The treatment of progressive pigmented purpura with ascorbic acid and a bioflavonoid rutoside

by Fred Laufer

Abstract

Progressive pigmented purpura (PPP) is a group of dermatoses that are benign and usually self-limited. However, they may persist for months or years with frequent recurrences. Numerous treatments have been tried, but no effective therapy has yet proven to be successful. This report documents the treatment of a patient with one subtype of PPP using ascorbic acid and a bioflavonoid rutoside given orally. A 42-year-old male with biopsy-proven Eczematoid-like Purpura of Doucas and Kapetanakis was treated with ascorbic acid 500 mg twice a day and a bioflavonoid rutoside 50 mg twice a day. Treatment was successful, with quick defervescence of the rash and no recurrence to date.

Introduction

Progressive pigmented purpura (PPP) is a group of dermatoses that consist of 5 subtypes. These subtypes are usually known as progressive pigmented purpura (Schamberg's Disease), eczematoid-like purpura of Doucas and Kapetanakis, purpura annularis telangiectodes (Majocchi's Disease), lichen aureus and lichenoid dermatosis of Gougerot and Blum. Although these subtypes are sometimes distinguished from each other by their appearance, symptoms (such as itching), or histological findings, they probably represent a varied presentation of the same disease.

The etiology of PPP is unknown, although immune mechanisms are thought to be involved. (1,2) Rare familial cases of Schamberg and Majocchi Diseases have been reported, implying a genetic cause in a few patients. Venous hypertension, exercise, and dependence of the lower extremities can influence the presentation of the disorder. Histologically, the subtypes of PPP are similar, and reveal a nonspecific capillaritis, consisting of perivascular lymphocytic infiltrates, extravasation of erythrocytes, and hemosiderin deposition. (3)

Case Report

A healthy 42-year-old male presented with an asymptomatic rash of 2 weeks duration. The patient was on no medication, and his pertinent history and review of systems revealed no etiologic factors. On examination, the rash was noted to extend from the ankles, up the legs, to the buttocks and lower abdomen. The most pronounced areas of the rash, however, were the posterior thighs and buttocks (Figure 1). The rash consisted of numerous petechiae from 1 to 2 mm in diameter to large areas of coalescence that formed plaques. The individual petechiae were orange-red in color, non-blanchable, and non-palpable. The rash was non-pruritic. The remainder of the physical examination was unremarkable.

Laboratory studies were obtained, which included a chemistry panel, CBC with differential and platelet count, erythrocyte sedimentation rate, urinalysis, antinuclear antibodies, antineutrophil cytoplasmic antibodies, and prothrombin time. The results of these tests were normal. Ten days after his initial presentation, the patient returned for follow-up. The rash was unchanged. A skin biopsy of the thigh was obtained and sent for histological evaluation. This was reported as: "Negative for vasculitis. Consistent with pigmented purpuric dermatitis. Histologic changes most consistent with eczematoid purpura of Doucas and Kapetanakis rather than Schamberg's Disease, due to the presence of focal spongiosis and scale crust."

The patient was seen again 2 weeks after his initial presentation. His rash appeared worse, with larger areas of plaques and crusting, and darkening of pigmentation (Figure 2). At this point, with the diagnosis of Progressive Pigmented Purpura confirmed, the patient was started on a course of oral therapy. This consisted of ascorbic acid (vitamin C) 500 mg twice a day and a bioflavonoid rutoside 50 mg twice a day.

The patient was followed for 4 weeks on this treatment with significant improvement in his rash (Figure 3). After 6 additional weeks of treatment, or 3 months after initial presentation, the rash was resolved and treatment was discontinued. The only residual sign of the rash was mild post-inflammatory hyperpigmentation of the upper thighs and buttocks, which later faded (Figure 4).

While undergoing treatment, the patient remained asymptomatic and had no side effects or reactions to the ascorbic acid or rutoside. At 8 months after presentation, the patient has had no recurrence of the rash.

[FIGURE 1 OMITTED]

Discussion

Although PPP is a benign, self-limited disorder, it can be a distressful experience for the patient. It can last months or years, affecting the appearance and, in some cases, be associated with intense itching and lichenification of the skin. This can be very disconcerting to those suffering from this condition. In addition, PPP can mimic more serious disorders, and these must be quickly ruled out. For example, PPP may be confused with a cutaneous vasculitis, a drug reaction or early cutaneous T-cell lymphoma. For this reason, a skin biopsy is usually recommended as part of the initial evaluation of this disorder.

There are some general characteristics for each of the subtypes of PPP. However, variations do occur. Schamberg's Disease is the most common subtype. It can occur at any age, is usually asymptomatic, and generally forms "cayenne pepper" spots on the legs. Eczematoid-like purpura of Doucas and Kapetanakis is more common in adult males, is more generalized, sometimes involving the trunk and arms, and can be intensely pruritic. Majocchi's Disease usually occurs in children or adolescents and is characterized by small, annular plaques of purpura that contain telangiectasia. Lichen aureus also occurs in children and adolescents, is usually unilateral, appears most commonly on the leg, and consists of a solitary lesion or group of lesions that are golden in color with purpura. Lichenoid dermatosis of Gougerot and Blum presents as lichenoid papules or plaques, with purpura, on the legs.

[FIGURE 2 OMITTED]

When the patient in this case study was initially diagnosed with PPP, it was thought that he had Schamberg's disease. This was due to the fact that the rash was mainly localized to the legs and buttocks, and there was no pruritus. However, the skin biopsy turned out to reveal eczematoid-like purpura of Doucas and Kapetanakis. This supports the theory that these 5 subtypes of PPP are all different manifestations of the same disorder. Consequently, the treatment of PPP is not dependent on which subtype is involved.

No specific therapy has yet to be established for the treatment of PPP. Antihistamines and topical and oral corticosteroids have been used to reduce pruritus. Leg elevation and compression stockings have been used to reduce venous stasis and edema. Topical tacrolimus was used in a 30-year-old woman with good control of her PPP. (4) Griseofulvin, (5) pentoxifylline, (6,7) cyclosporine, (8) and PUVA (9) have been used successfully in a few patients. However, considering the benign nature of PPP, these treatments may not be justified due to their potential side effects.

[FIGURE 3 OMITTED]

The combination of ascorbic acid with a bioflavonoid has also been tried as a treatment for PPP. In a study by Reinhold et al, (10) 3 patients were treated orally with ascorbic acid (500 mg twice a day) along with a bioflavonoid rutoside (50 mg twice a day). All 3 patients had rapid, marked improvement in their rash with complete clearing of skin lesions after 4 weeks of treatment. There were no recurrences after 3 months of treatment.

The mechanism by which these 2 drugs work in treating PPP is thought to be their effect on capillary stability. Bioflavonoids reduce capillary permeability and fragility, inhibit elastase and hyaluronase, and inhibit leukocyte activation. (11,12) A form of bioflavonoid, known as a rutoside, is available without prescription (One such product is Venastat[R]). As the active ingredient in horse chestnut seed extract, rutosides are used to treat the symptoms of varicose veins and venous insufficiency. Ascorbic acid (Vitamin C) has antioxidant effects and may also reduce permeability of endothelial cells. (13,14) Ascorbic acid is also available without prescription.