fibrinolytic activity of affected blood vessels were markedly impaired or absent. The endothelial cells were swollen and showed minor degenerative changes, but no cytoplasmic inclusions suggestive of virus particles could be detected. The results of intradermal skin tests and lymphocyte transformation cultures did not suggest the presence of any immunological defect. It is suggested that the skin lesions of malignant atrophic papulosis could be secondary to the slow occlusion of deeper arterioles.     

Malignant atrophic papulosis (K?hlmeier-Degos disease)

On the basis of 106 patients with malignant atrophic papulosis in the literature, including one case of our own, the clinical, histopathological and nosological features of this disease are reviewed. Histopathologically, the typical papules with central porcelain-white atrophy show broad wedge-shaped necrosis of the connective tissue due to thrombotic occlusions of small vessels in the corium. The disease leads to death in 50% of these cases, mostly due to involvement of the gut and the central nervous system. Malignant atrophic papulosis is the prototype of a disease, in which skin lesions are the primary sign of a systemic disorder demanding the interdisciplinary cooperation of various specialists.     

Fibroelastolytic papulosis: histopathologic confirmation of disease spectrum variants in a single case

Fibroelastolytic papulosis is a rare, acquired fibroelastolytic disorder that presents clinically as white‐to‐yellow papules and plaques most commonly occurring on the neck of elderly patients. The term fibroelastolytic papulosis encompasses two closely related conditions previously described as pseudoxanthoma elasticum‐like papillary dermal elastolysis (PDE) and white fibrous papulosis of the neck (WFPN). Here we present a case of a 78‐year‐old white female with a several‐year history of numerous, asymptomatic 2–3 mm yellowish, non‐follicular papules distributed symmetrically over the posterior neck, axillae, arm and antecubital fossae. Histopathologic examination revealed thickened and clumped elastotic fibers admixed with thick, sclerotic appearing collagen bundles in the mid and deep reticular dermis. Rare melanophages, loss of vertically oriented elastic fibers and scattered elastotic globes were noted in the papillary dermis. Based on the shared clinicopathologic features showed in this case, strong consideration should be made for the additional inclusion of papillary dermal elastosis as existing along the disease continuum of fibroelastolytic papulosis. This occurrence of fibroelastolytic papulosis shows unique histopathologic findings of pseudoxanthoma elasticum‐like PDE, papillary dermal elastosis and WFPN, further supporting the theory that these entities exist as variants along the fibroelastolytic papulosis spectrum.     

白色纤维性丘疹病1例

报告1例白色纤维性丘疹病.患者女,28岁,双上肢及上背部丘疹1年,既往鱼鳞病病史20年.皮肤科检查:双上肢及上背部散在白色丘疹,直径2～3 mm,瓷白色,未有融合倾向.皮损组织病理检查示表皮网篮状角化过度,真皮浅层血管周围少量淋巴组织细胞,真皮中上层胶原纤维束增粗.弹性纤维染色显示真皮浅层弹性纤维减少.胶原纤维及弹性纤...     

淋巴瘤样丘疹病1例

患者男,27岁。躯干、四肢反复发生暗红色丘疹、结节、坏死、结痂4月余,无痛,偶有瘙痒。皮肤科情况:躯干、臀部及四肢暗红色丘疹、结节,部分中央坏死、破溃,结黑褐色厚痂,留有萎缩性瘢痕或色素沉着。皮损组织病理示:真皮结节状淋巴及大组织样细胞浸润,可见多数异型细胞,约占30%,少数细胞呈双核或多核;免疫组化染色示:CD3(+),CD4(+),CD30(+),CD68(+),CD8个别(+),CD20个别(+),Ki-67约10%(+)。诊断:淋巴瘤样丘疹病。     

Papulosis maligna atrophicans (Köhlmeier-Degos) Diagnose, Therapie, Verlauf

A 71-year-old male patient presented with malignant atrophic papulosis (Köhlmeier-Degos disease). He developed multiple distinctive cutaneous lesions. The histopathological findings showed an obliterated arteriole with a wedge-shaped area of ulcerated and necrobiotic dermis. Laboratory tests were within normal limits or not specific. Although the involvement of the gastrointestinal tract and other organs has been noted in approximately 60% of the reported cases, in this patient so far the skin seems to be the only involved site. There is no proven effective therapy for Köhlmeier-Degos disease. In our case treatment with pentoxifylline and aspirin led to healing of all skin lesions within 3 months. One year after beginning treatment, the patient showed neither new cutaneous lesions, nor any signs of systemic involvement. The combination of pentoxifylline and aspirin should be considered when planning treatment strategies for malignant atrophic papulosis.     

Benign cutaneous Degos' disease

Malignant atrophic papulosis is a rare systemic vaso-occlusive disorder characterized by thrombosis of vessels of the dermis, gastrointestinal tract, central nervous system and, occasionally, other organs. Cutaneous lesions consist of erythematous, dome-shaped papules that develop a central area of necrosis to leave a porcelain-like scar. The most accepted theory of pathogenesis is based on endothelial cell damage. There is no effective treatment of the disease. We describe a 26-year-old man with Degos' disease, a diagnosis based on the clinical and histologic pattern of skin lesions. The good response to antiplatelet therapy and the absence of systemic involvement over 8 years' follow-up is noteworthy. We believe that this case represents the benign form of the disease, typically referred to as benign cutaneous Degos' disease.     

Nekrotische Knoten am unteren Abdomen

The case described in this paper shows that malignant atrophic papulosis can occur in connection with antiphospholipid syndrome (APS) and should be interpreted as a cutaneous manifestation of APS. In patients with clinically suspected malignant atrophic papulosis, it is therefore vital to conduct more comprehensive diagnostic procedures to rule out APS.     

Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis

Malignant atrophic papulosis (MAP), or Degos syndrome, is a rare disorder of unknown etiology. It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules. We report the case of a 42-year-old woman with a history of progressive systemic sclerosis who presented with painful subcutaneous nodules on her abdomen along with chronic atrophic papules on her upper and lower limbs. Biopsy results of both types of lesions revealed vascular thrombi without surrounding inflammation. We briefly review the literature on MAP and its association with various connective tissue diseases. To our knowledge, there have been no previous reports of a patient with the clinical and histologic presentations described here. Although the histologic appearance of the subcutaneous nodules was very similar to that of the atrophic papules, the clinical characteristics of the 2 types of lesions were strikingly different. It is fair to theorize that Degos lesions do not start as atrophic porcelain-white papules but rather evolve from a primary lesion. We hypothesize that these lesions start as painful red nodules and may represent part of the disease spectrum in the evolution of MAP.     

Atrophie blanche lesions closely resembling malignant atrophic papulosis (Degos' disease) in systemic lupus erythematosus

Two patients with systemic lupus erythematosus are described who in the course of their disease developed small atrophic blanche lesions that closely resembled those found in malignant atrophic papulosis. Preliminary investigation of these two cases indicates that considerable similarities probably exist in the pathogenesis of malignant atrophic papulosis and the atropic blanche lesions of systemic lupus erythematosus. It is concluded that a diagnosis of malignant atrophic Papulosis should only be made after systemic lupus erythematosus has been excluded by full investigation.