The University of Arizona


S.A. Salvia, M.A. Amore, C.M. Papendieck


Microcystic lymphatic malformations as
described in the international literature form a
subgroup of low-flow congenital vascular malformations
(VM) resulting from irregular
embryological development. Microcystic lesions
normally manifest as an accumulation of
lymph- and blood-filled vesicles that, when externalized,
cause skin maceration with consequent
pain and potential infection resulting in
the impairment of the patient's quality of life.
There is no consensus on a standardized
algorithm nor clear guidelines for successful
treatment of this type of lymphatic malformation,
and treatment options employed often result
in ambivalent and transient outcomes with
a high rate of recurrence. The topical formulation
of tacrolimus is a well-known FDAapproved
anti-T cell agent that was recently
identified as a potent activator of ALK1, which
is involved in several processes and functions
including angiogenesis. We investigated if
topical administration of tacrolimus may be an
effective therapy for directly targeting cutaneous
microcystic lymphatic malformations as a
complement to systemic treatment. The study
enrolled four patients with cutaneous microcystic
lymphatic malformations: three male
(ages: 13,15,18) and one female (age: 30). Two
of the patients presented lesions on their backs,
one patient on the left hand and one on the left
lower limb. All four patients received treatment
with topical tacrolimus 0.1% twice a day for 10
weeks on a previously selected area for
application. Weekly clinical follow-ups were
conducted along with close physician-patient
contact. All patients displayed a satisfactory
response after treatment. Lymphorrhea and
bleeding were stopped in all cases and the
esthetic aspect of lesions improved in two
patients. To date, all patients presented no
clinically significant changes to the size or
extension of the lesion. Topical tacrolimus
treatment is a promising and reasonable option
for microcystic lymphatic malformations. Our
results encourage further exploration in larger
populations with the consideration that it is a
safe and effective alternative or complementary
therapy to systemic treatment.

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