Peutz-Jeghers syndrome - Skin
For more information, see OMIM.
Q85.8 – Other phakomatoses, not elsewhere classified
54411001 – Peutz-Jeghers syndrome
- Familial lentiginosis syndromes such as LEOPARD syndrome and NAME may appear similar, but they have other associated systemic signs and the pigmentation is not as clearly periorificial.
- Addison's disease – This may give rise to multiple melanotic macules on the oral mucosa but ACTH levels are elevated.
- Laugier-Hunziker syndrome – This consists of oral melanotic macules and melanotic striations on the nails.
- Albright's syndrome – This produces oral melanotic macules but is also associated with precocious puberty and polyostotic fibrous dysplasia.
- Drug-induced pigmentation – This may affect the skin and oral mucosa but not usually in this periorificial configuration.
- HIV infection – This often causes diffuse eruptive pigmentary macules of the oral cavity but they are usually intraoral.