Rare idiopathic disorder characterized by pigmented
purpuric dermatitis caused by chronic inflammation of capillaries. A chronic
disease of mostly unknown etiology showing very distinctive clinical
appearance (extravasation of erythrocytes in the skin with marked
Purpura Annularis Telangiectodes; Pigmented Purpuric
Uncommon disease that occurs predominantly in children
and young adults; seems to affect males more frequently.
During a 10-month
period, ten cases were reported in the United kingdom from a dermatology
that serves a population of 300,000 persons. Five patients were diagnosed
lichen aureus and the remainder had more extensive capillaritis.
Chronic inflammation of capillaries in the upper
dermis. Venous hypertension, exercise, and gravitational dependency are
important cofactors that appear to influence disease presentation. There is
perivascular T-cell lymphocytic infiltrate centered on the superficial small
blood vessels of the skin, which show signs of endothelial cell swelling and
narrowing of the lumen.
Characteristic small, reddish macules that vary in shape
and distribution, ranging from 2 mm to more than 2 cm, and found
predominantly on the buttock and lower extremities. Biopsy demonstrates
capillary telangiectasis, pericapillary infiltration of mononuclear cells,
erythrocyte extravasation into superficial dermal connective tissue, and
(rare) hemosiderin deposition into macrophages.
Although this disease has a benign nature, it
must be distinguished from other systemic purpuric diseases.
Check cell blood count to rule out
thrombocytopenia and anemia.
Per procedure. There are no case reports
of anesthetics in patients with this disease.
No known pharmacological
implications. However, pigmented purpuric dermatoses may show
hypersensitivity to drugs such as carbamazepine, furosemide, and
Schamberg Disease: Pigmented purpuric dermatitis that may occur
at any age. It is a chronic discoloration of the skin that usually affects the
legs and spreads slowly. Leaky blood vessels allow red cells to extravasate
into the skin. It gives an appearance of “cayenne pepper" spots because of
the hemosiderin deposits. Some itching may be present. It was
described by J. Schlamberg in 1901.
Gougerot Blum Disease: Mainly affects men between 40 and 50
It is characterized by a peculiar form of hemorrhagic pigmentary
and lichenoid plaques on the legs. Rust-colored papules, purpuric and
telangiectatic. Both sexes affected; prevalent in females, onset in middle
life. First described by H. Gougerot, French dermatologist, in 1925.
Lichen Aureus Disease: Similar to Majocchi disease because of
its increased incidence in children and young adults.
yellowish/red papules or patches appearing bilaterally on the lower limbs;
also be seen on the trunk and upper limbs. Usually responds to high-dose
Nichamin SJ: Chronic progressive pigmentary purpura: Purpura annulares
telangiectodes of Majocchi-Schamberg. Am J Dis Child