Endocrine Abstracts

A 60 year old pest control worker presented to the dermatologists with a 3-month history of progressive thickening and change in texture of skin lesions affecting the face, lips and eyelids. Histology revealed papular mucinosis/lichen myxoedematosus and he was treated with plasma exchange, high dose steroids and cyclophosphamide. Investigations for associated tiredness, four months following presentation and initiation of therapy revealed impaired GH and cortisol responses to hypoglycaemia (Peak GH=6.1 nmol/L; Peak cortisol 355 nmol/L), normal TSH response to TRH with low normal T4, and an exaggerated LH/FSH response to GnRH stimulation with decreased basal testosterone. Diabetes mellitus was excluded and ferritin levels were normal. MR imaging of the pituitary gland was also normal. The impaired cortisol response was attributed to high dose steroid therapy and a diagnosis of primary hypogonadism was made and he was commenced on testosterone replacement. He later went on to develop MGUS, a known association of scleromyxedema, and was also diagnosed with type 2 diabetes mellitus that remains diet controlled. Repeat testing following 18 months of therapy revealed a normal GH response to hypoglycaemia (Peak GH=45 nmol/L). Various neurological, endocrine and haematological conditions have been associated with scleromyxoedema. We report primary hypogonadism as a possible new endocrine association of scleromyxoedema, not previously described. Our patient was on cyclophosphamide at the time of diagnosis, the use of which is known to be associated with hypogonadism. However, symptoms predate the initiation of cyclophosphamide and the short duration of exposure to therapy makes this unlikely. Normalisation of the initially impaired GH response may also suggest possible pituitary involvement improving following medical therapy for scleromyxedema.