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[Marked endocrine abnormalities in polyneuritis with skin hyperpigmentation, hypertrichosis, edema and increased
Nihon Naibunpi Gakkai Zasshi
|September 20, 1983
Summary
This study details a rare plasma cell dyscrasia syndrome with polyneuropathy and endocrine issues. It highlights unusual endocrine abnormalities and their varied clinical manifestations in a patient case.
Area of Science:
- Endocrinology
- Neurology
- Hematology
Background:
- Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, abnormal M protein, and skin changes (POEMS) syndrome presents with diverse symptoms.
- Understanding the full spectrum of POEMS syndrome manifestations is crucial for diagnosis and management.
Observation:
- A 47-year-old female presented with edema, gait disturbance, paresthesia, skin hyperpigmentation, and hypertrichosis.
- Clinical examination revealed cyanotic legs on standing, clubbed fingers, cervical lymphadenopathy, mild hepatomegaly, and loss of tendon reflexes.
- Cerebrospinal fluid showed elevated protein without pleocytosis.
Findings:
- The patient exhibited polyneuropathy, endocrine disturbances, and characteristic skin changes associated with POEMS syndrome.
- Treatment with prednisolone showed a slight improvement in walking but did not resolve edema or paresthesia.
Implications:
- This case expands the known clinical spectrum of POEMS syndrome, particularly regarding miscellaneous endocrine abnormalities.
- Further research is needed to elucidate the underlying mechanisms and optimize treatment strategies for POEMS syndrome.