Case report Blackwell Oxford, International IJD © 0011-9059 XXX 2007 The UK Publishing International Journal Ltd of Dermatology Society of Dermatology A case of Degos disease with pleuropericardial fibrosis, jejunal perforation, hemiparesis, and widespread cutaneous lesions A case of systemic Shahshahani et al. Degos disease with rare manifestations Mostafa Mirshams Shahshahani, MD, Pantea Hashemi, MD, Reza Nemati, MD, Azita Nikoo, MD, Hamed Mazoochy, MD, and Armin Rashidi, MD From the Department of Dermatology, Tehran University of Medical Sciences, Tehran, Iran Correspondence Armin Rashidi, MD No. 12 Aramesh Alley Mellat Park Tehran Iran E-mail: rasidiarmin@yahoo.com Introduction Malignant atrophic papulosis, or Degos disease, is a rare thrombo-occlusive disease of uncertain etiology, with fewer than 200 cases reported so far.1,2 Two distinct forms have been described for Degos disease. The systemic or malignant form, which comprises the majority of cases and is usually fatal within 2–3 years from the onset of systemic involvement, is characterized by multiple infarcts in the skin and internal organs, especially the gastrointestinal tract and nervous system.3 The cutaneous or benign variant has characteristic cutaneous lesions without evidence of systemic disease during a prolonged period of observation.4 We report a case of Degos disease presenting with acute abdomen, hemiparesis, and skin involvement on the genitalia, palms, and soles. Case Report A 47-year-old man was referred to our clinic with a 7-year history of an asymptomatic erythematous papular eruption on the trunk and extremities. The lesions developed as several pink, dome-shaped papules (about 5 mm in size) on the trunks and arms, became established with marginal edema and sometimes a peripheral telangiectatic rim, and gradually healed within a few months, leaving behind a small porcelain-white central atrophic scar. Gradually, the palms, soles, and shaft of the penis were affected by similar lesions (Fig. 1). The patient was never free of lesions during these 7 years, and the lesions were resistant to various local and systemic treatments. The patient recently experienced an episode of acute abdominal pain, leading to emergency laparotomy with a © 2008 The International Society of Dermatology final diagnosis of jejunal perforation. The resected segment of the jejunum was sent for pathologic evaluation. He had also recently experienced an ischemic cerebral vascular accident, with residual left-sided hemiparesis at the time of physical examination in our clinic. He was a 30-pack a year smoker with a previous history of imprisonment for 3 years. His past medical history, drug history, and general physical examination were otherwise normal. A skin purified protein derivative (PPD) test was negative. Laboratory tests showed a normal complete blood cell count, erythrocyte sedimentation rate, routine blood chemistry analysis, immunoglobulins, CH50, complement levels, and antinuclear antibody and antiphospholipid antibody titer. A serologic test for human immunodeficiency virus (HIV) was negative. Magnetic resonance imaging (MRI) of the brain revealed small vessel disease in both cerebral hemispheres, with an ischemic process in the right side of the pons, without abnormal tissue enhancement (Fig. 1). Spinal MRI was normal. Histopathologic examination of the resected jejunal segment showed subendothelial fibrous thickening of the wall of medium-sized arteries with no evidence of thrombosis or endothelial proliferation. Examination of an ellipse biopsy from a lesion on the trunk revealed an atrophic and ulcerated epidermis. There was an inverted wedge-shaped area of sclerosis in the underlying dermis (with the apex in the deep reticular dermis) with mucin deposition in the periphery. Focal perivascular lymphocytic infiltration was observed in the dermis (Fig. 1). The diagnosis of systemic Degos disease was made and the patient was given antiplatelet, anticoagulant, and pentoxifylline therapy. Nevertheless, his general condition International Journal of Dermatology 2008, 47, 493–495 493 494 Case report A case of systemic Degos disease with rare manifestations Shahshahani et al. Figure 1 Top left: brain magnetic resonance image showing the ischemic lesion in the right pons. Top middle: dermatoscopy showing a papule with central porcelain-white pallor and a telangiectatic border. Top right: a typical inverted wedge-shaped dermal sclerosis with an ulcerated atrophic epidermis (hematoxylin and eosin, ×10). Bottom left: plantar involvement. Bottom middle: palmar involvement. Bottom right: small vessel vasculopathy (hematoxylin and eosin, ×40) worsened gradually and he died of severe cardiopulmonary failure a few months later. Autopsy revealed severe diffuse fibrotic thickening of the pericardium and pleura. Discussion Amongst the suggested mechanisms for Degos disease, abnormality in the clotting system of blood seems most likely.5,6 Nevertheless, the pathophysiology of the disease still remains largely unknown. Systemic manifestations usually develop from weeks to years after the onset of skin lesions. The gastrointestinal tract and central nervous system are affected in 50% and 20% of cases, respectively. Intestinal perforation is the most severe complication and the most common cause of death in systemic Degos disease. Our case was unique in that cardiopulmonary involvement has only rarely been described in Degos disease.7 The eruption of Degos disease develops slowly and is usually mostly asymptomatic, but may be accompanied by a slight burning sensation. The eruption consists of erythematous, dome-shaped papules, 2–15 mm in size, that develop a central area of necrosis to leave a porcelain-like scar surrounded by an erythematous ring.8 The lesions are not confluent and appear in a variable number on the trunk and the proximal portions of the extremities.9 They may also occur on the penis. The face, palms, and soles are usually spared.10 To our knowledge, only two cases of palmoplantar involvement have been reported so far.8,11 International Journal of Dermatology 2008, 47, 493–495 No successful medical therapy for Degos disease is known. Anticoagulants, antiplatelet therapy, and stimulators of fibrinolysis have been used, with some improvement in visceral lesions.5,12–14 Nevertheless, no treatment has been able to change the fatal outcome of systemic Degos disease. In conclusion, we report a case of fatal systemic Degos disease with two extremely rare manifestations, i.e. cardiopulmonary and palmoplantar involvement. References 1 Degos R, Delort J, Tricot R. Dermatite papule-squameuse atrophiante. Bull Soc Fr Dermatol Syphiligr 1942; 49: 148–150. 2 Loewe R, Palatin M, Petzelbauer P. Degos disease with an inconspicuous clinical course. J Eur Acad Dermatol Venereol 2005; 19: 477–480. 3 Degos R. Malignant atrophic papulosis. Br J Dermatol 1979; 100: 21–35. 4 Zamiri M, Jarrett P, Snow J. Benign cutaneous Degos disease. Int J Dermatol 2005; 44: 654–656. 5 Vázquez-Doval FJ, Ruiz de Erechun F, Páramo JA, et al. Malignant atrophic papulosis. A report of two cases with altered fibrinolysis and platelet function. Clin Exp Dermatol 1993; 18: 441–444. 6 Black MM, Nishioka K, Levene GM. The role of dermal blood vessels in the pathogenesis of malignant atrophic papulosis (Degos’ disease). Br J Dermatol 1973; 88: 213– 219. © 2008 The International Society of Dermatology Shahshahani et al. A case of systemic Degos disease with rare manifestations Case report 7 Mauad T, De Fatima Lopes Calvo Tiberio I, Baba E, et al. Malignant atrophic papulosis (Degos’ disease) with extensive cardiopulmonary involvement. Histopathology 1996; 28: 84–86. 8 Herrera Saval A, Pedregal González Villanueva L. Gangrena de Fournier como complicación terminal de la papulosis atrófica maligna (enfermedad de Degos). Med Cut ILA 1993; XXI: 218–223. 9 Ryan TJ. Malignant atrophic papulosis. In: Champion RH, Burton JL, Ebling FJG, eds. Textbook of Dermatology, 5th edn. Oxford: Blackwell Scientific Publications, 1992: 1951– 1953. 10 Scheinfeld N. Degos’ disease. In: Emedicine Dermatology Book. URL http://www.emedicine.com/derm/topic931.htm [accessed on 20 November 2006]. 11 Ojeda Cuchillero RM, Sanchez Regana M, Umbert Millet P. Benign cutaneous Degos’ disease. Clin Exp Dermatol 2003; 28: 145–147. 12 Daniel F, Parigny P. Papulose atrophiante maligne. In: Dubertret L, ed. Thérapeutique Dermatologique, 1st edn. Paris: Flammarion Médecine-Sciences, 1992: 425–428. 13 Stahl D, Thomsen K, Hou-Jensen K. Malignant atrophic papulosis. Treatment with aspirin and dipyridamole. Arch Dermatol 1978; 114: 1687–1689. 14 Assier-Bonnet E, Chosidow O, Frances C. La maladie de Degos. Ann Dermatol Venereol 1997; 124: 273–279. © 2008 The International Society of Dermatology International Journal of Dermatology 2008, 47, 493–495 495