Raccoon Eye-like Presentation: Clue Toward a Hematological Life-threatening Condition

Madam, A 10-year-old boy presented to our dermatology outpatient department with sudden onset bluish-red discoloration involving the left periorbital region for 7 days. There was no history of head injuries. The patient also gave a history of intermittent episodes of bleeding from the nose and gums for 7 days. There was no history of similar episodes in the past or any other medical condition. There was no history of hematuria, melena, or drug intake in the recent past. There was no history of similar illness among any other family members. There was no history of fever, cough, running nose, sore throat, myalgia or diarrhea suggestive of viral infections. The child was completely immunized for his age. On examination, there was erythematous to dusky discoloration of the entire left eye involving the eyelids and periorbital region [Figure 1]. Furthermore, multiple discrete erythematous macules were present over the face, neck, and upper trunk which were nonblanchable suggestive of petechiae and ecchymoses [Figures 2 and 3]. There was no subconjunctival hemorrhage or mucosal hemorrhagic vesicle or bullae noted. There was no macroglossia, periorbital edema, or peripheral edema noted in the patient. There was no associated muscle weakness or any other feature of connective tissue disease. A complete blood count and peripheral blood smear confirmed thrombocytopenia and a platelet count of 8000/μl with a normal erythrocyte and leukocyte count. The patient's Coomb's test, coagulation profile, fibrin degradation product (FDP), and muscle enzymes were all within the normal limits. Urine routine microscopy and Electrocardiogram (ECG) were also within the normal limits. The patient's viral markers were negative.Figure 1: Ecchymosis present around the left peri-orbital region giving rise to a "Raccoon-eye" like presentation and multiple petechial spots present over the central part of the faceFigure 2: "Raccoon-eye" like presentation of the left eye with multiple petechiae present over the left side of the face extending up to the neckFigure 3: Petechial spots involving the right side of the faceBased on the above findings, primary idiopathic thrombocytopenic purpura (ITP) was diagnosed. The patient was later referred to the hematology department for further evaluation and management. Discussion Primary ITP is an acquired autoimmune condition where immune-mediated platelet destruction is seen.[1] Primary ITP is characterized by isolated thrombocytopenia and is a diagnosis of exclusion after ruling out any underlying pathology.[2] ITP is a female predominant disease; however, increased incidence has been noted among males in early childhood and the elderly population.[3] Based on duration, ITP has been classified broadly into three categories: newly diagnosed, persistent (3–12 months), and chronic (>12 months).[2] The clinical presentation in the case of ITP can vary from asymptomatic to frank bleeding from the gums and mucosa.[3] Kurata et al. reported the following clinical symptoms in their study: purpura (62.8%), gingival bleeding (19.9%), epistaxis (10%), hematuria (6.6%), menorrhagia (3.9%), melena (3.8%), cerebral bleeding (0.7%), and other bleeding symptoms (3.1%).[4] Primary ITP is a diagnosis of exclusion and the close differentials include secondary ITP, myelodysplastic syndrome, drug-induced thrombocytopenia, inherited thrombocytopenia, and bone marrow failure.[1] To rule out the differentials, the following laboratory investigations are recommended: complete blood count with peripheral smear, coagulation profile, bleeding time, D-dimer, fibrinogen levels, FDP, erythrocyte sedimentation rate, C-reactive protein, and viral markers.[3] Raccoon-eyes can be a clinical presentation of systemic amyloidosis. Furthermore, the heliotrope rash of dermatomyositis can easily mimic "Raccoon-eyes." In this case, the absence of any systemic symptoms with normal renal and cardiac profile ruled out amyloidosis. There was no clinical evidence of muscle weakness or any other cutaneous features with normal muscle enzyme levels, which ruled out dermatomyositis. The primary goal of treatment in ITP is to arrest active bleeding, if any, which is usually seen in cases with a platelet count below 20,000/μl.[2] Treatment options include high-dose systemic glucocorticoids, intravenous immunoglobulin, and platelet transfusion.[2] For the treatment of refractory cases, rituximab has been used successfully. Long-term use of thrombopoietin receptor agonists such as romiplostim, eltrombopag, and avatrombopag has shown good results.[2] In patients with persistent low platelet counts (<30,000/μl) and bleeding symptoms even after 4–6 weeks of standard therapy, splenectomy is an effective treatment option.[3] Our case had a sudden onset of ecchymosis involving the left eye, causing, apprehension and anxiety in parents and was diagnosed with ITP after thorough examination and investigation. Dermatologists should be aware of the cutaneous manifestation of ITP so that early diagnosis of this fatal but treatable condition can be made to save the life of the patient. Declaration of patient consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.