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An Unusual Presentation of Acquired Hypothyroidism: The Van Wyk-Grumbach Syndrome.
MedLine Citation:
PMID:  22170796     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
AbstractThe association in young females of long standing primary hypothyroidism, isosexual precocious pseudopuberty and multi-cystic enlarged ovaries was first described in 1960 by Van Wyk and Grumbach. Since then, sporadic case reports have contributed to clarifying the key features of this syndrome.The unique elements that lead to this diagnosis are FSH-dominated sexual precocity combined with a delayed bone age in the presence of hypothyroidism. It is important to recognise this syndrome because initiating simple thyroid hormone replacement completely resolves symptoms and hormone abnormalities, avoiding unnecessary investigations for malignancies or surgical intervention.We describe an 8-year old girl with autoimmune thyroiditis and severe long standing hypothyroidism presenting with the clinical features of Van Wyk-Grumbach syndrome, a secondary TSH secreting adenoma and hyperprolactinaemia. In addition, this girl presented with microcytic anaemia, elevated ESR and two unusual features - a newly developed streaky hyperpigmented skin lesion and PTH suppression despite vitamin D deficiency. Thyroxine replacement normalized all hormone abnormalities, shrunk the pituitary adenoma within 9 months but the new skin lesion persisted. We review the literature and explore the pathophysiology of known and new features which give rise to speculation indicating stimulation of the FSH G protein coupled receptor by excessive TSH, but LH suppression by hyperprolactinaemia.
Authors:
Elizabeth Baranowski; Wolfgang Hoegler
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-12-14
Journal Detail:
Title:  European journal of endocrinology / European Federation of Endocrine Societies     Volume:  -     ISSN:  1479-683X     ISO Abbreviation:  -     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-12-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9423848     Medline TA:  Eur J Endocrinol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
E Baranowski, Diabetes and Endocrinology, Birmingham Children's Hospital, West Midlands, B4 6NH, United Kingdom.
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