Schamberg's Disease Research Articles

Schamberg's Disease: A Comprehensive Review

Schamberg's disease, also known as progressive pigmented vasculitis, is a rare, chronic skin disorder characterized by petechiae and brown spots, mainly on the lower extremities. In this review article, we provide a detailed overview of Schamberg's disease. We review its causes, pathogenesis, and histopathological features. We also describe the clinical presentation, diagnostic methods, and treatment options. We emphasize the crucial role of a multidisciplinary approach in the treatment of this condition. We also emphasize the need for further research to better understand the mechanisms underlying Schamberg's disease. This could lead to the development of more targeted therapies in the future.

Therapeutic Approach in Pigmented Purpuric Dermatoses-A Scoping Review.

Pigmented purpuric dermatoses (PPD) encompass a group of chronic skin conditions characterized by the presence of petechiae, purpura, and pigmentation changes. While generally benign, these dermatoses can be persistent and aesthetically bothersome. Key clinical features include red to brownish patches with a distinctive "cayenne pepper" appearance, predominantly localized on the lower extremities, particularly the shins. Subtypes include Schamberg disease, Majocchi's disease, Gougerot-Blum disease, Ducas and Kapetanakis pigmented purpura, and lichen aureus. Diagnosis relies primarily on clinical evaluation of skin lesions, with biopsy as a confirmatory tool. Although the exact cause of PPD remains unclear, capillary fragility and red blood cell extravasation are implicated. Treatment strategies for PPD aim to alleviate symptoms, considering the generally benign and chronic nature of the condition. As there is no standardized treatment, various methods with varying efficacy are employed. After searching SCOPUS and PubMed databases, we assessed 42 original articles to present current knowledge regarding therapy of PPD. This review will compare treatment approaches specifically in Schamberg disease and other manifestations of pigmented purpuric dermatoses.

Schamberg's Purpura: A Comprehensive Review of Clinical Manifestations, Pathophysiology, and Management Strategies

Schamberg's Purpura, also known as progressive pigmented purpura or Schamberg disease, is a dermatological condition characterized by a distinctive purpuric rash. This article provides a thorough examination of the clinical manifestations, pathophysiological mechanisms, and current management approaches associated with this intriguing disorder. The review aims to consolidate existing knowledge, highlight diagnostic challenges, and explore emerging therapeutic interventions. Through an in-depth analysis of the latest research findings, this comprehensive overview aims to enhance the understanding of Schamberg's Purpura among healthcare professionals, facilitating more accurate diagnosis and improved patient outcomes.

Experience of successful use of intense pulsed light in the treatment of Shamberg patients

Schamberg's disease (chronic purpura pigmentosa) is the most common type of pigmentary purpuric dermatosis, most commonly seen in men and primarily affecting the areas of the tibia, thighs, buttocks, trunk, or upper extremities, with a classic presentation characterized by petechiae, purpura, and enhancement skin pigmentation. This is a benign disease that is often asymptomatic and requires treatment if there are associated symptoms or if the patient wishes to improve a cosmetic defect.
 Schamberg's disease is usually not difficult to diagnose, but its treatment can be challenging. The current standard of treatment for chronic purpura pigmentosa includes systemic and external drug therapy, correction of identified concomitant diseases that can maintain and worsen the course of cutaneous vasculitis. There are many other, including non-pharmacological, methods described in the literature, few of which have been shown to be effective. Considering the small number of studies on this topic, numerous exacerbations and the possibility of spontaneous resolution of the disease, patients’ concerns about residual pigmentation, the search for an optimal therapeutic approach remains an urgent task.
 This article describes the experience of using broadband pulsed light in the treatment of Schamberg disease in a 34-year-old man. The equipment settings, diagram and exposure protocol for broadband pulsed light are presented. As a result of a follow-up examination carried out 1 month after treatment, a positive dynamic of the therapeutic process was determined: there were no new rashes, individual foci of the rash regressed completely, the remaining elements of the rash paled significantly. At a follow-up examination, four months after the therapy, a stable treatment result was recorded.

The debut of Schamberg disease (a clinical case)

A group of pigmented purpuric dermatoses includes a list of skin diseases characterized by multiple petechial hemorrhages, purpura, and increased skin pigmentation (yellow, brown, or red patchy). Schamberg disease is the most common representative of this group with a benign, chronic and recurrent course of idiopathic origin. The lesions are often asymptomatic or associated with mild pruritus, usually occurring on the lower extremities. The diagnosis is not a dilemma, as it is made based on clinical examinations and identification of the classical rash morphology, but is also emphasizing the role of a wide range of specialists in the evaluation and treatment of this pathological condition. The aim of the study. To analyze a clinical case of the debut of Schamberg disease. Results. Considering the sporadic nature and rarity of the disease, the clinical case of Schamberg disease diagnosed in a 53-year-old man who visited a pediatrician concerning his child’s illness is presented. The probable cause of the disease debut was a complex trigger effect (a long-term use of diclofenac sodium, drinking alcohol on the eve). Conclusions. The report is aimed at drawing attention to Schamberg disease (Schamberg purpura) as a diagnostic and therapeutic challenge not only for general practitioners, internal medicine specialists but also for dermatologists, phlebologists, hematologists in particular.

Development of Schamberg's disease after SARS‐CoV‐2 vaccination

Schamberg's disease is one of a group of pigmented purpura diseases. We report a 43-year-old woman, who developed Schamberg’s disease after SARS-CoV-2 vaccination. Three days after vaccination, a purpuric eruption occurred on the lower extremities, extending to the buttocks, and subsided in 4 weeks. No recurrence was observed thereafter. It is suggested that the vaccination triggered the development of the eruption, which was not merely coincidental. SARS-CoV-2 vaccination may stimulate a certain population of T cells, resulting in the development of pigmented purpuric dermatoses.

CPC14: Histological and direct immunofluorescence pattern in Schamberg disease: an analytical study

CPC14: Histological and direct immunofluorescence pattern in Schamberg disease: an analytical study

Rutoside and Ascorbic Acid in the Treatment of Schamberg Pigmented Purpuric Dermatosis

Schamberg disease is a type of pigmented purpuric dermatosis (PPD), which is a benign cutaneous capillaritis characterized by macules and patches most commonly found on the lower extremities. Rutoside and ascorbic acid have been shown in previous cases to be efficacious in the treatment of PPD lesions due to their free radical scavenging effect on capillaries. We present the case of a 19-year-old woman with Schamberg disease who achieved complete clearance of lesions within four months of daily rutoside and ascorbic acid treatment. The goal of this case report is to further demonstrate the effectiveness of this treatment and to call for investigation of its use as a standard of care in PPD.

A Cross-Sectional Study of Clinico-etiological Profile and Associated Comorbidities in Indian Patients of Pigmented Purpuric Dermatoses.

Background:Pigmented purpuric dermatoses (PPDs) are a group of chronic benign vascular disorders with varied clinical presentation. The etiopathogenesis of the condition largely remains unknown with a paucity of clinico-epidemiological and/or clinico-etiological studies.Objective:To study the clinico-epidemiological pattern, etiological factors and associations of PPD and correlate them with its severity in a set of Indian patients.Materials and Methods:In a cross-sectional study, all clinically diagnosed and histopathologically confirmed cases of PPD attending the outpatient department of dermatology from November 2015 to October 2016 were included in the study. Patients were evaluated based on a detailed history of the illness, comorbid conditions, drug usage, general physical, systemic, and cutaneous examinations, severity of disease (mild, moderate, or severe), laboratory parameters, and Doppler ultrasonography of the lower extremities.Results:There were a total of 60 patients with a female-to-male ratio of 1.14:1. The mean age of patients was 47 ± 12.10 (range: 15–70) years. Majority (70%) of the patients were housewives, bankers, and businessmen. The possible etiological and/or aggravating factors included prolonged standing (28.3%), drug intake (13.3%), alcohol ingestion (10%), strenuous exercise (5%), and varicose vein (3.3%). Schamberg's disease (90%) was the most common type observed. The most common systemic comorbidity identified was hypertension (58.3%) followed by diabetes mellitus (31.6%) and dyslipidemia (28.3%). A positive correlation was found between severity of the disease and presence of comorbidities (Mantel–Haenszel method, P < 0.0001).Conclusion:PPD was found to be associated with a variety of disorders and comorbidities. The number of the comorbidities increased with increasing severity of the disease. Besides exposing the patient to various risk factors, this may contribute to the vessel wall damage seen in the condition. All patients with PPD should, therefore, undergo an initial screen for these comorbidities.

Schamberg Disease / Progressive Purpuric Dermatosis � Diagnosis and Management Challenges

Schamberg disease, also called progressive pigmented purpuric dermatitis is a rare disease characterized by pigmentary, non-palpable, progressive lesions with symmetrical distribution on lower extremities with vasculitic appearance. The etiology of this disease is unknown. Schamberg disease is still considered idiopathic, without an established therapy. Given the fact that this disease has many recurrences, management and treatment is a challenge for the dermatologist.

Schamberg Disease / Progressive Purpuric Dermatosis - Diagnosis and Management Challenges

Schamberg disease, also called progressive pigmented purpuric dermatitis is a rare disease characterized by pigmentary, non-palpable, progressive lesions with symmetrical distribution on lower extremities with vasculitic appearance. The etiology of this disease is unknown. Schamberg disease is still considered idiopathic, without an established therapy. Given the fact that this disease has many recurrences, management and treatment is a challenge for the dermatologist.

Pigmented purpuric dermatoses: analysis of epidemiological, clinical and histopathological aspects in relation to the prognosis of 70 patients, including infants and adults.

Pigmented purpuric dermatoses (PPDs) are a group of chronic-relapsing, inflammatory purpuras without vasculitis that typically involve the lower limbs. Five major types could be distinguished, according to the clinical and histopathologic analysis. The etiopathogenesis is still unknown and multiple factors have been considered. Prognosis seems not to be influenced by the different clinical forms of PPDs; nevertheless, no previous studies have investigated whether it could be influenced by the anatomical distribution of the disease. We enrolled 70 consecutive patients, including both adults and children with a clinical and histopathological diagnosis of PPD, to investigate possible correlations between the different types of PPDs, their anatomical distribution and prognosis. We observed that patients, both adults and children, with an uncommon localization of the dermatoses, in particular with a diffuse localization (more than one body area affected), presented more frequently a persistent form of PPDs. This is the first study that attempts to relate the anatomical extension of PPDs and their evolution over time. According to our analysis, it seems to be a statistical significance for the prognosis only for patients with Schamberg disease and involvement of lower limbs. However, studies on a larger population are needed.

The color of skin: brown diseases of the skin, nails, and mucosa

Brown diseases comprise disorders leading to hyperpigmentation in skin and nails. Melasma is an acquired skin disorder that is characterized by brownish macules that typically occur on the face. Schamberg disease, also known as progressive pigmented purpura, is characterized by brown pigmentation with pepper spots on their edges. We summarize the epidemiology, pathogenesis, histologic features, and treatment choices for additional brown diseases, including melasma, pigmented purpuric dermatoses, postinflammatory hyperpigmentation, drug-induced hyperpigmentation, and pigmentations due to systemic or physiologic conditions.

Contact sensitivity to textile dyes in patients with pigmented purpuric dermatosis

Purpose: The aetiology of pigmented purpuric dermatoses is unclear. Recent studies speculate about contact sensitivity to play a role in the aetiology of the disease. In most patients, the lesions begin and stay limited on the lower extremities as textile products are in tight contact with the skin. Also, textile dyes can cause contact dermatitis of purpuric type. This study was conducted in order to understand whether the contact sensitivity plays a critical role in the aetiology of pigmented purpuric dermatoses.Materials and methods: Patients with diagnosis of pigmented purpuric dermatoses were included in the study. Patch tests were applied at the back of all patients with textile series (Chemotechnique Diagnostics TF-1000) which have 33 allergens containing textile dyes, material and protectors by IQ Ultra Chamber. Test sites were evaluated at 48, 72, 96. h and on the 7th day according to the criteria of the International Contact Dermatitis Research Group. The evaluation results were recorded in patient files and the results were statistically compared.Results: Eighteen males (60%) and 12 females (40%), a total of 30 patients were included in the study. Schamberg disease in 23 patients (76.7%), lichen aureus in 5 patients (16.7%) and Majocchi disease in 2 patients (6.7%) were described out of total 30 patients. No contact sensitivity was detected in any of the patients.Conclusion: Contact sensitivity to textile dyes was not found as an etiologic factor in our study group.

Schamberg's disease; a forgotten dermatoses: A case report with review

Schamberg's Disease (SD) also known as progressive pigmented purpuric dermatitis is the most common PPD (Pigmented Purpuric Dermatitis). PPD includes a spectrum of vascular diseases with an incidence of 0.18% of all skin and VD lesions. SD has Idiopathic aetiology, affects all races and ages (with mean age of 34 years). Males are affected more, especially the legs showing reddish brown purpuric patch/macules which has a chronic slow progression to other body parts. Long standing medications are thought to be one of the precipitating factor. Extravasation of RBCs from the superficial venous plexus of skin is thought to be the cause of skin discoloration. Histopapathology (HP) of the skin lesion show mild epithelial hyperplasia, occasional foci of spongiosis and lymphohistiocytic infiltrate with extravasation of RBCs. The disease has a cosmetic problem. No malignant transformation has been documented yet and to date there is no effective treatment. It is usually diagnosed by Clinical suspicion followed by HP confirmation.

Gastrointestinal Nodules and Bleeding with Long-Term Lanthanum Use; DRESS and Hepatotoxicity Due to Rivaroxaban; Thrombocytopenia Induced by Pentoxifylline; Amlodipine-Induced Schamberg's Disease; Varenicline-Induced Acute Liver Injury.

The purpose of this feature is to heighten awareness of specific adverse drug reactions (ADRs), discuss methods of prevention, and promote reporting of ADRs to the US Food and Drug Administration's (FDA's) MedWatch program (800-FDA-1088). If you have reported an interesting, preventable ADR to MedWatch, please consider sharing the account with our readers. Write to Dr. Mancano at ISMP, 200 Lakeside Drive, Suite 200, Horsham, PA 19044 (phone: 215-707-4936; e-mail: mmancano@temple.edu). Your report will be published anonymously unless otherwise requested. This feature is provided by the Institute for Safe Medication Practices (ISMP) in co-operation with the FDA's MedWatch program and Temple University School of Pharmacy. ISMP is an FDA MedWatch partner.

幼児の Schamberg 病の1 例

3 歳，女児。9 カ月前に背部に 1∼2 cm の紅斑が出現し，1 カ月後には四肢や体幹に拡大した。初診時，全身に淡紅斑，淡赤褐色斑が散在しており，斑内には点状出血が混在していた。病理組織所見と合わせ，Schamberg 病と診断した。成人の Schamberg 病と異なり，乳幼児の症例では皮疹の分布が下肢のみでなく，上肢や体幹など広範囲にわたる例がしばしばみられる。乳幼児の報告例は極めて少ないが，皮疹の非特異的な分布と，年齢的に生検が行い難いことから，診断がついていない症例が潜在している可能性がある。乳幼児の慢性に経過する皮疹として点状出血を混じる赤褐色斑を認めた場合，その分布に関わらず Schamberg 病は鑑別疾患となる。

An Uncommon Cause of Skin Discoloration: Purpura Pigmentosa Progressiva

Purpura Pigmentosa Progressiva is also known as Progressive Pigmentary Dermatitis or Schamberg's Disease. It is a disorder causing skin discoloration on the trunk and extremities, and is a lymphocytic capillaritis of unknown etiology. The disease can occur at any age.

Schamberg Disease in a 54-Year-Old Chinese Woman

Introduction Schamberg disease, also known as Schamberg purpura or progressive pigmentary purpura, is characterized by orange-brown or reddish-brown macules/patches with overlaying purpuric spots simulating “cayenne pepper” [1,2]. The condition was first described in 1901 by Jay Frank Schamberg who reported a 15-year-old boy with a 5-year history of “diffuse, reddish-brown, non-elevated, irregular oval patches with borders consisting of pin-head size, reddish-brown, scarcely elevated puncta or cayenne-pepper spots” [3]. The condition now bears his name. To our knowledge, Schamberg disease has not been reported in Chinese patients. The purpose of this communication is to alert the medical profession to the existence of this condition in Chinese patients so that the diagnosis would not be overlooked.

Pigmented purpuric dermatosis: clinicopathologic characterization in a pediatric series.

Pigmentary purpuras (PPs) are a group of chronic disorders of unknown origin seldom described in children. With this study we sought to better characterize PP eruptions, including clinical evolution and management. A retrospective chart review from 2003 to 2013 querying characteristics of children with a biopsy-proven diagnosis of PP in the Centre Hospitalier Universitaire Ste-Justine dermatology clinic (Montreal, Quebec, Canada) was performed. Follow-up was obtained through telephone interviews. Descriptive statistical analysis was used. Of the 17 subjects, 8 were male and the mean age of onset was 9 years. PP was asymptomatic in 11 patients, pruritic in 3, and of cosmetic concern in 3. Schamberg's disease was the most frequent subtype in 12 cases. Resolution of PP was found in 13 cases with a median duration of less than 1 year (range 6 months-9 years). Five patients experienced spontaneous clearing without treatment, and improvement was observed in 75% of cases treated with topical corticosteroids and 100% with narrowband ultraviolet B (nbUVB). No associated disease, significant drug exposure, or contact allergens were found. Those findings support that PPs in children are idiopathic, chronic eruptions that can benefit from watchful waiting, although topical corticosteroids or nbUVB are may be useful if the patient or family desires faster resolution. This study was limited by its small size, its retrospective nature, and selection and recall bias.