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Table of Contents
CORRESPONDENCE
Year : 2019  |  Volume : 37  |  Issue : 2  |  Page : 108-109

A rare case of degos disease presenting with severe spinal impairment


1 Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China
2 Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China

Date of Web Publication18-Jun-2019

Correspondence Address:
Dr. Xinyue Qin
Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Yuzhong District, Chongqing 400010
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ds.ds_20_18

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How to cite this article:
Wu Q, Yu G, Shan K, Qin X. A rare case of degos disease presenting with severe spinal impairment. Dermatol Sin 2019;37:108-9

How to cite this URL:
Wu Q, Yu G, Shan K, Qin X. A rare case of degos disease presenting with severe spinal impairment. Dermatol Sin [serial online] 2019 [cited 2019 Sep 15];37:108-9. Available from: http://www.dermsinica.org/text.asp?2019/37/2/108/259855



Dear Editor,

Degos disease is a rare, chronic, thrombo-obliterative vasculopathy involving small-and medium-sized arteries and affecting multiple organ systems. Neurologic manifestations occur in around 20% of patients, including cerebral hemorrhage, subdural hematoma, thrombosis of cerebral arteries, venous sinus thrombosis, encephalitis, meningitis, polyradiculoneuropathy, cranial neuropathy, and myopathy.[1],[2] However, there have been few reports documenting spinal involvement. We herein present a case of Degos disease with severe ischemic myelopathy.

A 20-year-old female with unremarkable family history and no history of drug abuse presented with multiple papules on both legs since June 2014. The skin lesions were asymptomatic but rapidly proliferated to trunk and upper extremities over a course of one year. In mid-April 2016, she developed sudden onset of weakness and sensory disturbance in the right leg with dysuria and dyschesia. Since then, the symptoms lasted without any deterioration.

About 5months later, the patient suddenly developed significant lower back pain, which rapidly progressed to an acute worsening of sensory and motor function disturbances in both lower extremities. On spinal magnetic resonance imaging(MRI), T2-weighted high-intensity signals at the level of medullary cone(T12-L1) were confirmed. Yet, the patient was pregnant for 6months. She refused further treatment and was discharged home. One week later, she had a similar episode of lower back pain, followed by a complete loss of sensory and motor function of legs as well as bladder and intestinal continence. On admission, physical examination revealed multiple asymptomatic white to pink papules(5–10mm in diameter with a central, porcelain-white atrophic center surrounded by an erythematous rim) on the trunk and proximal extremities[Figure1]a and [Figure1]b, which spared the scalp, genitals, and palmoplantar regions. Neurological examination showed paralysis and sensory disturbance below T4. On serological examinations, the coagulation test demonstrated abnormal fibrinogen(4.26g/l) and D-Dimer levels(1.41mg/l). Besides, increased platelet account(730*109/l) was noted. Venereal disease research laboratory test and the fluorescent treponemal antibody absorption test were negative. In cerebrospinal fluid, lymphocyte count was 16/μl, and there was a markedly raised protein level(2.69g/l). Oligoclonal bands and NMO-IgG were negative. On neuroimaging exams, spinal MRI revealed T2-weighted high-intensity signals between T3 and T4, T6 and T8, as well as T12 and L1[Figure2]a, [Figure2]b, [Figure2]c. However, gadolinium enhancement was not observed on these lesions as expected. Only MRI of cauda equine showed mild enhanced T1-weighted signals after intravenous meglumine gadopentetate infusion[Figure2]d. Meanwhile, we noticed the skin eruptions and carried out a biopsy of lesions, which showed epidermal atrophy, wedge-shaped dermoepidermal necrosis, arteriolar obliteration in the corium and perivascular lymphomonocitic infiltration[Figure1]c, [Figure1]d, [Figure1]e, [Figure1]f. These findings led to the diagnosis of Degos disease, and her central nervous system(CNS) manifestations were considered as a result of this rare disease. She was subsequently prescribed anticoagulated and antiplatelet therapy. Her condition stayed stable. Until now, she has no further deterioration.
Figure1: Skin photograph showing white to pink papules, 5–10mm in diameter, with central, porcelain-white atrophic center surrounded by a peripheral telangiectatic rim(a and b). Histopathology of the skin biopsies, visualized by hematoxylin and eosin staining(c-f). Epidermal atrophy, hyperkeratosis, and wedge-shaped dermoepidermal necrosis were visible(×40, ×100)(c and d); the panels demonstrated occlusion of blood vessels in the corium and perivascular lymphocytic infiltration(×100, ×200)(e and f)

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Figure2: Spinal MRI scan showed T2-weighted high-intensity lesions between T3 and T4(a), T6 and T8, as well as T12 and L1(b) with segmental and patchy distribution on sagittal images; T2-weighted axial image showed high-intensity signals with a moth-eaten appearance in the periphery of the spinal cord(c); T1-weighted image showed subtle enhancement in the area of cauda equine after intravenous meglumine gadopentetate infusion(d)

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Degos disease is a rare and life-threatening disease, especially when associated with gastrointestinal tract or CNS. It is characterized by papular skin lesions with central porcelain-white atrophy and surrounding telangiectatic rim.[3] The cutaneous clinical picture is almost pathognomonic. The histology in most cases shows a wedge-shaped connective tissue necrosis in the corium due to a thrombotic occlusion of the small arteries.[4] Systemic symptoms may develop suddenly, either occurring simultaneously or subsequently, even before skin manifestations. In our case, systemic manifestations developed 2years after the occurrence of skin lesions leading to ischemic myelopathy. Myelitis was first suspected. However, the patient did not respond to steroid treatment. Moreover, increased circulating levels of fibrinogen and platelet account were noticed, and on MRI, patchy lesions from periphery to center of the spinal cord, a characteristic of Degos disease[5] was noted, indicating ischemic changes of spinal cord related to a possible disseminated occlusive vasculopathy. To the best of our knowledge, only two cases of Degos disease involving spinal cord damage were reported. Occlusive vasculopathy and diffuse myelomalacia or ischemic myelopathy were discovered at autopsy.[5],[6]

In conclusion, the features of our case highlight the importance of considering Degos disease in case of spinal vasculopathy especially when there is skin involvement. Skin biopsy is essential for the diagnosis of this entity. Platelet antiaggregants as well as anticoagulants seemed effective to the control of the disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
PirollaE, FregniF, MiuraIK, MisiaraAC, AlmeidaF, ZanoniE, etal. Degos disease–Malignant atrophic papulosis or cutaneointestinal lethal syndrome: Rarity of the disease. Clin Exp Gastroenterol 2015;8:141-7.  Back to cited text no. 1
    
2.
NouhA, SpeiserJ, BillerJ. Acquired neurocutaneous disorders. Handb Clin Neurol 2015;132:29-73.  Back to cited text no. 2
    
3.
WallaceMP, ThomasJM, MeligonisG, HaT. Systemic lupus erythematosus, following prodromal idiopathic thrombocytopenic purpura, presenting with skin lesions resembling malignant atrophic papulosis. Clin Exp Dermatol 2017;42:774-6.  Back to cited text no. 3
    
4.
ZouboulisCC, TheodoridisA, BrunnerM, MagroCM. Benign atrophic papulosis(Köhlmeier-Degos disease): The wedge-shaped dermal necrosis can resolve with time. JEur Acad Dermatol Venereol 2017;31:1753-6.  Back to cited text no. 4
    
5.
MatsuuraF, MakinoK, FukushimaT, MatsubaraN, ShibuyaM, HiguchiT, etal. Optic nerve and spinal cord manifestations of malignant atrophic papulosis(Degos disease). JNeurol Neurosurg Psychiatry 2006;77:260-2.  Back to cited text no. 5
    
6.
McFarlandHR, WoodWG, DrownsBV, MenesesAC. Papulosis atrophicans maligna(Köhlmeier-Degos disease): Adisseminated occlusive vasculopathy. Ann Neurol 1978;3:388-92.  Back to cited text no. 6
    


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