Scleromyxedema with acute lymphoblastic leukemia
A 21-year-old female patient with unremarkable medical, family, and psychological history was diagnosed with acute lymphoblastic leukemia. The patient was on the appropriate chemotherapeutic regimen in the hematology ward. There, she developed post-chemotherapy pancytopenia with neutropenic sepsis, which was treated with appropriate intravenous antibiotics (tigecycline, colistin, meropenem, and ceftazidime), and blood product transfusion as needed. During her hospitalization, and due to the low platelet counts, the patient developed a pulmonary hemorrhage that required intubation and prolonged mechanical ventilation in the medical intensive care unit (ICU), during which her cell counts have recovered, but she remained in sepsis in the form of intractable fever with positive sputum and blood cultures for Pseudomonas aeruginosa.

During her ICU stay, the patient started to develop multiple, well-defined velvety, thickened, darkly pigmented papules over her thighs, external genitalia, lower abdomen, and sub-mammary flexures, which gradually enlarged, merged, and thickened to form hyper-pigmented infiltrated plaque. These lesions were associated with intractable fever which was not fully explained by persistent sepsis, as the latter was well controlled with hemodynamic stability and normalization of her inflammatory markers. Her complete blood count, routine examination and culture of urine, liver function test, renal function test, serum protein electrophoresis, blood sugar, and thyroid profile were within the normal limits. A Computed Tomography scan of her body during a routine evaluation of her persistent fever and sepsis showed incidental left thalamic ischemic stroke and multiple splenic infarctions. Trans-esophageal echocardiography was non-contributive.

Based on these clinical suspicions of scleromyxedema, a skin biopsy was performed and revealed abundant amounts of dermal amorphous mucinous material separating increased thickened collagen bundles, which was confirmed by mucicarmine special stain. In addition, the biopsy showed proliferation of stellate fibroblasts, and superficial and deep perivascular lymphoplasmacytic infiltrate. The patient was started on high dose IVIG (2 g/kg) given over 2 days, in monthly cycles for a total of 6 months, and this was well tolerated. After starting the first dose of IVIG, the patient’s state considerably ameliorated in the form of improvement of cutaneous lesions and the complete disappearance of her intractable fever.