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Birt‐Hogg‐Dubé‐syndrome and Hornstein‐Knickenberg‐syndrome are the same. Different sectioning technique as the cause of different histology
Author(s) -
Schulz Tilman,
Hartschuh Wolfgang
Publication year - 1999
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.1999.tb01792.x
Subject(s) - pathology , histology , medicine
The auiosomal dominant inherited syndromes of Horornstein and Knickenberg (HKS). and Birt, Hogg and Dubé (BHDS) are both characterized clinically by the overall spread of multiple flesh coloured papules of the skin. However, it is a mailer of debate il cnlonic neoplasms ladenomas as well as adenocarcinomas) are associated findings in the HKS or rather in the BHDS. Furthermore, histological differences are said to exist between the skin lesions in the two syndromes: whereas perifollicular fibromas were described m the HKS, fibroiolliculomas and trichodiscomas were found in the BHDS. In the present study, we report on a father and his daughter in whom we initially diagnosed a BHDS. We then examined a greater number of the papular lesions in histologic sections cut vertically as well as horizontally to the epidermis. Our results indicate that the histologic differences between the skin lesions in the two syndromes are artificial ones, caused by interpretation of different sectioning planes, and that consequently HKS and BHDS are the same. Therefore, it is necessary to look for colonic polyps in the syndrome in question, regardless if one prefers the name HKS or BHDS for it.