Dermatology Online Journal

Progressive pigmentary purpura
Jeremy A Brauer MD, Jyoti Mundi MD, Julie Chu MD, Rishi Patel MD, Shane Meehan MD, Alan H Greenspan MD, Jennifer Stein MD PhD
Dermatology Online Journal 17 (10): 14

Department of Dermatology, New York University, New York, New York

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Abstract

A 58-year-old man presented for evaluation and treatment of non-tender, non-pruritic, annular patches on the right temple and frontal aspect of the scalp that reddened with exercise. A biopsy specimen showed a purpuric dermatitis with features of lymphocytic vasculitis; a diagnosis of exercise-induced progressive pigmentary purpura was made. Whereas progressive pigmentary purpura is purported to be caused by exercise, other similar appearing entities are associated with exercise, namely exercise-induced vasculitis (EIV). EIV may be considered as an acute microcirculatory deficiency and thermoregulation decompensation that occurs after episodes of exhaustive major muscular activity or after unusual or excessive exercise. The combination of age greater than 50 years, heat, and prolonged exercise are the most potent contributing factors. This is the first report of exercise-induced progressive pigmentary purpura.

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History

A 58-year-old man presented to the Charles C. Harris Skin and Cancer Pavilion for evaluation and treatment of non-tender, non-pruritic macules on the right temple and frontal aspect of the scalp that have been recurring for the last eighteen months. Upon conclusion of vigorous exercise, he noted worsening and reddening of the lesions, which typically resolve several days later. The patient regularly runs and practices yoga. During yoga positions that include standing on his head for prolonged periods of time, he has noted exacerbation of skin lesions after approximately ten minutes. There has been no treatment. He denies fevers, chills, or sweats.

Past medical history included hypothyroidism and kidney stones. Medications include levothyroxine and potassium citrate. He has a drug allergy to sulfonamides. He denies smoking and drinks alcoholic beverages socially.

Physical examination

Figure 1	Figure 2

On the right temple were pink and brown annular patches with red puncta. On the frontal aspect of the scalp were non-blanching, erythematous and brown patches.

Laboratory data

None.

Histopathology

Figure 3

There is a superficial, perivascular infiltrate of lymphocytes, with scattered, extravasated erythrocytes and focal perivascular fibrin deposition.

Discussion

Pigmented purpuric eruptions represent a group of diseases that are characterized by purpuric or petechial lesions without systemic findings or associated coagulaopathy. Five clinical variants have been identified, which include progressive pigmentary purpura of Schamberg, pupura annularis telangiectodes of Majocchi, pigmented purpuric lichenoid dermatosis of Gougerot and Blum, eczematid-like purpura of Doucas and Kapetanakis, and lichen aureus. Clinically, lesions vary from the pinpoint, yellow-brown, cayenne pepper macules of Schamberg disease to the solitary, chronic rust-colored plaque or patch of lichen aureus. Histopathologic features include extravasted erythrocytes, endothelial-cell swelling, a perivascular lymphocytic infiltrate, and hemosiderin-containing macrophages, with additional findings among the individual subtypes. Whereas many reports of drug-induced, pigmented purpuric eruptions exist in the literature, the etiologies of these conditions are largely unknown but may include exercise.

Exercise-induced purpura, now known as exercise-induced vasculitis (EIV) is an entity to be considered in the differential diagnosis of progressive pigmentory purpura. This condition may be considered as an acute, microcirculatory deficiency and thermoregulation decompensation that occurs after episodes of exhaustive major muscular activity or after unusual or excessive exercise [1, 2]. These erythematous, urticarial, or purpuric patches and plaques almost exclusively are localized on the lower extremities and often spare areas of compression. The lesions typically resolve within several days to a week after the inciting event [1, 2]. The eruption may be asymptomatic or accompanied by burning, pain, or pruritus [1, 3].

Most often there are no associated medical conditions, systemic symptoms, or a relationship to sun exposure [1, 2, 4]. However, there are two reports of cases of exercise-induced vasculitis that are associated with celiac disease, dermatitis herpetiformis, autoimmune hepatitis, and Sjögren syndrome [5, 6] as well as a case of exercise-induced urticarial vasculitis related to a cystic teratoma [7]. Patients may or may not suffer from chronic venous disease [1, 2, 4]. Histopathologic features include a leukocytoclastic vasculitis, with erythrocyte extravasation and C3 and IgM deposits [1, 2, 5, 6].

Almost all reports of exercise-induced vasculitis occur in patients over the age of 50 [3], in warm environments [4], and involve aerobic activity, such as walking, jogging, running, and golfing [1, 4, 8, 9]. There is one report of involvement of the chest and back after intense basketball training [10]. There have been no reports of exercise-induced vasculitis of the scalp.

The aforementioned prolonged physical efforts increase venous return, with concurrent diminished effective venous drainage that results in stasis. Although most lesions spontaneously resolve within several days to a week, relapses are common and preventative measures, such as compression therapy, may prove beneficial [1, 2, 4]. Additionally, the use of vasoactive drugs, topical glucocorticoids, and colchicine also may partially prevent relapses [1, 2, 11].

References

1. Ramelet AA. Exercise-induced vasculitis. J Eur Acad Dermatol Venereol 2006;20:423 [PubMed]

2. Ramelet AA. Exercise-induced purpura. Dermatology 2004;208:293 [PubMed]

3. Kelly RI, et al. Golfer’s vasculitis. Australas J Dermatol 2005;46:11 [PubMed]

4. Veraart JCJM, et al. Influence of endurance exercise on the venous refilling time of the leg. Phlebology 1994;23:120

5. Knoell KA. Exercise-induced vasculitis associated with autoimmune disease. Cutis 2009; 83: 319 [PubMed]

6. Kano Y et al. Cellular and molecular dynamics in exercise-induced urticarial vasculitis lesions. Arch Dermatol 1998; 134:62 [PubMed]

7. Di Stefano F, et al. Exercise-induced urticarial vasculitis as a paraneoplastic manifestation of cystic teratoma. Rheumatology (Oxford) 2003;42:1418 [PubMed]

8. Prins M, et al. Leucocytoclastic vasculitis induced by prolonged exercise. Br J Dermatol 1996;134:915 [PubMed]

9. Mailler-Savage EA, Adams BB. Skin manifestations of running. J Am Acad Dermatol 2006;55:290 [PubMed]

10. Leung AKC, et al. Exercise-induced purpura. J Sports Med Phys Fitness 1990; 3:329 [PubMed]

11. Garg A, et al. Relapsing leukocytoclastic vasculitis triggered by activity in a young woman. Arch Dermatol 2009; 145:601 [PubMed]

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