Systemic Dego`S Disease. Our Approach to the Diagnosis and the Follow-Up

Albanian Journal of Trauma and Emergency Surgery (2020) 4/2: 724 - 726 DOI: 10.32391/ajtes.v4i2.95 CASE REPORTS

Systemic Dego`s Disease. Our Approach to the Diagnosis and the Follow-Up. A Case reports

Fadil GRADICA1*, Daniela XHEMALAJ1

Received: 5 February 2020 / Accepted: 15 March 2020 / Published online: 20 July 2020 © The Author(s) 2020. This article is published with open access at https://journal.astes.org.al

Abstract Degos’ disease, also known as “malignant atrophic papulosis” is a rare vasculopathy characterized by typical cutaneous lesions with an unknown etiology which was first described by Dego in 1942 (1), but another case, reported in 1941 by Köhlmeier, who interpreted it as thromboangiitis obliterans of the mesenteric vessels (2). It is an occlusive arteriopathy involving small-caliber vessels. Specifically, it is a progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. Degos disease occurs both in a limited benign, cutaneous form and in a potentially lethal multiorgan, systemic variant. The disease has a male predominance (3:1), and sporadic cases of familial involvement have been recorded. (3-7). The involvement of the gastrointestinal tract and other organs has been noted in approximately 60% of reported cases (8). There are fewer than 50 living patients presently known worldwide, and fewer than 200 reported in medical literature. However, many individuals may go undiagnosed due to rarity of the disease (9,10). Most individuals develop symptoms between the ages of 20-50; however, cases outside of this age range have been reported as well, even as early as 8 months (1,6). Key words: Degos’ disease, malignant atrophic papulosis, pleural effusion.

Case report:

A 58 -year-old man was presented to the hospital with productive cough, dyspnea, chest wall pain, and in chest x-ray examination was observed left pleural effusion. Five months ago, the patient was diagnosed Case report withritic Dego ands disease painless, which was which manifested were by more wide-spread than 50 remittent buteruptions located of small prinpapules- with whitecipally centers on sur rounded trunk by antero-posteriorerythematous telangiectatic position borders, 5-8 ,thatmm in diameter,started nonpruritic one and painless,year ago which without were wide-spread the butcomplete located principally disappearance on trunk antero -poofsterior older position ones ,that started A 58 -year-old man was presented to the hospital with one year ago without the complete disappearance of older ones (Figure 1, 2) The soles, mucosae and face productive cough, dyspnea, chest wall pain, and in chest were(Figure not affected. 1, 2) The soles, mucosae and face were not affected. x-ray examination was observed left pleural effusion. Five The patient patient was also was taking also medication taking for hypertension. medication for hypertension. months ago, the patient was diagnosed with Degos disease which was manifested by more than 50 remittent eruptions of small papules with white centers surrounded by erythem- atous telangiectatic borders, 5-8 mm in diameter, nonpru-

Original article, no submission or publication in advance or in parallel

* Corresponding author: Asc. Prof. Dr. Fadil GRADICA MD, PhD FigureFigure 1: Skin 1: photograph Skin photographshowing white to pink showing papules, 5–10mm whitein diameter, to withpink central, papules, porcelain-white * [email protected] atrophic center surrounded by a peripheral telangiectatic rim 5–10mm in diameter, with central, porcelain-white atrophic 1 University Hospital “Shefqet Ndroqi” Tirana, ALBANIA. center surrounded by a peripheral telangiectatic rim

Discussion

Degos’ disease, also known as malignant atrophic papulosis (MAP), is a rare condition with reportedly stereotypical skin lesions on the trunk and proximal portion of the extremities, but the palms, soles and face tend to be spared, as in our case, consisting of largely asymptomatic, porcelain-white, atrophic papules, with surrounding erythema and telangiec- tasias. The disease has been reported to be purely cutaneous in 37% of patients (11). Degos disease occurs both in a limited benign, cutaneous form and in a potentially lethal multiorgan, systemic variant (12,13). The disease is characterized by endothelial prolifera- FigureFigure 2 2Histopathology Histopathology 2 Histopathology of of the the skin skin biopsy biopsy byof byhematoxylin the hematoxylin skin and andbiopsy eosin eosin staining: staining: by Epidermalhematoxylin Epidermal atrophy, atrophy, hyperkeratosis, and hyperkeratosis, tion and swelling of small and medium-sized vessels. andandeosin wedge wedge -shapedstaining:-shaped dermo dermo epidermal Epidermalepidermal necrosis necrosis wereatrophy, were visible visible the thehyperkeratosis, panels panels demonstrated demonstrated occlusion occlusionand of wedge- blood of blood vessels vessels in the in the coriumcorium and and perivascular perivascular lymphocytic lymphocytic infiltration. infiltration. The etiology of the disease remains controversial. shaped dermo epidermal necrosis were visible the panels demon- Some believe that Degos’ disease is a thrombotic rather than DuringDuringstrated the the hospitalization occlusionhospitalizationwere ofwere bloodperformed performed vessels a series a series of in examinationof the examination corium to find to findand the thecause perivascular cause of pleural of pleural fluid. fluid.A A therapeutictherapeutic thoracentesis thoracentesis was was performed performed and and 1L 1L of greenof green exudative exudative fluid fluid was was removed removed with with pH 7.26,pH 7.26,an autoimmune disease, because no circulating immune lymphocytic infiltration. WBCWBC 260 260 cells/mm3 cells/mm3 (PMN (PMN 16%), 16%), LDH LDH 746 746 IU/L, IU/L,triglyceridestriglycerides 30 mg/dL, 30 mg/dL, and andtotal total protein protein 5.2 g/dL,5.2 g/dL,complexes, anti-endothelial cell antibodies, or anticardio- cholesterolcholesterol 60mg⁄dl, 60mg⁄dl, neutrophils neutrophils3%,3%,lymphocyteslymphocytes 27 27%, %, macrophages macrophages70 %.70 G%.-expert G-expert was was negative negative for for TBTB infection. infection. lipin antibodies are isolated, even there a a few cases, where

OtherOther analyses analysesDuringresultsresults were:the were: erythrocyteshospitalizationerythrocytes sedimentation sedimentation were 7, fibrinogen7, fibrinogenperformed 442, 442, PT 13.5,PT a 13.5, PT⁄INR1,seriesPT⁄INR1, APTTof APTT 28.5, antiphospholipid28.5, antibodies are identified. CRPCRPexamination 2.9. 2.9. to find the cause of pleural fluid. A therapeutic The internal organs most commonly involved in the or- HepaticHepaticthoracentesis panel panel w aswas normal, normal, was mineral mineral performed panel panel normal, normal, andLDHLDH 230,1L 230, BNPofBNP green 54, 54,totaltotal proteinexudative protein 7.9, 7.9, RPR RPR nonactive,flu nonactive,- der of decreasing frequency are the GI tract, CNS, thoracic urine analysis wit microscopy was normal. urineid was analysis removed wit microscopy with was pHnormal. 7.26, WBC 260 cells/mm3 (PMN organs and kidney. In the condition of the persistence of pleural fluid even with the wide specter of antibiotic therapy was In the condition of the persistence of pleural fluid even with the wide specter of antibiotic therapy was The involvement of the gastrointestinal tract and other decided16%),, in the LDH multidisciplinary 746 IU/L,team triglycerides for diagnostic and therapeutic 30 mg/dL, VATS (Videoand totalAssisted pro - decided, in the multidisciplinary team for diagnostic and therapeutic VATS (Video Assisted Thoracoscopy). organs has been noted in approximately 60% of reported Thoracoscopy).tein 5.2 g/dL, cholesterol 60mg⁄dl, neutrophils 3%, lympho- Withcytes lidocaine 27 2% %,- 40 macrophages ml diluted, in 4-5-th space,70 %. linea G-expert axillaries anterior was and negativemedia, we opened for pleural cases (3). With lidocaine 2%- 40 ml diluted, in 4-5-th space, linea axillaries anterior and media, we opened pleural cavity, aspirated the pleural fluid and taken some pieces from parietal pleura for histopathological cavity,TB infection. aspirated the pleural fluid and taken some pieces from parietal pleura for histopathological The lesions affect the bowel in approximately 47% examination. examination.Other analyses results were: erythrocytes sedimenta- of cases. Intestinal symptoms are variable, although any tion 7, fibrinogen 442, PT 13.5, PT⁄INR1, APTT 28.5, CRP portion of the intestinal system (from the oral cavity to the 2.9. anus) may be involved, and the small bowel is predominant- Hepatic panel was normal, mineral panel normal, LDH ly affected (9). 230, BNP 54, total protein 7.9, RPR nonactive, urine analy- The course of the disease may be as long as 20 years sis wit microscopy was normal. but once intestinal lesions, especially perforation have ap- In the condition of the persistence of pleural fluid even peared, death usually occurs within a few months (6). with the wide specter of antibiotic therapy was decided, Neurologic manifestations occur in around 19 % of pain the multidisciplinary team for diagnostic and therapeutic tients, including cerebral hemorrhage, subdural hematoma, VATS (Video Assisted Thoracoscopy). thrombosis of cerebral arteries, venous sinus thrombosis, With lidocaine 2%- 40 ml diluted, in 4-5-th space, linea encephalitis, meningitis, polyradiculoneuropathy, cranial axillaries anterior and media, we opened pleural cavity, as- neuropathy, and myopathy (14,15). pirated the pleural fluid and taken some pieces from parietal There have been very few published reports of cardio- pleura for histopathological examination. pulmonary and pleural complications related to DD. The patients were in good condition after VATS with There are very few reported cases of pleuritis and peri- medication. Pleural biopsy resulted with fibrosis and chron- carditis, most diagnosed as incidental findings at autopsy. ic inflammation perivascular lymphocytic mononuclearis, Another case by Notash et al. mentioned that severe restric- no granulomatous process, wich reveals a systemic Dego tive cardiopulmonary insufficiency led to death of a patient disease. with Degos disease. Another case with cardiopulmonary in- Three months later the patient came again to the hos- volvement was reported in 1996. An infant case of Degos pital with pleural fluid and a drainage and medication was disease in which the patient died due to disseminated myo- done. cardial infarct was presented by Cabre et al. Also, two cases of constrictive pericarditis have been described. The other case, described by Pierce and Smith, died due to heart failure and respiratory disorder three months

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Autoimmune results support the theory that Degos disease is an endothe- pancreatitis in MRL/Mp mice is a T-cell-mediated disease lial disease (16,17). responsive to cyclosporine A and rapamycin treatment. Gut. In conclusion, the features of our case highlight the 2014;63(3):494–505. importance of considering systemic Degos disease in case 10. Moulin G. Les formes benignes de la papulose atrophiante maligna de Degos. [Benign forms of Degos’ malignant atro- of pleural effusion especially when there is skin involve- phic papulosis] Ann Dermatol Venereol. 1988; 115: 1289– ment. 1290. Skin biopsy is essential for the diagnosis of this entity. 11. Burg G, Vieluf D, Stolz W, et al. Malignant atrophic papulo- Platelet antiaggregant as well as anticoagulants seemed ef- sis. Hautarzt. 1989; 40: 480–485. fective to the control of the disease. 12. Magrinat G, Kerwin KS, Gabriel DA. The clinical manifesta- COI Statement: This paper has not been submitted tions of Degos’ syndrome. 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J Child Neurol. 1988; 3: 42–46 Disclosure: The authors declared no conflict of inter- 16. Scheinfeld N. Commentary on ‘Degos disease: a C5b-9/in- est. No funding was received for this study. terferon-α-mediated endotheliopathy syndrome’ by Magro et al: a reconsideration of Degos disease as hematologic or endothelial genetic disease. Dermatol Online J. 2011;17(8):6. 17. Meephansan J, Komine S, Hosoda S, et al. Possible involvement of SDF-1/CXCL 12 in the pathogenesis of Degos disease. J Am Acad Dermatol. 2013;68(1):138–143.