Entry - 167300 - PAGET DISEASE, EXTRAMAMMARY - OMIM

 
ICD+
167300

PAGET DISEASE, EXTRAMAMMARY


Clinical Synopsis
 

Oncology
- Extramammary Paget disease
- Internal malignancy
Skin
- Eczematous eruptions with weeping and crust formation
Inheritance
- Rare familial cases
▲ Close

▼ TEXT

Extramammary Paget disease is a cancerous disease seen at various sites, most often in the anogenital region. The clinical features are usually those of eczematous eruptions with weeping and crust formation. This disease has been shown to be a skin manifestation of internal malignancy. In a study of 40 patients with Paget disease of the anogenital region, Helwig and Graham (1963) did not find a family history of the disease in any of the cases. Kuehn et al. (1973) described a case occurring in a father and son. The father, aged 66, presented with extramammary Paget disease in the right scrotal area. No mention was made of other family history.


▼ REFERENCES

  1. Helwig, E. B., Graham, F. N. Anogenital (extramammary Paget's disease). Cancer 16: 387-403, 1963. [PubMed: 13953662, related citations] [Full Text]

  2. Kuehn, P. G., Tennant, R., Brennerman, A. R. Familial occurrence of extramammary Paget's disease. Cancer 31: 145-148, 1973. [PubMed: 4345608, related citations] [Full Text]


Creation Date:
Victor A. McKusick : 6/2/1986
Edit History:
mimadm : 1/14/1995
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/27/1989
marie : 3/25/1988
reenie : 6/2/1986

167300

PAGET DISEASE, EXTRAMAMMARY


SNOMEDCT: 232336001, 71447003;   ORPHA: 2800;  



TEXT

Extramammary Paget disease is a cancerous disease seen at various sites, most often in the anogenital region. The clinical features are usually those of eczematous eruptions with weeping and crust formation. This disease has been shown to be a skin manifestation of internal malignancy. In a study of 40 patients with Paget disease of the anogenital region, Helwig and Graham (1963) did not find a family history of the disease in any of the cases. Kuehn et al. (1973) described a case occurring in a father and son. The father, aged 66, presented with extramammary Paget disease in the right scrotal area. No mention was made of other family history.


REFERENCES

  1. Helwig, E. B., Graham, F. N. Anogenital (extramammary Paget's disease). Cancer 16: 387-403, 1963. [PubMed: 13953662] [Full Text: https://doi.org/10.1002/1097-0142(196303)16:3<387::aid-cncr2820160314>3.0.co;2-0]

  2. Kuehn, P. G., Tennant, R., Brennerman, A. R. Familial occurrence of extramammary Paget's disease. Cancer 31: 145-148, 1973. [PubMed: 4345608] [Full Text: https://doi.org/10.1002/1097-0142(197301)31:1<145::aid-cncr2820310119>3.0.co;2-1]


Creation Date:
Victor A. McKusick : 6/2/1986

Edit History:
mimadm : 1/14/1995
supermim : 3/16/1992
supermim : 3/20/1990
ddp : 10/27/1989
marie : 3/25/1988
reenie : 6/2/1986



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OMIM® and Online Mendelian Inheritance in Man® are registered trademarks of the Johns Hopkins University.
Copyright® 1966-2024 Johns Hopkins University.

NOTE: OMIM is intended for use primarily by physicians and other professionals concerned with genetic disorders, by genetics researchers, and by advanced students in science and medicine. While the OMIM database is open to the public, users seeking information about a personal medical or genetic condition are urged to consult with a qualified physician for diagnosis and for answers to personal questions.
OMIM® and Online Mendelian Inheritance in Man® are registered trademarks of the Johns Hopkins University.
Copyright® 1966-2024 Johns Hopkins University.
Printed: Nov. 29, 2024