Angiokeratoma

Question 1

an X-linked recessive disease, is aninbornerroro metabolisminwhichthereisade ciencyo α-galactosidaseAleadingtoan accumulation o neutral glycosphingolipid ceramide trihexoside in endothelial cells, brocytes, and pericytes in the dermis, heart, kidneys, and autonomic nervous system. Lesions are numerous dark red, punctate, and tiny (< 1 mm) (Fig. 9-26), located on the lower hal o the body: lower abdomen, g e n i t a l i a , a n d b u t t o c k s , a l t h o u g h l e s i o n s m a y a l s o o c c u r o n t h e l i p s . Th e h o m o z y g o u s m a l e s a l s o havesymptomsrelatedtoinvolvemento otherorgansystems:acroparesthesias,excruciatingpain, transient ischemic attacks, and myocardial in arction. Heterozygous emales may have corneal opaci- ties. Fabry disease is very rare but serious.

Answer 1

Angiokeratoma corporis di usum (Fabrydisease

Question 2

comprises pink to dark red and even black papules that occurontheelbows,knees,anddorsao thehands.Thisautosomal-dominantdiseaseisrareand occursinyoung emales.

Answer 2

angiokeratoma of mibelli

Question 3

most common angiokeratoma.

this disease involves the scrotum and vulva; the lesions are multiple papules (≤ 4 mm) that are dark red to black in color and present in quite large numbers

Answer 3

Angiokeratoma of fordyce

Question 4

are dark violaceous to black, o ten keratotic papules or small plaques that are hard upon palpation and cannot be compressed by diascopy

Answer 4

angiokeratoma

Question 5

verifies diagnosis for angiokeratoma of fordyce

Answer 5

Reddish, violaceous, and black papules on the scrotum. They blanch upon diascopy and this veri ies the diagnosis. Note: Thrombosed angio- keratomas do not blanch