Endocrine Abstracts

I would like to present a case of autoimmune poly glandular syndrome who first presented as profround hypothyroidism and secondary amenorrhea 8 years after her first baby. TSH was >100 and LH and FSH 44.3 and 27 at diagnosis with oestradiol of 18. As patient remained symptomatic after being on thyroxine replacement she went on to undergo a synacthen test which showed a much blunted response (baseline cortisol 264, 30 min 273 and 60 min 264). While being investigated as an inpatient, she was found to have a cyst on her forehead which was subsequently excised and the histology report was consistent with Non Caseating Granulomatous Dermatitis (Darier Roussy Syndrome)/Cutaneous Sarcoidosis. Her Cx-ray was reported as normal with normal ACE level and no other systemic manifestations of sarcoidosis were elicited. This association of autoimmune polyglandular syndromes and sarcoidosis has been very rarely reported in the literature (5 cases). The pathogenesis of sarcoidosis is unknown, it is probably a disease promoted by aberrations in immunological reactivity’ and a combination of abnormalities of some aspects of T cell function with enhanced or normal ability of B cells to produce antibodies’ has been documented. The HLA haplotype Al, B8, DR3, Dw3, DR4 being more frequent in patients with the PGA syndromes type II and III, with adult coeliac disease or with sarcoidosis, as compared to the general population. This indicates a common underlying genetic predisposition to these conditions.