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.

A Rare Skin Rash: Linezolid-Induced Purpuric Drug Eruption without Vasculitis in a Puerto Rican Man.

BACKGROUND Cutaneous adverse drug reactions are the skin's response to a systemic exposure to drugs. Linezolid is an oral oxazolidine used to treat methicillin-resistant Staphylococcus aureus infections. Even though it has well-known adverse effects, purpuric cutaneous adverse drug reactions to linezolid have been scarcely described. This report is of a Puerto Rican man in his 80s who developed an extensive purpuric drug eruption secondary to linezolid use. Clinicians should be aware of this phenomenon, since prompt identification and discontinuation of the agent are essential for recovery. CASE REPORT An 89-year-old Puerto Rican man was given oral linezolid therapy for healthcare-associated pneumonia and developed a widespread, purpuric cutaneous eruption 5 days into therapy. His condition prompted immediate discontinuation of the drug. Forty-eight hours after stopping the medication, he visited the Emergency Department. Abdominal punch biopsy revealed a superficial and perivascular lymphocytic infiltrate with dermal eosinophils, a pathologic finding consistent with a purpuric drug eruption. This allowed for a timely diagnosis, exclusion of other mimickers, such as cutaneous vasculitis, and effective management. CONCLUSIONS Cutaneous adverse drug reactions to linezolid have been scarcely reported in the literature. Due to the low incidence of this manifestation, the identification of the causative agent and accompanying treatment may be delayed. Mainstays in therapy are avoidance of the offending agent and treatment with corticosteroids, antihistamines, barrier ointments, and oral analgesics. Primary healthcare providers should be aware of linezolid-induced cutaneous manifestations, diagnostic clues, and treatment options so they can rapidly identify and effectively treat such complications.

Vulvar Lichen Planus Presenting as Chronic Vulvar Purpura.

Background: There is a broad spectrum of vulvar pigmented lesions that differ based on their histopathological and clinical features. Chronic vulvar purpura is a rare entity, associated with a broad morphological spectrum, from lichen aureus, Zoon's vulvitis, pigmented purpuric dermatosis and with lichen planus as in our case. Case presentation: In this article we discuss a case of an 86-year-old white woman with hyperpigmentation on her upper vulva, next to the introitus, with complaints of urine incontinence. Biopsy revealed subepithelial stromal lichenoid inflammatory infiltrate containing plasma cells, lymphocytes and some neutrophilic granulocytes as well as dilated and congested vessels. Hemosiderin deposits and erythrocyte extravasation were found. There was evidence of hyperkeratosis with hyper granulosis and erosions. Spongiosis was also noted. Few melanocytes were identified with no sign of malignancy. These findings correlate with the diagnosis of vulvar lichen planus. Conclusions: Chronic vulvar purpura is a clinical term used for different chronic inflammatory dermatoses presenting as red bluish or violaceous discolorations on the vulva, often associated with cayenne-pepper-like speckling. Considering a great overlap of possible diseases, the final diagnosis could be challenging. It is important to exclude a melanocytic tumour in these cases.

Dramatic cutaneous eruption of nodular purpuric lesions in an otherwise well older man.

We describe a dramatic presentation of a rare condition, presenting as a diffuse ecchymosis-like area. This entity has a predilection to primary cutaneous involvement, placing dermatologists as important primary assessors. Clinical knowledge and a high level of clinical suspicion are essential for timely diagnosis. Click here for the corresponding questions to this CME article.

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

BACKGROUND: To investigate the aetiology of pigmented purpuric dermatoses (PPD). METHODS: 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 4 weeks 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. RESULTS: 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. CONCLUSION: 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.

Annular urticarial lesions.

Annular urticarial configurations are often associated with acute and chronic urticaria. Such lesions may be short-lived, migratory, transient, pruritic, and resolving with no residual evidence, making the diagnosis of urticaria an obvious one. Annular urticarial lesions can be the presenting signs of various cutaneous and systemic diseases. The differentiation of urticarial lesions may be made by considering the duration of an individual lesion longer than 24 hours, with burning and pain sensation in the lesions or lack of pruritus; skin marks such as postinflammatory pigmentation or purpura after resolution of the lesions; associated scaling or vehiculation in the lesions; systemic symptoms such as arthralgia, fever or fatigue; and several abnormal laboratory findings. The main differential diagnoses of annular urticarial lesions include urticarial vasculitis, autoinflammatory syndromes, hypersensitivity reactions, and connective tissue diseases.

Leukocytoclastic vasculitis with features of flagellate purpura: a comparison with flagellate erythema.

Leukocytoclastic vasculitis is a histopathologic term describing a type of small-vessel vasculitis characterized by a predominantly neutrophilic inflammatory infiltrate and nuclear debris. Skin involvement is common and can have a heterogeneous clinical presentation. Herein, we describe a 76-year-old woman with no history of chemotherapy or recent mushroom ingestion that presented with focal areas of flagellate purpura secondary to bacteremia. Histopathology revealed leukocytoclastic vasculitis and her rash resolved after antibiotic treatment. It is important to distinguish flagellate purpura from a similar condition, flagellate erythema, as they have been reported in association with distinct etiological and histopathological features.

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

BACKGROUND: 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. OBJECTIVES: This study aimed to survey the dermoscopic findings of PMF in comparison with PPD. METHODS: 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). RESULTS: 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). CONCLUSIONS: 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.

Mitochondrial Dysfunction and Redox Homeostasis Impairment as Pathomechanisms of Brain Damage in Ethylmalonic Encephalopathy: Insights from Animal and Human Studies.

Ethylmalonic encephalopathy (EE) is a severe intoxication disorder caused by mutations in the ETHE1 gene that encodes a mitochondrial sulfur dioxygenase involved in the catabolism of hydrogen sulfide. It is biochemically characterized by tissue accumulation of hydrogen sulfide and its by-product thiosulfate, as well as of ethylmalonic acid due to hydrogen sulfide-induced inhibition of short-chain acyl-CoA dehydrogenase. Patients usually present with early onset severe brain damage associated to encephalopathy, chronic hemorrhagic diarrhea and vascular lesions with petechial purpura and orthostatic acrocyanosis whose pathophysiology is poorly known. Current treatment aims to reduce hydrogen sulfide accumulation, but does not significantly prevent encephalopathy and most fatalities. In this review, we will summarize the present knowledge obtained from human and animal studies showing that disruption of mitochondrial and redox homeostasis may represent relevant pathomechanisms of tissue damage in EE. Mounting evidence show that hydrogen sulfide and ethylmalonic acid markedly disturb critical mitochondrial functions and induce oxidative stress. Novel therapeutic strategies using promising candidate drugs for this devastating disease are also discussed.

A clinicopathological description of COVID-19-induced chilblains (COVID-toes) correlated with a published literature review.

BACKGROUND: The abundance of publications of COVID-19-induced chilblains has resulted in a confusing situation. METHODS: This is a prospective single-institution study from 15 March to 13 May 2020. Thirty-two patients received PCR nasopharyngeal swabs. Of these, 28 patients had a thoracic CT-scan, 31 patients had blood and urine examinations, 24 patients had skin biopsies including immunohistochemical and direct immunofluorescence studies, and four patients had electron microscopy. RESULTS: COVID-19-induced chilblains are clinically and histopathologically identical to chilblains from other causes. Although intravascular thrombi are sometimes observed, no patient had a systemic coagulopathy or severe clinical course. The exhaustive clinical, radiological, and laboratory work-up in this study ruled-out other primary and secondary causes. Electron microscopy revealed rare, probable viral particles whose core and spikes measured from 120 to 133 nm within endothelium and eccrine glands in two cases. CONCLUSION: This study provides further clinicopathologic evidence of COVID-19-related chilblains. Negative PCR and antibody tests do not rule-out infection. Chilblains represent a good prognosis, occurring later in the disease course. No systemic coagulopathy was identified in any patient. Patients presenting with acral lesions should be isolated, and chilblains should be distinguished from thrombotic lesions (livedo racemosa, retiform purpura, or ischemic acral necrosis).

Angiokeratoma-like purpuric palmar nodules following chemotherapy.

We describe a patient with leukemia undergoing chemotherapy who developed painful purpuric nodules of the digits. These findings were concerning for endocarditis (clinically) and angiokeratomas on gross histology. After extensive evaluation, we report the development of painful purpuric nodules as a likely side effect of the patient's therapeutic regimen (hydroxyurea, danorubicin, cytarabine, and methotrexate).