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www.asianpedderm.com a publication of ASIAN JOURNAL OF PEDIATRIC DERMATOLOGY AJPD Vol. 3/2021 InfantileInfantile OnsetOnset SystemicSystemic DegosDegos DiseaseDisease Abstract Case Report Discussion Reference Acknowledgements a publication of the ASIAN JOURNAL OF PEDIATRIC DERMATOLOGY AJPD Vol. 3/2021 Infantile Onset Systemic Degos Disease Henrietta Albela1, Sabeera Begum1, Siti Nuraihan Nordin1, Wai Quen Lee1, Lalitha Pillay Gopinathan1, Kin Fon Leong1 1Women & Children Hospital Kuala Lumpur, Malaysia ABSTRACT Degos disease, also known as malignant atrophic papulosis (MAP), is a rare CORRESPONDING AUTHOR disorder that has classical skin signs of papular lesions with central porcelain-white Henrietta Albela Women & Children Hospital Kuala atrophy and telangiectatic rim. Cutaneous variant of Degos Disease involves the Lumpur, Pediatric Dermatology Unit, skin alone whereas the systemic type has multi-organ involvement, most commonly Department of Paediatrics gastrointestinal tract and neurological systems. This disease is exceedingly rare in Jalan Pahang, Kuala Lumpur, 1 Malaysia 50586 children with less than 40 reported cases to date. We report a case of 14 month old Email: [email protected] Malay girl with systemic Degos Disease whose pathognomonic skin lesions first appeared since 5 months of age. To best of our knowledge, this is the first reported case in Malaysia. CASE REPORT Several skin Biopsies were performed which showed superficial A 14-month old Malay girl presented with recurrent episodes of vessels vasculopathy. Luminal thrombosis with fibrinoid necrosis was fever, Billous vomiting, aBdominal distension and diarrhea over a demonstrated in small and medium vessels. No evidence of period of 5 weeks. She was treated as infective gastroenteritis with leukocytoclastic vasculitis was noted. Immunofluorescence staining intravenous fluids and antibiotics. However, her condition for IgG, IgA, IgM, C2 and C1q was negative. Clinicopathological deteriorated and she went into septic shock. On retrospective correlation led us to the diagnosis of systemic Degos Disease. questioning, she had multiple papules which progressed to ulcers since 5 months of age and gradually increasing in numBers. However, her condition rapidly deteriorated and she developed multiple episodes of focal seizures due to worsening suBdural On examination she had multiple erythematous papules and discrete effusion, requiring extra ventricular drainage. Magnetic resonance porcelain-white atrophic ulcers with erythematous rim (Figure 1) angiography/venography Brain was normal. She soon developed over her trunk and limbs. Her abdomen was distended with bowel perforation which needed repeated laparotomies. hepatosplenomegaly and no neurological signs detected on Intraoperatively, there were multiple punctate perforation with admission. thromBosis of small vessels and Bowel thinning. In view of worsening intracranial Bleeding resulting in deteriorating neurological function Figure 1: Clinical Findings Multiple erythematous papules and discrete porcelain-white atrophic ulcers with and multiple Bowel perforations which were not amendable to erythematous rim on feet (Panel A) and abdomen (Panel B) surgery; a multidisciplinary team joint meeting was done together with the parents and decided for withdrawal of treatment. Figure 2: Histopathological findings in the skin A: Intraluminal thrombosis (arrow) in superficial vessels of the skin with epidermal necrosis (hematoxylin-eosin, original magnification x200) B: At a higher magnification of one of the vessels showed luminal occlusion and fibrinoid necrosis. (hematoxylin-eosin, original magnification x400) Full-body computed tomography (CT) was performed in view of suspected disseminated tuBerculosis (TB) revealed Bilateral suBdural collections, Bilateral pleural effusion and generalized circumferential small Bowel thickening with suBcentimer nodes. Her Blood, urine and cereBrospinal fluid (CSF) cultures were all negative, including TB culture. Connective tissue disease screening, primary immunodeficiency and metabolic diseases workup were normal. AJPD Vol. 3/2021 Page 2 DISCUSSION As the morphology of the lesions progress, the classical central Degos disease is an extremely rare thrombotic vasculopathy leading porcelain-white atrophy appears and shows more prominent interface to multiple infarcts of skin and viscera. ABout 200 cases of MAP dermatitis with classical histopathological findings of thrombotic have been reported worldwide. The average age of disease onset is occlusion of small arteries resulting in an inverted wedge-shape 35.4 years and less than 40 cases involved children or adolescents.1 ischaemic zone in the dermis. 5 In a review of case reports of paediatric Degos Disease, aBout 58% had a fatal course, with death occurring on average 3.6 years after The treatment option for Degos Disease include antiplatelet agents, presentation.2 anticoagulants and immunosuppressants without much effectiveness. Recent reports have shown satisfactory responses to eculizumaB (C5 6 The classical skin signs of papular lesions with central porcelain- inihibitor) and treprostinil (prostacyclin analogue), however, these white atrophy and telangiectatic rim is almost pathognomonic and treatment modalities were not availaBle to our patient at that time. defined the onset of the disease. Systemic complications were present in 29% of patients with gastrointestinal system being the REFERENCE commonest involved, followed By central nervous system. Systemic 1. Theodoridis A, Konstantinidou A, Makrantonaki E, ZouBoulis disease carries a poorer prognosis with a mortality rate of CC. Malignant and Benign forms of atrophic papulosis approximately 21% as compared to cutaneous disease alone.3 In our (Köhlmeier-Degos disease): systemic involvement determines patient, she first developed the classical skin lesions about 9 months the prognosis. Br J Dermatol. 2014;170(1):110–115. prior to her gastrointestinal and neurological signs. Unfortunately, 2. Wilson J, Walling HW, Stone MS. Benign cutaneous Degos’ our patient succumbed within 2 months of developing systemic disease in a 16-year-old girl. Pediatr Dermatol. 2007;24:18-24. complications. 3. Güven FO, Bozdag˘ KE, Ermete M, Karaman A. Degos’ Disease. Int J Dermatol. 2000;39:361-362. The exact pathogenesis is still unknown, But it has Been suggested 4. Magro CM, Poe JC, Kim C, et al. Degos disease: a C5B- by Magro et al that the distinct vascular injury pattern seen in Degos 9/interferon-α-mediated endotheliopathy syndrome. Am J Clin Disease involves complement component C5B–9 complex Pathol. 2011;135(4):599–610. deposition and dysregulated interferon-α expression.4 5. Harvell JD, Williford PL, White WL. Benign cutaneous Degos' disease: a case report with emphasis on histopathology as papules Diagnosis of Degos Disease is usually made Based on chronologically evolve. Am J Dermatopathol. 2001 histopathology with varied findings in different stages of the skin Apr;23(2):116-23. 5 lesions. Early papular lesions showed superficial and deep 6. Huang YC, Wang JD, Lee FY, Fu LS. Pediatric Malignant perivascular, perineural and periadnexal chronic inflammatory cell Atrophic Papulosis. Pediatrics. 2018 Apr;141(Suppl 5):S481- infiltrate with interstitial mucin deposition, suBtle interface S484. dermatitis and relatively normal epidermis. ACKNOWLEDGEMENTS Author Contributions Dr(s) Zuliatul Faizah Baharom, Henrietta Albela, Sabeera Begum, Siti Nuraihan Nordin, Wai Quen Lee, Lalitha Pillay Gopinathan and Kin Fon Leong had Full access to all of the data in the study and take responsibility of the integrity of the data and the accuracy of the data analysis. Obtained Funding: None. Declaration oF ConFlict oF Interest: None. Financial Disclosure: None reported All Financial Interests (including pharmaceutical and device product(s): None. .
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