Sezary disease, lymph nodes of axilla and upper limb

This code identifies Sezary disease, a rare and aggressive form of cutaneous T-cell lymphoma, specifically when it involves the lymph nodes located in the axilla and/or upper limb. Sezary disease is characterized by widespread erythematous skin lesions, generalized lymphadenopathy, and the presence of malignant T-cells (Sezary cells) in the blood, skin, and lymph nodes.

Use this code when documentation confirms a diagnosis of Sezary disease with clear evidence of involvement of the lymph nodes in the axillary region or anywhere in the upper limb. This typically requires pathology reports confirming Sezary cells in lymph node biopsies from these specific anatomical sites. Clinical notes describing palpable or imaging-confirmed lymphadenopathy in these areas, coupled with a definitive Sezary disease diagnosis, support its use.

• Ensure documentation explicitly states lymph node involvement in the axilla or upper limb; generalized lymphadenopathy alone is insufficient.
• Distinguish from other cutaneous T-cell lymphomas, such as Mycosis Fungoides, which have different ICD-10-CM codes.
• Always sequence this code as the primary diagnosis when it is the reason for the encounter, or as a secondary diagnosis if it is a co-morbidity impacting treatment.