Schamberg's Disease Research Articles

Characteristics and Clinical Manifestations of Pigmented Purpuric Dermatosis.

BackgroundPigmented purpuric dermatoses (PPD) are a spectrum of disorders characterized by a distinct purpuric rash. Although PPD can be easily diagnosed, the disease entity remains an enigma and a therapeutic challenge.ObjectiveThe purpose of this study was to investigate the characteristics and clinical manifestations of PPD and to elucidate the relationship between assumed etiologic factors and the clinical manifestations of PPD and treatment responses.MethodsRetrograde analyses were performed to identify appropriate PPD patients who visited Korea University Medical Center Anam Hospital from 2002 to 2012.ResultsInformation on 113 patients with PPD was analyzed, and 38 subjects with skin biopsy were included for this study. Schamberg's disease was the most frequent clinical type (60.5%). Concomitant diseases included hypertension (15.8%), diabetes (10.5%), and others. Associated medication histories included statins (13.2%), beta blockers (10.5%), and others. Possibly associated etiologic factors were recent upper respiratory infection (5.3%), high orthostatic pressure due to prolonged standing (2.6%), and strenuous exercise (2.6%). A total of 36 patients (94.7%) were treated with one or more treatment methods, including oral antihistamines, pentoxifylline, topical steroids, and/or phototherapy. There was no significant difference in disease progress according to underlying diseases, medications, or association factors (p>0.05).ConclusionOur overall results were grossly consistent with the existing literature, excluding several findings. Although a possible relationship between PPD and cardiovascular disease or cardiovascular medication was proposed at the beginning of the study, no statistically significant correlations were found according to the specific clinical types and treatment responses (p>0.05).

Early treatment with rutoside and ascorbic acid is highly effective for progressive pigmented purpuric dermatosis.

Progressive pigmented purpuric dermatosis (PPPD, Schamberg disease) is a rare benign, but chronic dermatosis frequently misdiagnosed as vasculitis or bleeding disorder. Although affected patients experience significant impairment in quality of life no effective treatment has been established. The aim of our two center case series was to evaluate efficacy and tolerability of the antioxidants rutoside and ascorbic acid as combination treatment for PPPD. A retrospective review was performed on 35 patients with PPPD treated with 2 × 50 mg rutoside and 1,000 mg ascorbic acid daily between 2004 until 2011. The mean treatment duration was 8.2 months. 71.4% of the participants experienced complete clearance and 20.0% an improvement of more than 50%, accompanied by increased quality of life. Nine participants (25.1%) relapsed after discontinuation. In seven, rutoside and ascorbic acid was re-initiated, and all responded again. Only three participants reported mild adverse effects. Participants with shorter disease duration showed better therapeutic success, shorter time to response and lower risk of recurrence. Oral rutoside and ascorbic acid may be an efficient and well tolerated treatment for PPPD. Early treatment is recommended to achieve best clinical outcome.

The routine use of iron stain for biopsies of dermatoses of the legs

Our goal was to study the utility of the routine use of the Gomori iron stain in the evaluation of leg biopsies of inflammatory lesions. One hundred consecutive skin biopsies from the legs were evaluated with hematoxylin and eosin and Gomori iron stains. Iron positivity was semi-quantitatively graded from trace to 4+. Forty-two (42) cases were positive with the Gomori iron stain as follows: stasis dermatitis (14), Schamberg's disease (7), folliculitis (2), psoriasis (2), trauma (2), arthropod bite (2), allergic contact dermatitis (2), lichen simplex chronicus (1), senile purpura (1), vascular ectasia (1), lobular capillary hemangioma (1), scar (1), pretibial pigmented patches (1), tinea (1), lentigo maligna (1), traumatic fat necrosis (1), lichen planus (1) and fixed drug eruption (1). Twelve of 14 cases of stasis dermatitis had 2+ or greater iron staining; 8 cases were 3+ or 4+. All other diagnoses had trace or 1+ staining for iron except for one scar (2+). One of 19 samples (5.3%) of 'normal' leg skin controls showed iron stain positivity, and was graded as trace. After correcting for iron staining in 5.3% of normal leg skin controls, we observed Gomori iron positivity in 36.7% of 100 consecutive leg biopsies. We suggest that routine application of an iron stain on biopsies of dermatoses from the legs is useful. Stasis dermatitis and Schamberg's disease are the most frequent iron positive diagnoses, and the diagnosis is aided by the pattern of hemosiderin deposition. Negative iron stain is also useful in suggesting the presence of melanin pigment in macrophages in lichenoid and other dermatoses. To confirm a truly negative iron stain, study of the section at ×400 rather than ×100 is recommended.

Capillarite purpurique chronique diffuse du petit enfant

Pigmented purpuric dermatosis (or Schamberg's disease) is characterized by chronic macular purpura and capillaritis. It is more common in young adult males and adolescents and is generally localized on the lower limbs. In this article, we report on five young children with generalized Schamberg's disease. Five children (aged 13 months to 5 years) were included in this retrospective study. Time to consultation delay ranged from 15 days to 1 year. All patients presented asymptomatic generalized macular purpura. Skin biopsies were performed in 4 cases and were characteristic. The results of coagulation tests and complete blood counts were within the normal range in all patients. The clinical course was chronic, with periods of improvement and worsening. No treatment was prescribed. Schamberg's disease is uncommon in childhood. Our observations suggest that this diagnosis is not exceptional. Clinical appearance, setting and normal blood count values are sufficient to enable a diagnosis to be made. The clinical course is generally chronic, and as yet no treatments have demonstrated efficacy.

Clinical and laboratory findings of pigmented purpuric dermatoses.

BackgroundPigmented purpuric dermatoses (PPD) are chronic, recurrent group of disorders characterized by petechial and pigmentary macules usually localized on the lower limbs. Its etiopathogenesis is unknown. There are very few clinical and etiological studies on PPD in the literature.ObjectiveWe aim to examine the etiopathogenetic factors of PPD retrospectively.MethodsDemographic characteristics, history of co-morbid disorders and drug usage, hepatitis markers, levels of serum lipids, findings of Doppler ultrasonography in lower extremities, and patch test results of the 24 patients of PPD were examined retrospectively. The patch test results, history of drug use, and co-morbid disorders of the patients were compared with those of the control groups.ResultsThe male-to-female ratio was 1 : 2, and 83.3% of the patients had Schamberg disease. Seventeen patients had co-morbid disorders and 16 used various drugs, but there was no statistically significant difference between the controls and patients. One patient was positive for hepatitis B surface antigen and 1, for anti-hepatitis C virus antibody. Nine had elevated total cholesterol levels, and 5 had elevated triglyceride levels. Further, 30% of them were positive for at least 1 allergen, while 16% of the control subjects were positive for at least 1 allergen, but statistically significant difference was not found between the 2 groups. Variable degrees of venous insufficiency were detected in 75% of the patients on Doppler ultrasonography of the lower extremities.ConclusionVenous insufficiency and hypercholesterolemia might be the basic predisposing factors for PPD. Further studies are needed to show if diabetes mellitus and hypertension may cause perivascular inflammation in PPD.

Schamberg disease with plantar involvement

Schamberg disease with plantar involvement

Generalized Schamberg Disease in a Child

Dear Editor: Schamberg disease is a type of pigmented purpuric dermatosis that is characterized by red-brown purpuric macules that are known as 'cayenne pepper' spots. It usually develops insidiously on one or both lower legs and onset occurs on average in the fifth decade of life. Herein, we report a generalized form of Schamberg disease that occurred in a child. A 12-year-old boy presented with a 2-month history of an asymptomatic widespread rash. There was no specific history such as recent drug intake. He had been treated previously with topical steroids, but there had been no significant change in his condition. Physical examination revealed purpuric and telangiectatic macules and patches scattered all over his trunk and extremities (Fig. 1). Laboratory findings, including clotting profile, were within normal limits. Histological examination of a skin biopsy revealed extravasated red blood cells and enlarged vessels with perivascular lymphocytic infiltrates in the upper dermis (Fig. 2). Based on these clinical and histopathological findings, the patient was diagnosed with Schamberg disease. After treatment with oral prednisolone (10 mg) and pentoxifylline (400 mg) daily for 4 weeks, the skin lesions improved, leaving hyperpigmentation. Fig. 1 Physical examination revealed purpuric and telangiectatic macules and patches scattered all over his trunk (A; inset: a close-up new) and extremities (B). Fig. 2 Skin biopsy revealed extravasated red blood cells and enlarged vessels with perivascular lymphocytic infiltrates in the upper dermis (A: HE B: H&E, ×200). The onset age in the present case was interesting as most cases occur in adulthood. However, some authors have suggested that Schamberg disease is relatively common in children, sometimes occurring before puberty1. Torrelo et al1. reviewed 13 cases of Schamberg disease that occurred before 10 years of age and found that it predominantly occurred in girls, consistently involved the lower limbs, and was a chronic persistent disease. A case of a 1-year-old infant with Schamberg disease has also been reported2. An interesting feature of the present case was that the eruptions were generalized. The lesions in Schamberg disease usually develop on one or both lower legs, and they sometimes spread and involve the trunk or upper extremities. Three cases of generalized Schamberg disease have been reported, all of which occurred in children3,4. In these three cases, the generalized lesions responded well to psoralen plus ultraviolet A or narrow band ultraviolet B therapy and treatment resulted in clearing of the lesions after either the tenth, twelfth, or twentieth treatment session. The present case improved after treatment with an oral corticosteroid and pentoxifylline. These results indicate the benign nature of generalized Schamberg disease in childhood. Corticosteroids are well known anti-inflammatory agents that have shown positive outcomes in several cases of Schamberg disease. The administration of pentoxifylline can have several effects, including decreased fibrinogen concentration, and reduced platelet adhesiveness. In addition, it inhibits T-cell adherence to endothelial cells and keratinocytes. Childhood Schamberg disease needs to be distinguished from other dermatoses such as drug hypersensitivity reactions, Henoch-Schonlein purpura, and thrombocytopenia associated with viral infection. Dermoscopic examination can be used for the diagnosis of pigmented purpuric dermatosis5, as findings will show purpuric globules over a purpuric and orange-brown background. In conclusion, we reported a case of generalized Schamberg disease occurring in a child. Accurate diagnosis of pigmented purpuric eruptions in childhood is needed considering the benign nature of this condition.

Pigmented Purpuric Dermatosis Associated with Primary Antiphospholipid Syndrome

Pigmented purpuric dermatosis (PPD) is a group of chronic disorders characterized by the extravasation of erythrocytes and marked hemosiderin deposits in the dermis. Schamberg disease is a subtype of pigmented purpuric dermatosis in which nonpalpable purpura is prominent on the lower extremities. No cases of primary antiphospholipid syndrome and pigmented purpuric dermatosis have so far been reported in the literature. We herein report a case of pigmented purpuric dermatosis associated with primary antiphospholipid syndrome.

Clinicoepidemiological study of pigmented purpuric dermatoses

Background:Pigmented purpuric dermatoses (PPD) are a group of vascular disorders with varied manifestations which cause concern and are resistant to treatment. The literature is still lacking in clinicoepidemiological studies.Aim:To study the epidemiology, etiological, host and environmental factors, clinical manifestations, its variations, and the type prevalent in this part of the world.Materials and Methods:All cases of PPD were selected for the study from Skin and Venereal Disease, Out Patient Department between January 2008 and June 2009. Their history, examination, hematological investigations, and, in a few, histopathology findings were also recorded and data obtained were evaluated statistically.Results:There were 100 cases of PPD of total 55 323 patients (0.18%). There were 79 males and 21 females between 11 and 66 years. They were working as police men, security guards, barber, chemist, teachers, students, farmers, businessmen, and housewives. In a majority, there was a history of prolonged standing in day-to-day work. Purpuric, brownish pigmented, lichenoid or atrophic lesions were seen depending upon the type of PPD on lower parts of one or both lower limbs. Blood investigations were normal. Schamberg's disease was seen in ninety five, Lichen aureus in three, lichenoid dermatosis and Majocchi's disease in one case each.Discussion:Three clinical types of PPD were diagnosed which may represent different features of the same disease. Cell-mediated immunity, immune complexes, capillary fragility, gravitational forces, venous hypertension, focal infection, clothing, contact allergy to dyes, and drug intake have been incriminating factors in the past. Patient's occupation and environmental factors may also be considered contributory in precipitating the disease.Conclusions:The study revealed the problem of PPD in this geographical area, its magnitude, clinical presentation, the type prevalent, and possible aggravating factors to be kept in mind while managing the disease.

Successful treatment of generalized childhood Schamberg's disease with narrowband ultraviolet B therapy

Pigmented purpuric dermatoses are a group of dermatoses characterized by petechia and hyperpigmented macules that occur predominantly on the lower extremities. Although it is most commonly seen in adults, this disease can also affect children. Phototherapy has been shown to be effective in some cases in the literature. Here, we present two cases of Schamberg's disease that improved rapidly with narrowband ultraviolet B (UVB) therapy. To our knowledge, they were the first pediatric cases of successful narrowband UVB therapy in this disease.

Treatment of pigmented purpuric dermatoses with Narrow‐band UVB: a report of six cases

Pigmented purpuric dermatoses (PPD) are a group of chronic disorders that pose a therapeutic challenge. In this study, we evaluated the effectiveness of narrow band ultraviolet B (NB UVB) in the treatment of PPD. Six patients of PPD were treated with NB UVB: one patient had Majocchi's purpura and five had Schamberg's disease. NB UVB was given on three weekly basis till clearance, then maintenance treatments were scheduled as twice weekly for 3 weeks and once weekly for another 3 weeks. The patients were followed up to 1 year. Successful treatment was achieved in all treatment patients after 24-28 NB UVB treatments and maintenance of nine treatments. Only two patients showed flare of their lesions after stoppage of NB UVB and efficiently controlled with further 14 NB UVB treatments. NB UVB is effective in the treatment of PPD and should be considered as a treatment option for PPD.

The Successful Treatment of Schamberg's Disease with the 595 nm Vascular Laser

The Successful Treatment of Schamberg's Disease with the 595 nm Vascular Laser

Two Cases of Schamberg's Disease Concomitant with Arthropathy of the Legs

Two Cases of Schamberg's Disease Concomitant with Arthropathy of the Legs

自家製桂枝茯苓丸が著効したＳｃｈａｍｂｅｒｇ病の１例

自家製桂枝茯苓丸が著効したＳｃｈａｍｂｅｒｇ病の１例

Schamberg disease: uncommon reaction to a common drug

A 75-year-old woman with long-standing rheumatoid arthritis presented with a mildly pruritic, symmetrical erythematous rash on her legs ([Figure 1][1]). She was not taking any specific therapy for her rheumatoid arthritis but had taken acetaminophen for 3 days because of knee pain. She noticed the

Aminaphtone in the control of Schamberg's disease

The aim of this case report is to describe control of Schamberg's disease using aminaphtone. We report on the case of a 28-year-old patient who presented with multiple purpuric lesions of the lower extremities which had appeared spontaneously. A biopsy of the skin was performed that showed a perivascular T-cell lymphocytic infiltrate centered on the small superficial blood vessels of the skin and so a diagnosis of Schamberg's disease was reached. The patient was prescribed corticoids and the lesions disappeared however on suspension of the medication the lesions re-emerged within three to seven days. This treatment was unsuccessfully continued for more than one year. Thus another therapeutic option was attempted: 75 mg of aminaphtone was prescribed twice daily for one month and the purpuric lesions disappeared within about one week. One year after suspending the medication no relapse of the purpura was observed.

A case of Schamberg's disease responding dramatically to PUVA treatment

Pigmented purpuric dermatoses are a group of chronic, recurrent disorders characterized by purpuric lesions mainly involving the lower extremities. Their etiology is unknown. Treatment options are limited and none of them have proven benefit. Phototherapy has been reported to be effective in a small number of patients in the literature. We present a case of Schamberg's disease showing a dramatic response to psoralen plus ultraviolet A therapy and discuss the current therapeutic options focusing mainly on phototherapy. We believe that phototherapy is a valuable alternative, especially for patients with long-standing and widespread pigmented purpuric dermatitis.

Narrowband UVB therapy as an effective treatment for Schamberg's disease

A 33-year-old man presented with a 3-month history of a widespread pigmented purpuric eruption over his trunk and limbs. The clinical presentation and histology were consistent with a diagnosis of Schamberg's disease. The rash initially cleared following a short course of oral prednisolone at 25 mg daily for 3 weeks, which was weaned over the subsequent 4 weeks. Topical mometasone furoate ointment 0.1% daily was also applied to active areas. The rash recurred when prednisolone was reduced to below 5 mg per day. To prevent a further recurrence with weaning prednisolone, narrowband UVB therapy was commenced three times per week. The patient was continued on UV therapy over the next 5 months. The rash would flare after 2 to 3 weeks without treatment. The frequency of UV therapy was weaned and the patient remained clear on as little as one treatment every 2 weeks. Any further reduction, however, was associated with a recurrence. Narrowband UVB therapy should be considered for difficult or persistent cases of pigmented purpuric eruption.

Capillarite purpurique généralisée traitée par PUVAthérapie chez un enfant

Chronic pigmented purpuric dermatitis includes various entities seen above all in adults, although they may occasionally appear in children. The various therapies available are generally unsuccessful. We report the case of a child who responded dramatically to PUVA therapy. A 10-year-old Caucasian boy of phototype III was evaluated for a one-month history of progressive eruption on the upper extremities, followed by spread to the trunk and the lower extremities. On examination, he had generalized red-yellow lesions with "cayenne-pepper" spots. Extracutaneous examination showed no abnormal features. A skin biopsy showed a superficial, perivascular mononuclear inflammatory infiltrate with extravasations of red blood cells. The laboratory findings were normal. PUVA therapy was given three times weekly. After 4 weeks of PUVA (21 J/cm2) the lesions cleared up. The patient was still free of lesions after 3-years of follow up. The clinical and histological findings in our case were consistent with Schamberg's purpura, a rare disease but nevertheless the most common form of pigmented purpura in children. Schamberg's purpura in children is a chronic disease that can persist for up to 7 years in the absence of treatment, although spontaneous remission may occur within 1 to 4 years. Phototherapy with PUVA and UVB-TL01 has been shown to be efficacious in various forms of pigmented purpuric dermatosis in adults, but only in isolated cases. To our knowledge, this is the first pediatric case of successful PUVA therapy in this disease.

Pigmented purpuric dermatosis (capillaritis) induced by bezafibrate

To the Editor: Pigmented purpuric dermatoses (PPD) are a group of chronic, persistent purpuric, variably pruritic eruptions occurring predominantly in men on the lower limbs, often in association with changes of chronic venous hypertension. PPD encompass the entities of Schamberg's disease, Gougerot–Blum disease, Majocchi's disease (purpura annularis telangiectoides), “itching purpura” (eczematid-like purpura), lichen aureus, unilateral linear capillaritis, and granulomatous pigmented purpuric dermatosis.