within plaques of linear atrophoderma of Moulin: a twin-spotting phenomenon?
Serious internal anomalies are also observed in association with the following circumscribed lentiginosis syndromes: Peutz-Jeghers syndrome, Cronkbite-Canada syndrome, Bandler syndrome, and lentiginosis centrofacialis.
The Cronkhite-Canada syndrome is characterized by circumscribed lentiginosis of the extremities associated with lesions of the buccal mucosa, loss of hair, and polyposis of the intestine, the latter causing severe mal absorption.
The presence or absence of circumscribed or multiple lentiginosis accompanied by mucosal lesions is an important factor in the differential diagnosis of the disease called pigmentovascular phakomatosis.
The term "generalized lentiginosis" is often reserved for such patients who have widespread lentigines without systemic abnormalities.
Giant nevus spilus and centrofacial lentiginosis
. Pediatr Dermatol.
Understanding the common etiologic pathway in lentiginosis syndromes may help in developing therapeutic strategies and identifying individuals with less frequent or nonclassic presentations of such syndromes, said Dr.
Many patients thought to have LEOPARD syndrome have been recognized to have Watson syndrome, a condition that presents with pulmonary stenosis and inherited lentiginosis but is actually a form of neurofibromatosis type 1 (NF-1).
Another condition with a PTEN gene mutation is the Ruvalcaba-Myhre-Smith syndrome, in which patients have penile lentiginosis.
We report a patient with unilateral segmental lentiginosis of the right side of face and ipsilateral segmental vitiligo of right axilla.
Segmental lentiginosis, partial unilateral lentiginosis, segmental vitiligo.
A circumscribed grouping of lentigenes on one side of the body is called segmental lentiginosis.2 The grouping may be localized or involve a larger area of the body.2 This disorder is also known as zosteriform lentiginous naevus, partial unilateral lentiginosis (PUL) or lentiginous mosaicism.