Scleromyxedema in a 21 Y/F patient with acute lymphoblastic
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Scleromyxedema is a rare, para-neoplastic, chronic, progressive condition of the Lichen myxedematosus (LM) family. The clinical picture consists of generalized confluent papular eruptions with possible systemic manifestations, which may be fatal as it still constitutes a therapeutic dilemma.

A 21 year old female patient reported with unremarkable medical, family, and psychological history, who was diagnosed with ALL. 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 IV 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 (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, 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 a persistent sepsis, as the latter was well controlled with hemodynamic stability and normalization of her inflammatory markers. CT scan of her body during a routine evaluation of her persistent fever and sepsis showed incidental left thalamic ischemic stroke and multiple splenic infarctions.

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, patient’s state considerably ameliorated in the form of improvement of cutaneous lesions and complete disappearance of her intractable fever.

In conclusion, scleromyxedema is a rare but disabling skin disease. It is very rarely associated with ALL. Atypical forms require a high index of suspicion for diagnosis.

Source: https://bmcdermatol.biomedcentral.com/articles/10.1186/s12895-020-00118-7
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