AGMINATED LENTIGINOSIS
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I have a 16 y/o male who since the age of 2 developed 1 to 4 mm brown macules
on the face. At 6 y/o gradually involved the right side of the neck and at
present extending to involve the right side of the trunk with sharp cut off
at the midline. Optha, ENT, Neuro exam normal. No axillary freckling nor
cafe au lait spots noted. However at his age no secondary sexual
characteristics noted. 4 mm skin punch biopsy was read as lentigo simplex.
Signed this patient out as Agminated lentiginosis. Search at silver platter
reveal only 13 cases reported.
Has anybody seen a similar case?
Course and Prognosis?
Treatment options?
Agree with the diagnosis or could we have missed something? 

Jonathan Yu, MD
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I'd consider a variant of the Carney Complex.  I'd also investigate his
endocrinologic and cardiac status.

Robert I. Rudolph, M.D., FACP
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I would second Dr. Rudolph's suggestion. We have seen a lot of patients
with subtle Carney's/NAME/LAMB presenting with head/neck > upper body
lentiginosis. Check oral, ocular, vaginal mucosae for lentigines, blue
nevi, and any stray "cysts", "lipomas", "moles", even "cellulite" as
possible myxomas. We have diagnosed Carney's on stray endocrine patients
referred for other problems that had lentiginosis, it may be more
common/asymptomatic than generally supposed.

David J. Altman, MD, PhD
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How about LEOPARD syndrome in which you have "A"bnormal genitalia and 
growth "R"etadartion.  Any family history? since this is inherited as 
autosomally dominant, but spontaneous mutation can sure happen.
 
Mychael Luu
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LEOPARD syndrome was initialy one of our differential diagnosis but there
was no ocular hypertelorism, ECG conduction defects and pulmonic stenosis.
What made our diagnosis for agminated lentiginosis rather than LEOPARD was
as follows: patient is not mentally retarded, no family history and what
really swayed us was that our patient's lentigines were in a segmental
pattern. Leopard syndrome usually would show a generalized pattern for its
lentigines.

Jonathan Yu, MD
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