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Purpura annularis telangiectodes of Majocchi

  • Author(s): Hale, Elizabeth K, MD
  • et al.
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Purpura annularis telangiectodes of Majocchi
Elizabeth K Hale MD
Dermatology Online Journal 9(4): 17

From the Ronald O. Perelman Department of Dermatology, New York University

Abstract

A case is presented of a man with a 3-year history of ulcers in the setting of pigmented, annular and purpuric lesions of the lower extremities. A skin biopsy suggested a diagnosis of purpura annularis telangiectodes of Majocchi. First described in 1896 by Majocchi [1], purpura annularis telangiectodes is an uncommon pigmented purpuric eruption, which is characterized by symmetrical, purpuric, telangiectatic, and atrophic patches with a predilection for the lower extremities and buttocks. Histopathology and immunopathogenesis of this disease are similar to the other subtypes of pigmented purpuric dermatoses.



Clinical summary

History.—A 41-year-old man was presented to the Charles C. Harris Skin and Cancer Pavilion in December 2001 with a 3-year history of a problem on his lower extremities. He was referred for evaluation of ulcers on his feet and a pruritic eruption on his legs. He had been discharged from Staten Island Hospital 2 weeks earlier after receiving treatment for a lower-extremity cellulitis. According to the patient, the ulcers developed at the sites of the skin eruption and have waxed and waned for approximately 3 years. Past medical history was unremarkable.

Physical examination.—A violaceous, nonblanchable, 3-cm, annular plaque was present on the right dorsal foot, with a central superficial erosion. A slightly smaller annular plaque was noted on the left medial malleolus. Mottled violaceous and hyperpigmented patches were present on the lower extremities.


Figure 1 Figure 2

Laboratory data.—A complete blood count, erythrocyte sedimentation rate, cryoglobulins, antinuclear antibodies, and complement levels were normal or negative.

Histopathology.—There is a perivascular and predominantly bandlike infiltrate of lymphocytes that extend to the overlying epidermis, where there is vacuolar alteration of the basal layer and spongiosis. Numerous extravasated erythrocytes and scattered hemosiderin-laden histiocytes are present within the lichenoid infiltrate.

Diagnosis.—Purpura annularis telangiectodes of Majocchi.


Comment

At least five different subtypes of pigmented purpuric dermatoses have been described. Clinical overlap between the various subtypes may occur. First described in 1896 by Majocchi [1], purpura annularis telangiectodes is a pigmented, purpuric eruption that occurs most commonly in adolescents and young adults. The disease is characterized by symmetrical, purpuric, telangiectatic, and atrophic patches, with a predilection for the lower extremities and buttocks. Individual lesions coalesce to form annular patches and plaques, which range from 2-20 mm in diameter and do not blanch with pressure. Although primarily annular, these patches may be linear, stellate, or serpiginous in shape [2]. The lesions may persist for years and often develop central atrophy. Pruritus is usually absent or mild.

The cause of purpura annularis telangiectodes is unknown. One hypothesis implicates the effects of gravity and elevated venous pressures, as the lesions predominantnly occur on the lower extremities and buttocks [2]. It has also been postulated that Langerhans-cell mediated injury and immune-complex deposition result in capillary leakage [3]. Each subtype of pigmented purpuric eruptions examined with a panel of immunophenotypic markers showed a T-cell infiltrate irrespective of the histologic pattern. These observations suggested a common pathogenesis of vascular damage in the setting of a localized cell-mediated immunologic event [4].

There is no consistently successful therapy for purpura annularis telangiectodes. Supportive measures to decrease venous stasis, which include leg elevation and compression stockings, may be helpful. Topical glucocorticoids may be useful in patients with an eczematous or pruritic component. Anecdotal treatments include griseofulvin [5], pentoxifylline, ascorbic acid, and PUVA photochemotherapy.

References

1. Majocchi D. Sopra una dermatosis non ancora descritta, "purpura annularis telangiectode". G Ital Mal Venereol 1896;31:263-4.

2. Kim HJ, et al. Pigmented purpura over the lower extremities: purpura annularis telangiectodes of Majocchi. Arch Dermatol 1998;134:1477.

3. Newton R, Raimer S. Pigmented purpuric eruptions. Dermatol Clin 1985; 3:176-9.

4. Smoller BR, Kamel OW. Pigmented purpuric eruptions: immunopathologic studies supportive of a common immunophenotype. J Cutan Pathol 1991; 18:423-7.

5. Tamaki K, et al. Successful treatment of pigmented purpuric dermatosis with griseofulvin. Br J Dermatol 1995;132:159-60.

© 2003 Dermatology Online Journal