INTRODUCTION

Degos disease is a sporadic condition whose pathophysiology results in widespread blockage of small and medium-sized veins and arteries, which leads to impaired blood flow to and from organs perfused and drained by the affected blood vessels [1]. It commonly occurs in white male teenagers. Patients suffering from Degos disease initially present with trunk skin papules. Additionally, since the disease entails the systemic obstruction of small and medium-sized veins and arteries, it might affect other organs such as the kidneys, liver, and small intestine. This might result in acute renal failure, generalized abdominal pain, and fever due to the perforation of the small intestine, and peritonitis. Its etiology is unknown. However, various theories have been developed to explain it, and this includes theories on autoimmunity, vasculitis, coagulation defects, endothelial cell defects, and viral infections. Many researchers claim that the statistics on Degos disease are inaccurate as it is massively underdiagnosed. A lot of attention has been given to the systemic form of Degos disease, yet some people only develop skin papules. Most people who only develop skin papules have no life-threatening complications. Its cutaneous lesions are mainly found on the trunk or extremities and are characterized by pink papules about 3 to 10 mm and have porcelain-white centers [2]. These lesions are typically not pruritic and not painful.

Herein, we present a case of Degos disease and briefly discuss prednisolone’s role in treating patients suffering from Degos disease.

CASE REPORT

A 54-year-old male presented with a two-month history of non-pruritic purpura predominantly on both legs, along with ankle edema. He also presented with a generalized feeling of leg weakness. The patient’s medical history included a recent coronary angioplasty and stent insertion, bronchial asthma, and diabetic retinopathy (HbA1c: 8.1%, normal rage: 4.0-6.0), thus was treated with tablets of acetylsalicylic acid 100 mg once daily and omalizumab injection every two weeks, respectively. On physical examination, atrophic, porcelain-white scars surrounded by an erythematous rim were seen on the patient’s lower extremities (Fig. 1). Biochemical profiling showed an increased SGPT level of 96 UL (normal range: 20–60 UL) and SGOT level of 47 UL (normal range: 10–40 UL). An extensive laboratory evaluation was performed and showed no concurrent clotting disorder (normal platelets, PT, APPT, INR, fibrinogen, lupus anticoagulant) along with no inflammatory markers detected (ESR, CRP). Regarding autoimmune screening markers such as ANCA, rheumatoid factor, anti-RNP, anti-RO, anti-LA, anti-ENA, anti-dsDNA antibodies, anti-TPO, aCL IgG, aCL IgM, Beta-2-Glycoprotein 1 IgG/IgM, anti-CCP3 IgG, anti-PM/Scl were found within the normal values, whilst a positive antinuclear antibody test with high ANA titers (1:640), increased levels of anti-TPO (119.6 U/mL; normal range: <60) and elevated IgE (919.4 IU/mL; normal range: 0.5100 IU/mL) were also revealed. Overall, the histologic findings were consistent with small/medium vessel vasculitis (leukocytoclastic vasculitis). The diagnosis was based on cutaneous manifestations and histological findings of a punch biopsy (Figs. 2a – 2f).

Figure 1: Patient’s left leg skin.

Figure 2: a) Skin biopsy: superficial and deep perivascular and periadnexal lymphocytic infiltrate. b) Skin biopsy: thrombotic vasculopathy. c) Skin biopsy: homogenization of the superficial dermis. d) Skin biopsy: edema with homogenization of the superficial dermis. e) Skin biopsy: lymphocytic infiltrate and vasculopathy with fibrin thrombi. f) Skin biopsy: lymphocytic infiltrate, vasculopathy change with fibrin thrombi, and extravasated red blood cells.

A thoracic CT scan was performed, and no findings of recent onset were reported. A complete eye exam showed no macular edema in either eye. In agreement with the ophthalmologist’s recommendation, he was treated with tablet hydroxychloroquine at a daily dose of 300 mg in conjunction with topical corticosteroid cream and oral prednisolone at a dose of 15 mg daily in order to promote tissue microcirculation. He is gradually recovering following a schedule for the tapering of prednisolone.

DISCUSSION

There are no clear-cut guidelines that may be used in the management of Degos disease. Hence, no specific pharmacologic agent has been shown to be exclusively effective in the management of Degos disease. Thus, various agents have been used in its management, such as aspirin, heparin, warfarin, dextran, sulfonamides, hydroxychloroquine, and even prednisolone. Prednisolone is a steroid and a potent anti-inflammatory drug used in the management of various inflammatory illnesses. Prednisolone may be administered intravenously as methylprednisolone or applied topically. Prednisolone is used to reduce inflammation, prevent the eruption of new lesions of the skin, and minimize the disease progression systemically, as the lesions of the disease are characterized by the infiltration of inflammatory cells. In a case study by Stavorn and Chanprapaph (2019), treatment was initiated using intravenous prednisolone for three days: high-dose prednisolone, approximately 1 mg/kg/day, then tapered down to 15 mg/day. Treatment is maintained until there is a marked improvement in symptomatology. Prednisolone has been shown to predispose a normal person without Degos disease to intestinal perforation [1]. Hence, in Degos disease, patients are already predisposed to intestinal perforation due to the disease process, thus it should be administered with care. In a study by Guo et al. (2014), malignant atrophic papulosis was successfully treated using a combination of intravenous immunoglobulin and corticosteroids. The case study involved a nine-year-old boy who had presented with asymptomatic skin lesions pathognomonic to Degos disease. The lesions ranged from 0.3 to 0.6 cm in diameter and had an erythematous border surrounding the porcelain-like central zone. It is reported that over the years, the disease started as asymptomatic skin lesions, and it eventually affected the central nervous system, resulting in the patient developing mild visual impairment and ptosis of the right eyelid. He was initiated on 300 mg of aspirin and 32 mg of oral prednisolone (1 mg per day). After administering prednisolone and aspirin for two months, prednisolone was tapered down to 26 mg per day and monthly injections of intravenous immunoglobulin. The patient’s condition markedly improved as there were no new neurological manifestations and no formation of additional skin lesions. The prednisolone dose was later tapered down and maintained at 12 mg after the patient’s condition had improved. These results correlated to the results from a study done by Kameda et al. (2010). A combination of cyclophosphamide and methylprednisolone was used to successfully treat a 42-year-old male suffering from Degos disease [3].

However, some research has shown that using immunosuppressants such as corticosteroids in the management of Degos disease may worsen skin lesions and result in complications in the progression of the disease [4]. For instance, Powell et al. (1999) described the case of a 61-year-old female who had been diagnosed with Degos disease and had undergone cadaveric renal transplantation. The patient was on immunosuppression therapy consisting of cyclosporine, azathioprine, and prednisolone. When the patient was initiated on the immunosuppressive therapy, the study observed numerous lesions pathognomonic to Degos disease developing. These lesions were markedly larger and could easily be seen compared to lesions that she had before the induction of the immunosuppressive therapy. Thus, the study concluded that prednisolone and other immunosuppressive agents worsened the patient’s cutaneous manifestations of the disease [5].

CONCLUSION

Degos disease is a rare disease whose benign form presents with dermatological manifestations, and the malignant form presents with systemic manifestations. There are no specific strategies for the treatment of the disease. There is an indication that Degos skin lesions, treated with hydroxychloroquine, are well managed and improved slightly with no development of new ones. Moreover, immunosuppressants such as prednisolone have been shown to be successful. Studies have shown that, when prednisolone is combined with other immunosuppressants such as cyclophosphamide, it reduces the dermatological manifestations of the disease. However, some studies have shown the worsening of dermatological manifestations with immunosuppressant therapy.

REFERENCES

1.Stavorn T, Chanprapaph K. Degos-like lesions in association with connective tissue diseases:Are port of three cases and literature review. Clin Cosmet Investig Dermatol. 2019;12:815.

2.Guo YF, Pan WH, Cheng RH, Yu H, Liao WQ, Yao ZR. Successful treatment of neurological malignant atrophic papulosis in a child by corticosteroid combined with intravenous immunoglobulin. CNS Neuroscience &amp;amp;Therapeutics. 2013;20:88-91. [PMID:24341936].

3.Kameda T, Dobashi H, Yoneda K, Susaki K, Kuno T, Murao K, Ishida T. A case of Degos disease successfully treated with corticosteroid combined with cyclophosphamide. Rheumatol Int. 2012;32:2169-73.

4.Theodoridis A, Makrantonaki E, Zouboulis CC. Malignant atrophic papulosis (Köhlmeier –Degos disease):A review. Orphanet J Rare Diseases. 2013;14:8-10.

5.Powell J, Bordea C, Wojnarowska F, Farrell AM, Morris PJ. Benign familial Degos disease worsening during immunosuppression. British J Dermatol. 1999;141:524-7.