Majocchi Disease

At a glance

It is a rare idiopathic disorder characterized by pigmented purpuric dermatitis caused by chronic inflammation of capillaries. It is defined as a chronic disease of unknown etiology and associated with distinctive clinical appearance (extravasation of erythrocytes in the skin with marked hemosiderin deposition). Clinically, the presence of bluish red 1- to 3-cm annular skin patches that results from dark red telangiectases and petechiae with a predilection for the lower extremities and buttocks.

Synonyms

Purpura Annularis Telangiectodes; Pigmented Purpuric Eruption.

Incidence

Uncommon disease that occurs predominantly in children and young adults; seems to affect males more frequently. During a 10-month period, 10 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.

History

It was first described in 1896 by Majocchi.

Genetic inheritance

There is no genetic association identified.

Pathophysiology

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.

Diagnosis

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.

Clinical aspects

Although this disease has a benign nature, it must be distinguished from other systemic purpuric diseases.

Precautions before anesthesia

Check cell blood count to rule out thrombocytopenia and anemia.

Anesthetic considerations

The anesthesia plan would be determined by the possible underlying medical condition that can be present at the time of the procedure. There are no case reports of anesthetics in patients with this disease.

Pharmacological implications

No known pharmacological implications. However, pigmented purpuric dermatoses may show hypersensitivity to drugs such as carbamazepine, furosemide, and nitroglycerin.

Other conditions to be considered

• 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 ...