Atypical Sweet's syndrome associated with non-Hodgkin lymphoma

Sweet's Syndrome (SS) is a rare neutrophilic dermatosis characterized by sudden appearance of painful indurated erythematous papules, nodules and plaques mainly on face, neck and extremities which is usually associated with haematological or solid tumors (20%-25%). Non-Hodgkin lymphoma is a well-recognized but rare association. Malignancy associated SS (MASS) can present with number of atypical clinical and histological features and carries a poor prognosis. Here we report a case of atypical SS associated with non-Hodgkin lymphoma.

A 62-year-old lady presented with multiple painful erythematous skin plaques mainly on limbs for 2-week duration. She also gave a history of chronic non-productive cough, shortness of breath, bilateral hip joint pain, and facial swelling for the past three months without fever. Examination revealed mild pallor, multiple erythematous indurated nodules, and confluent plaques on both forearms and lower legs. She has no peripheral lymphadenopathy or hepatosplenomegaly. Diagnosis of atypical SS was made, and screening for internal malignancy revealed associated para-aortic lymphadenopathy. Diagnosis of Non-Hodgkin lymphoma was confirmed by histological and radiological evaluation.

She was successfully treated with six cycles of combined chemotherapy but subsequently died after a year with complicated spinal metastasis.