Pigmented Purpuric Dermatosis Research Articles

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.

10% Thioglycolic Acid Peel in the Treatment of Pigmented Purpuric Dermatoses: A Pilot Study.

Pigmentary purpuric dermatosis (PPD) is a chronic dyspigmentation characterized by reddish-brown, irregular maculae with dermal hemosiderin deposition, usually affecting the legs. The -SH group in the thioglycolic acid (TGA) strongly binds to iron molecule, solubilizes it, and clears the pigment. We conducted a longitudinal, right-left leg study for assessing the effectiveness and side effects of TGA 10% peel in treating PPD. For preparation of 10% TGA peel, 80% TGA was diluted with distilled water to 10% concentration by mixing 0.5 mL of acid with 3.5 mL of water before every peel session. The peel was applied on the left leg, weekly for 6 weeks. Assessment was done at baseline and at weeks 3 and 6. Any improvement was noted by the patient and another independent dermatologist. The right leg was untreated. Any side effects during peel application and afterwards were noted. According to the patient assessment, 4/10 patients observed mild improvement, 5/10 patients had moderate improvement, and only a single patient had marked improvement. In the physician assessment, 2/10 patients had >50% improvement, 5/10 patients had 30-40% improvement, and 3/10 patients had 10-20% improvement. Side effects included slight burning during application and foul odor. A single patient had intense erythema and mild swelling of the leg after peel application. 10% TGA is effective in the partial clearance of PPD dyspigmentation with weekly sessions for 6 weeks without any serious side effects.

Pigmented purpuric dermatoses versus purpuric mycosis fungoides: Clinicopathologic similarities and new insights into dermoscopic features.

The diagnosis of purpuric mycosis fungoides (PMF) is often challenging to be clinically differentiated from inflammatory diseases such as pigmented purpuric dermatosis (PPD). Dermoscopy as a non-invasive method can be employed for the visualisation of features invisible to the naked eye. This study aimed to survey the dermoscopic findings of PMF in comparison with PPD. Forty-one patients with an established diagnosis of PMF (n = 28) and PPD (n = 13) were prospectively recruited. Dermoscopic images were taken by FotoFinder Medicam 1000 (FotoFinder Systems GmbH, Bad Birnbach, Germany). Characteristic dermoscopic patterns consisting of fine short linear vessels (35.7%, P = 0.017) and spermatozoa-like structures (50%, P = 0.014) were found to be significantly more common in PMF lesions, while PPD lesions were typified by erythematous globules (76.9%, P = 0.01), in the background colour of dull red (61.5%, P = 0.01) and reticular pigmentation (61.5%, P = 0.044). This study showed the usefulness of dermoscopy for the diagnosis of PMF and PPD cases. Studies with long-term follow-up are needed to affirm the value of these dermoscopic patterns in the differentiation between the two entities.

Clinical Patterns and Risk Factors Associated With Pigmented Purpuric Dermatoses

Objectives: Authors aimed to investigate clinical patterns, characteristics and risk factors associated with pigmented purpuric dermatoses (PPD) in Pakistani population and to correlate the relationship of various suggested aetiologic agents with PPD in our population. Methods: Retrospective analysis of 65 patients was done who visited The Indus Hospital, Karachi between October 2017 to March 2020. Demographics, comorbids, body mass index (BMI), PPD type, clinical findings, laboratory parameters and ultrasonographic findings (USF) of both lower limbs for venous insufficiency were reviewed.  Results: Mean age of patients was 42.6±13.9 years with male to female ratio of 1.2:1. Majority were housewives (40.3%). Schamberg’s disease was present in 64.6%. Obesity was present in 78.1%. Reflux in superficial venous system in 52.3% on ultrasonography, detectable dilated veins in 58.5% and presence of venous stasis in 30.8% PPD cases were found. Males were twice more commonly affected with varicosities. Hypertension (46.2%), diabetes (27.8%) and hyperlipidemia (18.5%) were most common comorbids. Statistically significant differences were detected between USF (p=0.01) and clinical varicosities (p=0.04) when Schamberg’s disease and other PPD were compared; while no association was found with respect to age, gender, occupation, BMI and type of comorbids in these two groups. Conclusion: Venous insufficiency, obesity, and comorbidities like hypertension, diabetes and hyperlipidemia were main cofactors linked with PPD in our population.

Clinical Role of Oral Vitamin C and E Therapy in Skin and Hair Disorders

Clinical Role of Oral Vitamin C and E Therapy in Skin and Hair Disorders

Pigmented Purpuric Dermatoses: A Complete Narrative Review

Pigmented purpuric dermatoses (PPD) include several skin diseases characterized by multiple petechial hemorrhage as consequence of capillaritis. PPD generally present with red to purple macules that progressively evolve to golden-brown color as the hemosiderin is reabsorbed. These lesions, often asymptomatic or associated with mild pruritus, usually occur on the lower extremities and may be a diagnostic and therapeutic challenge both for general practitioners and specialists in internal medicine or flebology. Clinical presentations include many subtypes that have been described over the years, although histology is usually superimposable. Prompt recognition and patient reassurance on the benign nature of these diseases is crucial. In this comprehensive review, we focused on pathogenesis and clinical pictures.

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.

Primary cutaneous T-cell lymphomas in childhood and adolescence.

Primary cutaneous lymphomas are extranodal non-Hodgkin lymphomas of T- or B- cell origin, that predominantly affect older patients but have been reported in all age groups and as early as in the first years of life. Diagnosis of cutaneous lymphomas is challenging and requires high clinical suspicion and close collaboration between dermatologists, pediatric oncologists and pathologists. Skin involvement of non-Hodgkin lymphomas in children or adolescents can either be primary cutaneous or secondary due to an underlying nodal lymphoma. The most common primary cutaneous lymphomas encountered in children are of T-cell origin, with mycosis fungoides being the most prevalent cutaneous T-cell lymphoma, followed by CD30+ lymphoproliferative disorders. While cutaneous lymphomas share clinicopathologic characteristics between juvenile and adult forms, there are important differences in terms of clinical presentation, diagnosis and treatment. The hypopigmented variant of mycosis fungoides seems to be overrepresented in the pediatric age group. Prognosis and treatment of mycosis fungoides are stage dependent. The majority of children present with early-stage disease and respond well to topical corticosteroids and phototherapy.

Pigmented purpuric dermatosis: Ten years of experience in a tertiary hospital and awareness of mycosis fungoides in differential diagnosis.

Pigmented purpuric dermatoses (PPD) can clinically mimic many diseases. Histopathology provides a definitive diagnosis. The aim of the study is to reveal the features of patients with PPD and to determine the disease frequency in the differential diagnosis, especially mycosis fungoides (MF). We retrospectively reviewed records of patients with PPD admitted to our hospital from January 2010 to May 2019. We studied the histopathological features of 127 patients, and performed pattern analysis on cases with a confirmed histopathologic diagnosis of PPD. Among the cases presenting with clinical features of PPD, but displaying different histopathological diagnoses, we focused on MF and tried to clarify the features of PPD-like MF. Overall, 389 patients were admitted to our hospital with PPD symptoms. Of them, 262 patients were diagnosed clinically and a histopathological examination was performed in 127 patients. Of 127, 87 were diagnosed with PPD, and in the remaining 40, non-specific features (9.4%), vasculitis (6.2%), pityriasis rosea (4.7%), MF (3.9%), suspected-MF (1.5%), and other dermatoses (%5.5) were detected. The biopsy findings of two patients showed PPD, but during follow-up, the diagnosis of MF was established. MF should be included in the differential diagnosis of PPD cases presenting with longstanding and widespread involvement.

Pigmented purpuric dermatosis in an infant.

Pigmented purpuric dermatoses (PPDs) are a group of idiopathic, chronic, and self-resolving conditions easily recognizable in adults but extremely uncommon in infants. We present the youngest patient with PPD reported to date.

Dermoscopic Findings and the Clinicopathologic Correlation of Pigmented Purpuric Dermatosis: A Retrospective Review of 60 Cases.

BackgroundPigmented purpuric dermatosis (PPD) is known as a chronic recurrent eruption which usually presents with petechiae and pigmented macules on the lower extremities. Dermoscopy is a noninvasive diagnostic tool in identifying pigmented and vascular lesions, which can also be beneficial in the evaluation of PPD.ObjectiveWe aimed to analyze the common dermoscopic characteristics of PPD, and correlate those findings with the histopathologic features. Additionally, dermoscopic and pathological findings in this study population were compared with other similar studies from the literature review.MethodsA retrospective analysis was performed using data of 60 patients who were diagnosed as PPD by skin biopsy and had dermoscopic examination. The pathologic analysis was performed by categorizing the pattern into lichenoid, perivascular, interface, and spongiotic subtype, and the dermoscopic assessment was performed by the three authors independently.ResultsIn dermoscopy, 96.7% of the patients showed red globules and dots, followed by brownish patch, coppery-red pigmentation, and annular comma-like vessels. The pathologic pattern analysis revealed statistically significant association of lichenoid pattern with coppery red pigmentation, perivascular pattern with annular/comma-like vessels, and spongiosis pattern with reticular pigmented network and linear vessels. The interrater similarity test showed total kappa value of 0.811 which referred to “very good”.ConclusionIn this study, the prevalence of dermoscopic features in Asian PPD patients was identified, which was similar with previous studies. The dermoscopic-pathologic correlation was found in four dermoscopic features. We suggest that dermoscopic examination is helpful in clinical diagnosis and pathological prediction of PPD.

Unusual Pigmented Purpuric Dermatosis with Linear Distribution and Granulomatous Inflammation.

Unusual Pigmented Purpuric Dermatosis with Linear Distribution and Granulomatous Inflammation.

Lichen Aureus with Dermoscopic Findings: A case report

Lichen aureus is a rare variant of pigmented purpuric dermatosis, which often affects young adults and is localized mainly on the lower extremities. We present here a 31-year-old female who had a solitary golden-brown-color patch on her right leg for 1 year. Punch biopsy of the lesion revealed lymphohistiocytic band-like infiltrate of the papillary dermis with erythrocyte extravasation and hemosiderin deposits. The diagnosis of Lichen aureus was made based on clinicopathologic findings. The dermoscopic findings that support this diagnosis were brownish yellow diffuse coloration of the background, round to oval red dots, globules and patches and twisted red loops. Herein, we present the dermoscopic findings of a 31-year-old female patient with a diagnosis of lichen aureus established with clinical and histopathological features. Keywords: pigmented purpuric dermatosis, lichen aureus, dermoscopy

Treatment of segmental lichen aureus in the pediatric age with a 755nm alexandrite picosecond laser. A new therapeutic approach for pigmented purpuric dermatosis.

JDDG: Journal der Deutschen Dermatologischen GesellschaftVolume 18, Issue 10 p. 1201-1203 Dermatosurgery – Tricks of the Trade Treatment of segmental lichen aureus in the pediatric age with a 755 nm alexandrite picosecond laser. A new therapeutic approach for pigmented purpuric dermatosis Diego Fernandez-Nieto, Corresponding Author fnietodiego@gmail.com Dermatology Department, Ramon y Cajal University Hospital, Madrid, Spain Correspondence to Diego Fernandez-Nieto, MD Dermatology Department Ramon y Cajal University Hospital Carretera Colmenar Viejo km 9.100 28034 Madrid, Spain E-mail: fnietodiego@gmail.comSearch for more papers by this authorJuan ­Jimenez-Cauhe, Dermatology Department, Ramon y Cajal University Hospital, Madrid, SpainSearch for more papers by this author­Daniel ­Ortega-Quijano, Dermatology Department, Ramon y Cajal University Hospital, Madrid, SpainSearch for more papers by this authorCristina Pindado-­Ortega, Dermatology Department, Ramon y Cajal University Hospital, Madrid, SpainSearch for more papers by this authorPablo Boixeda, Dermatology Department, Ramon y Cajal University Hospital, Madrid, SpainSearch for more papers by this author Diego Fernandez-Nieto, Corresponding Author fnietodiego@gmail.com Dermatology Department, Ramon y Cajal University Hospital, Madrid, Spain Correspondence to Diego Fernandez-Nieto, MD Dermatology Department Ramon y Cajal University Hospital Carretera Colmenar Viejo km 9.100 28034 Madrid, Spain E-mail: fnietodiego@gmail.comSearch for more papers by this authorJuan ­Jimenez-Cauhe, Dermatology Department, Ramon y Cajal University Hospital, Madrid, SpainSearch for more papers by this author­Daniel ­Ortega-Quijano, Dermatology Department, Ramon y Cajal University Hospital, Madrid, SpainSearch for more papers by this authorCristina Pindado-­Ortega, Dermatology Department, Ramon y Cajal University Hospital, Madrid, SpainSearch for more papers by this authorPablo Boixeda, Dermatology Department, Ramon y Cajal University Hospital, Madrid, SpainSearch for more papers by this author First published: 12 July 2020 https://doi.org/10.1111/ddg.14172Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume18, Issue10October 2020Pages 1201-1203 RelatedInformation

Granulomatous pigmented purpuric dermatosis favorably treated with the combination of colchicine and calcium dobesilate

Granulomatous pigmented purpuric dermatosis favorably treated with the combination of colchicine and calcium dobesilate

Pigmented purpuric dermatosis in children: a retrospective cohort with emphasis on treatment and outcomes.

Data regarding the course and treatment of pigmented purpuric dermatoses (PPD) in the paediatric population are limited. Although treatments for pigmented purpura are not well established, vitamin C and rutoside have been reported to be an effective treatment option and are widely utilized. To assess the clinical course and utility of vitamin C and rutoside in paediatric patients with PPD treated at Ann & Robert H. Lurie Children's Hospital of Chicago between 2008 and 2018. A retrospective review of all children with PPD managed at our hospital between 2008 and 2018 was performed. Additional follow-up was obtained via telephone interviews. A total of 101 patients met inclusion criteria. The female: male ratio was 1.3:1, and the median age at diagnosis was 8.8years (IQR, 5.7-12.9). Median follow-up was 7.13months (IQR, 3-17.4). The most common PPD subtypes were lichen aureus (43%) and Schamberg (34%). Fifty-three (52%) patients had evaluable follow-up documentation via their medical record or phone questionnaire. Twenty-eight patients were treated with vitamin C or rutoside or combination therapy. Twenty-five patients received no treatment. Clearance of the rash was noted in 24 (45.3%) patients overall, including 10 (42%) patients in the treated group and 14 (58%) patients in the untreated group. Recurrence was noted in seven (13.2%) patients. Treatment with vitamin C and/or rutoside was well tolerated without side effects. None of the patients were subsequently diagnosed with vasculitis, coagulopathy or cutaneous T-cell lymphoma. Pigmented purpuric dermatosis in children is a benign disorder with high rates of complete resolution. Treatment with vitamin C and rutoside is well tolerated, but in this cohort, there did not appear to be an advantage over watchful waiting without therapy.

A pediatric case of generalized lichen aureus.

Lichen aureus is a particularly rare subtype of pigmented purpuric dermatosis and is characterized by the sudden appearance of golden or rust-colored macules or needle-tip-sized flat papules (concentrated in one region to form lichenoid papules) on the lower limbs. These skin lesions are usually confined to an isolated, unilateral distribution, and linear segmental distribution is rare. In this report, we have documented one such case, where the lesions on the limb were arranged in strips (segmental distribution) that roughly followed the direction of the venous drainage. And the first attack and subsequent aggravation were both associated with the onset of allergic rhinitis, a Type I hypersensitivity.