Mucocutaneous Manifestations in a Case of Immune Thrombocytopenia: Reporting a Rare Presentation with Dermoscopic Findings

Sir, Immune thrombocytopenia (ITP) is a rare, acquired, autoimmune disorder characterized by low platelet count along with varying degrees of mucocutaneous bleeding. It is a diagnosis of exclusion, made only after ruling out other causes of thrombocytopenia.[1] Signs and symptoms may vary from mild or no bleeding to severe hemorrhage in a minority of cases, and is independent of the platelet count.[2,3] We report a case of ITP in an 18-year-old male who initially presented with acute-onset black nodules in oral mucosa, trunk, and extremities. This type of picture is rarely seen in ITP and can indicate the presence of severe thrombocytopenia. An 18-year-old male presented with sudden eruption of multiple asymptomatic blue-black blisters in oral mucosa and on the skin along with multiple pin head size red lesions on the body. Lesions resolved within 5 days without leaving any residual pigmentation or scarring. Individual did not give a history of any fever, trauma, insect bite, or bleeding from mucosal sites prior to the onset of the lesion. There were no similar complaints in the family. Dermatological examination revealed multiple soft, well-demarcated blue-black, nontender, nonpulsatile, nonblanching compressible papulonodules scattered on the body and similar lesions on the tongue and buccal mucosa. There was also multiple nonpalpable purpura on the body [Figures 1-3].Figure 1: Dry purpura, petechiae seen on the palmFigure 2: Purpura and petechiae seen over the tongue and scattered on the neck and shouldersFigure 3: Wet purpura on inner side of the lower lipDermoscopy of the purpuric lesion on the neck and trunk revealed homogeneous, violaceous structureless areas, which correlates with the noninflammatory form of purpura [Figure 4] while that from the papulonodular lesion in the buccal mucosa revealed a blot of dark-colored blood [Figure 5].Figure 4: (IDS-1100, polarized, ×10; Illuco, Republic of Korea) Dermoscopy of purpuric lesion on neck revealed broad, homogeneous, violaceous structureless areaFigure 5: (IDS-1100, polarized, ×10; Illuco, Republic of Korea) Dermoscopy of purpuric lesion on the lower lip revealed blot of dark-colored bloodAn urgent blood count was done, which showed platelets <10 × 109/L. The peripheral blood smear (PBS) examination was normal. Immediate consultation with the hematologist was done, and the patient was admitted with the diagnosis of acute ITP. He was investigated thoroughly to rule out any other underlying cause of thrombocytopenia. Bone marrow biopsy showed megakaryocytic thrombocytopenia. During admission, he also had one episode of severe epistaxis, which did not resolve even after postnasal pack. He was started on injection dexamethasone 40 mg daily for 4 days and synchronously administered intravenous immunoglobulin (IVIG) 1 g/kg daily for 2 days. Due to insufficient response in raising platelet count, he was then administered 5 doses of injection rituximab 375 mg/m2 IV weekly. When the platelet count reached >100 × 109/L, the patient was discharged on a tapering dose of prednisolone and eltrombopag 50 mg daily. He is on regular follow-up. ITP is defined as an isolated thrombocytopenia with platelet counts below 100 × 109/L without changes in the bone marrow and in the absence of other causes of thrombocytopenia.[1] ITP has a bimodal distribution affecting the age group of both children/adolescents and adults (55–60 years). Etiological classification divides it into primary (isolated thrombocytopenia) and secondary when thrombocytopenia is secondary to autoimmune diseases, viral infections, lymphoproliferative neoplasms.[3] Classification of ITP based on the duration of disease and therapeutic goals, divides it into three subtypes- newly diagnosed which is less than three months duration of thrombocytopenia, persistent- three to 12 months duration of thrombocytopenia and chronic-more than 12 months duration of thrombocytopenia.[4,5] Pathogenesis involves loss of tolerance to proteins expressed on the platelets, the major target antigen being glycoprotein IIb ⁄ IIIa as well as glycoprotein Ib ⁄ IX. Other mechanisms to explain platelet destruction in the absence of autoantibodies include impaired thrombopoiesis, immune dysregulation, and direct toxicity of T-lymphocytes toward platelets.[1-3] The cutaneous findings of ITP may be broadly divided into two categories: dry and wet purpura. Dry purpura or cutaneous hemorrhage appears as bruising or petechiae, which are flat and not palpable. In contrast, wet purpura (blood-filled vesicles) is associated with bleeding of the mucous membranes of the gastrointestinal tract, mouth, nose, and eyes.[1-4] Other signs and symptoms include spontaneous bleeding such as epistaxis or gum hemorrhage, menorrhagia, extensive hematomas, and joint hemorrhages. However, it is imperative to distinguish “platelet-type” mucocutaneous bleeding from “coagulation-type” hematomas.[3,5] The diagnosis of ITP is made after ruling out all other causes of platelet count <100 × 109/L, which can be both acquired and hereditary.[3] Acquired causes include posttransfusion purpura, drug-induced thrombocytopenia, malignancies, infections, and autoimmune diseases such as systemic lupus erythematosus, lymphoproliferative disorders, myelodysplasia, agammaglobulinemia, and alloimmune thrombocytopenia. Laboratory investigations are basic and a simple platelet count along with PBS examination is enough to diagnose ITP.[5] Dermoscopy can aid in differentiating between inflammatory and noninflammatory purpura. The basic dermoscopic patterns are homogeneous, mottled, perifollicular (with purpuric halos), and epidermal purpuric. Broad, homogeneous, structureless patterns are usually seen in noninflammatory purpura like bleeding diathesis, vessel wall, and stromal abnormalities whereas mottled patterns associated with speckled, purpuric splotches, and globules over a purplish background indicate an underlying inflammatory element such as vasculitis and pigmented purpuric dermatosis.[6] Systemic corticosteroids are the first-line treatment, while thrombopoietin receptor agonists romiplostim and eltrombopag form the second-line therapy. In case of severe and life-threatening bleeding, IVIGs and rituximab can be given.[5] ITP presents with a wide heterogeneity of the clinical course, from completely asymptomatic with no signs or symptoms of bleeding despite constant low platelet count to a frank brain hemorrhage. Hence, clinicians should be aware of all cutaneous features associated with ITP for early management of this life-threatening condition. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials 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.