key: cord-1011670-te5pxkbj authors: El Arabi, Y.; El Fetoiki, FZ.; Marnissi, F.; Dahbi Skali, H.; Hali, F.; Chiheb, S. title: Necrotic lesions on the face in a patient with COVID-19 date: 2021-12-22 journal: J Med Vasc DOI: 10.1016/j.jdmv.2021.12.002 sha: 51287053d90fba627cd5fa0ed2eb40706640d862 doc_id: 1011670 cord_uid: te5pxkbj SARS-Cov-2’s cutaneous manifestations are polymorphic and increasingly recognized in the literature. Maculopapular rashes, urticaria and chilblains are the most mentioned. We are reporting the case of necrotic lesions localized exclusively on the face in a patient with COVID-19, due to thrombotic microangiopathy. A 17-year-old patient was admitted in February 2021 for necrotic lesions on the face associated with fever evolving for 10 days. No drugs or cocaine were taken before the symptomatology. The patient didn’t report any stings, bites, or applications of corrosive products. He wasn’t vaccinated against the COVID-19. Dermatological examination found infiltrated erythematous and violaceous maculopapular lesions, necrotic and hemorrhagic, without local inflammatory signs on the face and the helix. Cutaneous biopsy showed vascular thrombosis in the dermis and hypodermis without inflammation around the vessels, and perivascular lymphocytic infiltrate. Direct immunofluorescence was negative. Tzanck smear didn't show ballooning cells. COVID-19 serology was positive. Tests searching for autoimmune diseases such as secondary post-infectious vasculitis, primary vasculitis (periarteritis nodosa, Churg and Strauss, cryoglobulinemia, or microscopic polyangiitis), systemic lupus erythematosus, or antiphospholipid syndrome were negative. We didn’t find enough biological signs in favour of hemolysis or disseminated intravascular coagulation. We retained the diagnosis of a thrombopathy associated with COVID-19. The patient was put on corticosteroids with rapid withdrawal, anticoagulants, and petroleum jelly leading to a good evolution. We are reporting the case of necrotic lesions localized exclusively on the face in a patient with COVID-19, due to thrombotic microangiopathy. A 17-year-old patient was admitted in February 2021 for necrotic lesions on the face associated with fever evolving for 10 days. No drugs or cocaine were taken before the symptomatology. The patient didn't report any stings, bites, or applications of corrosive products. He wasn't vaccinated against the COVID-19. Dermatological examination found infiltrated erythematous and violaceous maculopapular lesions, necrotic and hemorrhagic, without local inflammatory signs on the face and the helix. Cutaneous biopsy showed vascular thrombosis in the dermis and hypodermis without inflammation around the vessels, and perivascular lymphocytic infiltrate. Direct immunofluorescence was negative. Tzanck The main manifestations of SARS-Cov-2 are fever, asthenia and respiratory symptoms [1] . Cutaneous manifestations were unknown at the beginning of the pandemic, but are now increasingly recognized in the literature. They are reported in many sporadic cases or case series. The prevalence of cutaneous manifestations in COVID-19 patients ranges from 0.2 to 20.4% [2] . Chilblains-like lesions, maculo-papular eruptions, livedo, petechiae, purpura, necrosis, wheals and vesicles are the most commonly described [3] . We are reporting the case of necrotic lesions localized exclusively on the face in a patient with COVID-19, due to thrombotic microangiopathy. fever of 102.2 °F and lower back pain that appeared one week before the cutaneous symptoms. No drugs or cocaine were taken before the symptomatology. The patient didn't report any stings, bites, or applications of corrosive products. He wasn't vaccinated against the COVID-19. On clinical examination at admission, the patient was apyretic, hemodynamically stable and eupneic. The dermatological examination revealed erythematous and purplish maculo-papular lesions with infiltration, necrotic and hemorrhagic, telangectasias and fine scales, without local inflammatory signs, on the face and the helix (figure 1). We also noticed a cheilitis without mucosal erosion. The abdomen was supple with no palpable hepatomegaly or splenomegaly. The rest of the clinical examination was without abnormalities. We suspected a secondary post-infectious vasculitis, a primary vasculitis (periarteritis nodosa, Churg and Strauss, cryoglobulinemia, or microscopic polyangiitis), a systemic lupus erythematosus, an antiphospholipid syndrome, or a thrombopathy associated with COVID-19. A cutaneous biopsy showed an acanthosic epidermis, basal vacuolation, vascular thrombosis in the dermis and hypodermis without inflammation around the vessels, and a perivascular lymphocytic infiltrate. Direct immunofluorescence was negative. Tzanck Abdominal ultrasound showed ascites of small volume. Cardiac and renal explorations were both normal. The final diagnosis was necrotic facial lesions secondary to a thrombopathy due to SARS-Cov-2. The patient was first put on corticosteroids 1 mg/kg/day (90 mg/day) since we suspected a vasculitis, acetylsalicylic acid 100 mg/day, therapeutic dose of low molecular weight heparin, vitamin C 1000 mg/day and petroleum jelly, during 2 weeks until we got the result of the cutaneous biopsy and the immunological assessment. The evolution was good with complete clinical and biological remission (figure 2). We kept a preventive dose of anticoagulation and vitamin C for 15 days. The corticotherapy was rapidly decreased in a period of 2 months. The follow-up is 10 months without recurrence. The particularity of our observation is related to the rarity of cephalic necrotic lesions due to COVID-19. Only a few cases have been reported. Karagounis reported 16 cases of necrotic lesions on the face and/or ears [4] , while one case of maxillary necrosis was noticed in the meta-analysis of Tan et al [5] . The occurrence of cephalic necrotic lesions is frequent in young males, as the case of our patient. Normally, necrotic lesions in SARS-Cov-2 appear as digital necrosis or distal ischemic lesions [1, 3, 6, 7] . However, chilblains are the most frequent acrosyndrome in the context of COVID 19 pandemic [8] . The diagnosis was retained according to a set of arguments (questioning, cutaneous biopsy and serology), and after eliminating other possible diagnosis that could explain the erythematous, infiltrated and necrotic maculo-papular lesions on the face and helix: secondary or primary vasculitis, systemic lupus erythematosus, antiphospholipid syndrome, or thrombopathy associated with cocaine use. All the work-up done in this sense was negative. The pathophysiologic mechanisms of cutaneous manifestations during COVID-19 can be explained by many theories. The virus binds to angiotensin II converting enzyme receptors leading to activation of macrophages, monocytes, and neutrophils. This leads to the secretion of cytokines: Interleukins 1, 6, 8 and tumor necrosis factor α and a prothrombotic state. Endothelial adhesion of neutrophils and Neutrophil Extracellular Traps (NET) secretion lead to vasculitis lesions [9] . The consequence is the development of tissue damage in the skin and all tissues containing angiotensin II converting enzyme receptors such as; heart, lungs, kidneys, oral cavity, pancreas, gastrointestinal tract and J o u r n a l P r e -p r o o f brain [10] . Maculopapular, urticarial and vesicular lesions are due to overproduction of cytokines, while chilblain-like, purpuric, livedoid and necrotic lesions are due either to vasculitis or to a thrombogenic vasculopathy [11] . The presence of necrotic lesions in COVID-19 is highly suggestive of vascular damage and a hypercoagulable state. This results of a cytokine storm as observed in other viral diseases. The biological assessment shows a high level of D-Dimer, prothrombin, and fibrinogen. Treatment must be rapidly started to improve the prognosis. It requires the use of a therapeutic dose of anticoagulation associated with corticosteroids. The dose and duration of oral corticotherapy is still controversial. We suggest that it depends on the clinical and biological evolution [2, 4] . The prognosis of cutaneous manifestations during COVID-19 is generally good with healing times up to 10 days and without recurrence [12] . In the current pandemic context of COVID-19, any dermatological lesion of vascular or inflammatory origin should raise the suspicion of SARS-Cov-2 infection, especially if there are other suggestive signs. However, this is an elimination diagnosis. This case of necrotic lesions localized exclusively on the face due to SARS-Cov-2 joins the other few cases described. It is a rare feature, hence the interest to report all observed cases. No potential competing interest relevant to this article was reported. Ischemic Necrosis of Lower Extremity in COVID-19: A Case Report Presentation and Management of Cutaneous Manifestations of COVID-19 Eruptions and related clinical course among 296 hospitalized adults with confirmed COVID-19 Acrofacial purpura and necrotic ulcerations in COVID-19: a case series from New York City Skin manifestations of COVID-19: A worldwide review The clinical spectrum of COVID-19-associated cutaneous manifestations: An Italian multicenter study of 200 adult patients Cutaneous Manifestations in Patients With COVID-19: Clinical Characteristics and Possible Pathophysiologic Mechanisms Chilblains and COVID19 infection: Causality or coincidence? How to proceed? Thromboplasminflammation in COVID-19 Coagulopathy: Three Viewpoints for Diagnostic and Therapeutic Strategies Effects of SARS-CoV-2 on Cardiovascular System: The Dual Role of Angiotensin-Converting Enzyme 2 (ACE2) as the Virus Receptor and Homeostasis Regulator-Review Cutaneous Manifestations of COVID-19: A Systematic Review Skin and COVID-19