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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 hemosiderin deposition).

Purpura Annularis Telangiectodes; Pigmented Purpuric Eruption.

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 practice that serves a population of 300,000 persons. Five patients were diagnosed with 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 nitroglycerin.

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 years old. It is characterized by a peculiar form of hemorrhagic pigmentary dermatosis 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. Characterized by yellowish/red papules or patches appearing bilaterally on the lower limbs; can also be seen on the trunk and upper limbs. Usually responds to high-dose steroids.

Nichamin SJ: Chronic progressive pigmentary purpura: Purpura annulares telangiectodes of Majocchi-Schamberg. Am J Dis Child 116:429, 1968.  [PubMed: 5697979]

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