Pigmented Purpuric Dermatosis Research Articles

Clinicoepidemiologic Characteristics and Prevalence of Venous Insufficiency in Patients with Pigmented Purpuric Dermatosis

Clinicoepidemiologic Characteristics and Prevalence of Venous Insufficiency in Patients with Pigmented Purpuric Dermatosis

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.

P01 Pigmented and purpuric patches in sun protected areas of a 9 year old child

Abstract Pigmented purpuric dermatosis-like mycosis fungoides (PPD-like MF) is a rare variant of paediatric mycosis fungoides (MF). Clinically, it can be challenging to diagnose and differentiate from pigmented purpuric dermatosis (PPD). Usually, has an indolent course if diagnosed in the early stage. The lesions often localize to sun-protected areas with three stages: patch, plaque, and tumour. Clinical features alone are insufficient for diagnosis and a skin biopsy is mandatory.1 We report a nine year old boy who presented with a three year history of a persistent, slowly progressing, mildly pruritic and scaling rash on the right buttock and medial thighs bilaterally. He was otherwise well with no other relevant medical or family history of note. The physical examination revealed erythematous to purpuric, mild scaly patches with confluent involvement on the right buttock extending to less defined involvement of the both inner thighs. The body surface area involved was less than <10%. No other clinical findings on examination. A punch biopsy for haematoxylin and eosin (H&E) staining was consistent with mycosis fungoides. T cell receptor gene rearrangement testing detected a monoclonal T-cell population. A diagnosis of pigmented purpuric dermatosis-like mycosis fungoides was made. The patient responded well to a potent topical steroid (mometasone furoate) daily which was gradually tapered to twice weekly/intermittent usage to maintain response.

Pigmented purpuric dermatoses: A review

Pigmented purpuric dermatosis (PPD) comprises a group of skin conditions characterized by flat, nonpalpable petechiae and purpura as a result of capillaritis. Although the precise cause of PPD is as yet unknown, increased vascular permeability and capillary fragility are key factors in its pathophysiology. PPD has diverse clinical presentations, but the histopathology of all these variants essentially remains the same. The initial purpuric lesions develop a golden-brown hue owing to hemosiderin absorption and eventually progress to chronic pigmentary changes. These lesions, which often affect the lower limbs, are either asymptomatic or accompanied by moderate pruritus, and can be difficult to diagnose and treat. There are several subtypes of clinical presentations that have been documented over the years. In order to accurately diagnose PPD, a thorough clinical evaluation and, occasionally, a biopsy may be required. It is essential to identify the condition and reassure patients of its benign nature. Treatment is usually necessary due to the chronic nature of the condition, its consequences on physical and mental well-being, and the presence of significant lesions or itching. In this review, we outline the various prevalent PPDs and discuss their etiopathogenesis, clinical features, and diagnostic and treatment modalities.

Overlap of histological features of pigmented purpuric dermatosis and kaposi sarcoma – pseudo kaposi sarcoma

Pseudokaposi sarcoma can be congenital or acquired. Due to prolonged venous stasis, leads to peripheral tissue hypoxemia, which results in neovascularisation and fibroblast proliferation. Treatment includes rectification of the underlying pathology such as graded compression stockings or compression pumps for venous stasis and ulcer management with regular wound care. Medical management includes oral antibiotics, along with compression therapy with good results. Our patient presented mild lower limb pitting edema unilaterally with erythematous barely elevated plaque over the anterior aspect of the lower limb.

A Rare Presentation of Self-Resolving Purple Fingers

Achenbach syndrome is a rare, benign, self-limiting, syndrome of unknown etiology. Here, we report a 66-year-old female who presented to the dermatology clinic with waxing and waning non-palpable purpura for the last year on the volar aspect of her hands bilaterally. The patient admitted to tingling prior to the onset of the purpura and mild tenderness following presentation. The purpura would spontaneously resolve without discoloration or scarring after 2-7 days. The patient stated these episodes can occur anywhere from a few times a month to every 1-2 months. Evaluation and work-up performed by her primary care physician and rheumatologist did not reveal an explanation for her symptoms. Two punch biopsies were performed and revealed a perivascular lymphocytic infiltrate with extravasted erythrocytes and pigmented macrophages consistent with a pigmented purpuric dermatosis. Immunofluorescence was negative. The diagnosis of Achenbach syndrome was made by diagnosis of exclusion. Reassurance was provided to the patient and she was given DerMend Bruise formula cream, which contains retinol, glycolic acid, arnica oil, ceramides, niacinamide, and phytonadione, to monitor for improvement of her symptoms. It is important for healthcare providers to be aware of the benign nature of this syndrome to be able to provide reassurance to patients and avoid an unnecessary, more invasive evaluation.

A retrospective study on the direct immunofluorescence findings in pigmented purpuric dermatosis.

Pigmented purpuric dermatosis (PPD) is characterized by grouped petechiae, purpuric macules, and pigmentation in the bilateral lower extremities. It runs a chronic and relapsing course. Pathophysiology is poorly understood, but it has been proposed to be an immune-complex disease or capillaritis. This study aimed to determine the incidence and patterns of positive direct immunofluorescence (DIF) findings in patients with clinically and histopathologically confirmed PPD. The association between DIF deposition type and clinical profile was also analyzed. Patients with a clinical and histopathologic PPD diagnosis who had undergone DIF studies at a tertiary medical center with attached dermatopathology and immunofluorescence diagnostic centers between January 2002 and December 2021 were included in this study. Data on age, sex, disease duration, comorbidities, and drug intake were collected from medical records. There were 65 patients who satisfied the inclusion criteria. Among them, 58 (89%) had at least one positive finding and 53 (82%) were vascular deposition of immunoglobulin (Ig), complement, or fibrinogen. The most common vascular deposition was fibrinogen (71%) followed by C3 (62%), IgM (18%), IgA (6%), and IgG (3%). Fibrinogen deposition was associated with hypertension (p < 0.03). There was no association between vascular DIF deposition of IgG, IgA, and C3, with age, sex, comorbidities, disease duration, and drug history. The most common DIF findings in PPD were vascular deposition of fibrinogen and C3, with or without Ig presence. DIF findings supported a vascular origin in PPD but not an immune complex-mediated disease. Hypertension was associated with fibrinogen deposition and may play a role in its pathophysiology.

Pigmenting Purpuric Dermatoses: Striking But Not a Manifestation of COVID-19 Infection.

Purpuric or erythematous eruptions of the lower extremities can be perplexing, especially because this clinical sign has been associated with COVID-19 infection. Since the start of the pandemic, COVID-19 often has been the first differential diagnosis for many clinical findings; however, pigmented purpuric dermatoses (PPDs) are distinctive, reassuring, eponymous, and not linked to this virus. In this article, we differentiate the PPDs and delineate them from potentially serious differential diagnoses, including inflammatory retiform purpura, leukocytoclastic vasculitis, stasis dermatitis, and cellulitis.

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.

Clinico Epidemiological Study and Dermoscopic Findings of Pigmented Purpuric Dermatosis.

Clinico Epidemiological Study and Dermoscopic Findings of Pigmented Purpuric Dermatosis.

Clinical analysis of skin lesions in livedoid vasculopathy: a study of 46 Chinese patients.

Clinical data regarding cutaneous manifestations in Chinese patients with livedoid vasculopathy (LV) are limited. To assess clinical features of skin lesions in LV, especially the characteristics of extensive livedo reticularis and pigmented purpuric dermatosis-like lesions in these patients. This was a single-center retrospective study of 46 Chinese patients with LV between March 2021 and July 2021. The characteristics of skin lesions in LV were described in detail. A total of 29 females and 17 males were included in this study, with a mean age of 27.7 years (ranging from 13 to 51 years). Twenty (43.5%) patients developed their first skin lesions before age 18. Among 46 patients, 33 presented livedo reticularis with 78.8% (n=26) of these patients whose livedo reticularis was extensive. Seven patients had lesions simulating pigmented purpuric dermatosis, including four cases of pigmented purpura and three cases of telangiectatic purpura. Numbness was found in 16 patients, mainly in the lower limbs (62.5%), ankles (31.3%), and dorsum of the feet (18.8%). For patients with symptoms of extensive livedo reticularis, retiform purpura, or numbness, it is necessary to make a differential diagnosis.

Progressive pigmented purpuric dermatosis in skin of color: a dermoscopic and histopathological correlation

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Progressive pigmented purpuric dermatosis (PPPD) is a chronic cutaneous presents with petechiae, purpura on hyperpigmented yellowish-brown macules or patches. Diagnosis is straight forward, sometimes may be challenging for the diagnosis. Dermoscopy is a non-invasive tool. It gives characteristic patterns in PPPD. However, dermoscopic descriptions in skin of color are limited to case reports. Here, dermoscopic evaluation in skin of color is studied in detail. &lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; It was a cross sectional observation study. Ethical clearance and written consent was obtained. Patients of skin type 4 and 5, attending dermatology clinic with suspected lesions of PPPD were selected serially. Handheld dermoscope was used for examination. Site for dermoscopic examination was considered as target area. Skin biopsy was taken. Dermoscopic patterns were analysed and features were correlated with histopathological changes.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; Totally 30 patients with 27 males and 3 females were enrolled. Schamberg’s disease was commonest type noted in 21patients. Eczematoid, lichen aureus, and lichenoid types were seen in 2 (6.7%) each. Hence, overall occurrence of Schamberg’s disease was statistically significant (p=0.028). Commonest dermoscopic findings were brown pigment network and yellowish-brown dots/globules. Least common features included linear vessels and bluish globules. Distorted pigment network noted in 29 (96.7%).&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Dermoscopy of PPPD shows characteristic patterns of which pigment network, red globules and yellowish-brown globules are the basic features. These features correlate well with histopathological changes. Yellowish-brown globules and background are prominently observed in skin of color. Larger studies are recommended to validate these differences.&lt;/p&gt;&lt;p class="abstract"&gt; &lt;/p&gt;

Progressive ascending telangiectasias

Progressive ascending telangiectasias

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.

TNF-\u03b1 inhibitor induced pigmented purpuric dermatoses: a case report

BackgroundWe present a rare case of TNF-α inhibitor induced pigmented purpuric dermatoses (PPD) and explore its mechanisms and management.Case presentationA 44-year-old woman presented with non-pruritic non-tender petechial rash on bilateral lower limbs after being started on Adalimumab, with the rash progressing to worsen on Golimumab, both used for managing her seronegative peripheral arthritis. Laboratory panel revealed a negative vasculitis screen and skin biopsy confirmed the condition. After ceasing the TNF-α inhibitors and changing to Secukinumab, an Interleukin-17 inhibitor, the lesions stopped erupting and slowly resolved.ConclusionPPD is a benign skin condition and has been associated with various medications and exposure to chemicals in the literature. Different mechanisms have been proposed in the literature however its exact aetiology is unknown. To date, there is no standardized treatment however patients should be reassured that PPD is benign and will often regress by itself once the causative agent has been removed.

All that reddens is not psoriasis: a peculiar localization of pigmented purpuric dermatosis in two psoriatic patients.

article: All that reddens is not psoriasis: a peculiar localization of pigmented purpuric dermatosis in two psoriatic patients - Italian Journal of Dermatology and Venereology 2022 August;157(4):376-7 - Minerva Medica - Journals

Chronic venous disease, platelet and haemostatic abnormalities contribute to the pathogenesis of pigmented purpuric dermatoses.

To investigate the aetiology of pigmented purpuric dermatoses (PPD). 63 patients with a provisional diagnosis of PPD were assessed. Skin biopsies were performed to confirm the clinical diagnosis. Haemostasis was assessed using platelet function analyser-100 (PFA-100), light transmission aggregometry (LTA), impedance aggregometry (Multiplate) and measurement of clotting times and clotting factors. Chronic venous disease (CVD) was assessed by duplex ultrasound. When not contraindicated, patients were advised to discontinue haemostatic-modifying drugs or supplements for 4weeks after which the laboratory measurements were repeated and the clinical resolution of PPD was assessed. Subsequently, a cohort of patients identified with CVD underwent endovenous interventions and further resolution of PPD was assessed. CVD was found in 48 patients (76.2%) while haemostatic abnormalities were found in 36 (57.1%). 30 patients (47.6%) had concurrent CVD and haemostatic abnormalities. Modifiable risk factors such as the intake of platelet inhibitors or other drugs and supplements such as fish oil were identified in 53 patients (84.1%). These could be ceased in 35 patients of whom 28 (80.0%) achieved either complete or partial resolution of PPD. Treatment of the underlying CVD was performed in 18 patients resulting in complete or partial resolution in 17 (94.4%). In seven patients (11.1%), no CVD or haemostatic abnormalities were identified, and the risk factors included dietary factors such as excessive caffeine or soft drink consumption. Haemostatic abnormalities and CVD contribute to the pathogenesis of PPD. Resolution of PPD in the vast majority of patients may be achieved by cessation of modifiable risk factors and in particular platelet-modifying drugs or supplements and treatment of the underlying venous disease.

Granulomatous pigmented purpuric dermatosis: Clinical and histopathologic findings in a series of nine cases.

We report the largest case series to date of granulomatous pigmented purpuric dermatosis (GPPD), a rare variant of pigmented purpuric dermatoses (PPD). GPPD can cause diagnostic difficulties as it can be mistaken clinically and histopathologically with numerous inflammatory and infectious dermatoses or even cutaneous T-cell lymphoma. We compared the histopathological findings of nine cases of GPPD with a control group consisting of 10 randomly selected PPD of other subtypes. GPPD seems to predominantly affect the lower extremities of adult male patients; a clear association with hyperlipidemia or other systemic conditions could not be confirmed. Histopathologically, GPPD is characterized by a dermal histiocyte-rich interstitial infiltrate with or without granuloma formation, thickened capillaries, extravasated erythrocytes, and/or hemosiderin deposits. In contrast to other forms of PPD, the inflammatory infiltrate of GPPD can extend to the mid or deep dermis and the admixed lymphocytic infiltrate is mainly composed of CD8+ T-cells.

Venous Insufficiency is a Clear Provoker of Pigmented Purpuric Dermatosis.

BackgroundPigmented purpuric dermatosis (PPD) is a chronic disorder characterized by distinct petechial hemorrhage and brownish pigmentation. The cause of PPD is unclear, but several underlying conditions are associated with it. Previous reports suggest that venous insufficiency (VI) might be related to PPD; however, a clear correlation remains unelucidated.ObjectiveTo elucidate the causal relationship between PPD and VI.MethodsA total 118 patients diagnosed with PPD in the Department of Dermatology, Pusan National University Hospital from November 2006 to July 2019 were retrospectively reviewed. Doppler ultrasonography of the lower extremities was performed in 56 PPD patients, who were then divided into two groups: PPD with and without VI. We compared the clinical features between the two groups. In the PPD with VI group, we assessed the correspondence ratios between PPD and VI lateralities, and between the PPD distribution and the veins involved.ResultsVI was detected in 35 of the 56 patients (62.5%). The PPD with VI group was significantly associated with wider distribution, darker coloration and longer disease duration. There was a positive correlation of laterality between PPD and VI, and between PPD distribution and the vein involved.ConclusionThis findings suggest that VI is a clear provoker of PPD.

CD1a-positive dendritic cell-enriched pigmented purpuric dermatosis in association with dyslipidemia

Indeterminate cells (ICs) are dendritic cells (DCs) that share the histologic features of Langerhans cells and macrophages but lack Birbeck granules. It remains unclear whether IC histiocytosis is a separate disease entity or a reactive process. We report the case of a male with an 8-year history of dyslipidemia who developed extensive pigmented purpuric papules. Laboratory studies revealed peripheral monocytosis (8.2%), and two skin biopsies showed a dermal lichenoid lymphohistiocytic infiltrate composed of CD4+ T cells and CD8+ T cells and CD68+CD163+CD1a+Langerin histiocytes. PD-1/PD-L1+T cells were present in the dermis. Our case may help in understanding the pathogenesis of IC histiocytosis, and we hypothesize that expression of CD1a+ dermal DCs and T-cell dysfunction is a reactive process to dyslipidemia.