Retiform purpura: A diagnostic approach.

Retiform purpura is a specific morphology within the spectrum of reticulate eruptions of vascular origin. It develops when blood vessels serving the skin are compromised resulting in downstream cutaneous ischemia, purpura, and necrosis. Identifying retiform purpura is important particularly in the acutely ill patient. It may elucidate the underlying diagnosis, provide prognostic information, and suggest a treatment approach. The differential diagnosis of retiform purpura is vast, reflecting the myriad conditions that can lead to cutaneous vessel wall damage or lumen occlusion. In this article, we give an overview of the differential diagnosis of this cutaneous morphology, provide an approach to workup, and highlight updates in treatment of some of the more common conditions that manifest as retiform purpura.

Retiform purpura: Workup and therapeutic considerations in select conditions.

In this article we focus on updates in select etiologies of retiform purpura. These causes of retiform purpura, in addition to bacterial or fungal sepsis, disseminated intravascular coagulation, purpura fulminans, and catastrophic antiphospholipid syndrome, are important diagnoses with potential for morbidity and mortality. Important aspects in the pathophysiology, patient demographics and risk factors, updates in the diagnostic workup, histopathology, and treatment of these specific conditions are discussed.

Update and Review of Bleeding Considerations in Dermatologic Surgery: Hemostatic Techniques and Treatment Strategies for Bleeding Complications.

BACKGROUND: There are many intraoperative and postoperative techniques to aid hemostasis in dermatologic procedures. An updated understanding is critical for the surgeon. OBJECTIVE: To provide an updated review of methods for hemostasis and therapies for postprocedural purpura and ecchymosis applicable to dermatology. MATERIALS AND METHODS: A review of Ovid MEDLINE was performed to review the English-language medical literature of hemostatic options and their use in cutaneous surgery. All available publication years were included from 1946 to present. RESULTS: A comprehensive and current list of hemostatic options used in the intraoperative and postoperative period is provided along with traditional and emerging therapies for postprocedural purpura and ecchymosis. CONCLUSION: A myriad of options exist for minimizing and treating bleeding complications. The appropriate use and updated knowledge of hemostatic options is provided.

Cerebral Microhemorrhage and Purpuric Rash in COVID-19: The Case for a Secondary Microangiopathy.

INTRODUCTION: Since the emergence of Coronavirus Disease 19 (COVID-19) pandemic, multiple neurologic complications in infected patients have been reported. Despite these reports, the mechanism of COVID-19 nervous system injury is not well understood. We report the case of a COVID-19 patient with diffuse microhemorrhages on brain MRI, positive anticardiolipin antibodies, and purpuric rash with biopsy showing a thrombotic vasculopathy, all features suggestive of secondary microangiopathy. CASE REPORT: A 69-year-old male with history of hypertension, chronic kidney disease, and hypothyroidism presented with one week of dyspnea, cough, diarrhea, and fevers. Chest x-ray demonstrated bibasilar consolidations and nasopharyngeal reverse transcriptase polymerase chain reaction confirmed SARS-CoV-2 infection. He had subsequent respiratory decline requiring intubation the day after admission. He developed a truncal morbilliform rash and diffuse purpura, a biopsy of which showed small dermal blood vessels with intraluminal microthrombi consistent with thrombotic vasculopathy. He was found to have elevated aCL IgM and IgG and equivocal lupus anticoagulant study. Brain MRI obtained for persistent encephalopathy showed innumerable areas of susceptibility weighted imaging changes throughout the bilateral juxtacortical white matter, corpus callosum, basal ganglia, and brainstem, as well as multiple small areas of FLAIR hyperintensities, consistent with microhemorrhage DISCUSSION: While there have been several reported cases of neurologic manifestations of COVID-19, the pathophysiology may not be related to neurotropism of the virus itself. The new development of antiphospholipid antibodies and thrombotic vasculopathy in dermal blood vessels in this patient suggest a secondary microangiopathy potentially related to a virally-induced inflammatory state.

Improved clinical outcome following liver transplant in patients with ethylmalonic encephalopathy.

Ethylmalonic encephalopathy (EE) is a rapidly progressive autosomal recessive mitochondrial disease caused by biallelic pathogenic variants in the ETHE1 gene that encodes the mitochondrial sulfur dioxygenase. It is characterized by neurodevelopmental delay and regression, pyramidal and extrapyramidal signs, recurrent petechiae, chronic diarrhea, and orthostatic acrocyanosis. Laboratory findings include elevated serum levels of lactate and C4-C5 acylcarnitines, and elevated urinary excretion of ethylmalonic acid and C4-C6 acylglycines, notably isobutyrylglycine and 2-methylbutyrylglycine. These findings are attributed to deficiency of the mitochondrial sulfur dioxygenase resulting in toxic accumulation of hydrogen sulfide metabolites in vascular endothelium and mucosal cells of the large intestine. Medical management has thus far been directed toward decreasing the accumulation of hydrogen sulfide metabolites using a combination of metronidazole and N-acetylcysteine. More recently, orthotopic liver transplant (OLT) has been reported as a new therapeutic option for EE. Here, we report two additional cases of EE who achieved psychomotor developmental improvement after 7- and 22-months following OLT. The second case serves as the longest developmental outcome follow-up reported, thus far, following OLT for EE. This report provides additional evidence to validate OLT as a promising therapeutic approach for what was considered to be a fatal disease.

Clinical Profile of Levamisole-Adulterated Cocaine-Induced Vasculitis/Vasculopathy: A 30-Case Series.

OBJECTIVES: The aims of this study were to describe clinical and laboratory manifestations of patients with levamisole-adulterated cocaine-induced vasculitis/vasculopathy and to propose a skin classification according to the distribution and severity of lesions. METHODS: We report the characteristics of 30 patients admitted with levamisole-adulterated cocaine-induced vasculitis/vasculopathy in 4 high-complexity institutions in Colombia, from December 2010 to May 2017. We compare our findings with the main published series. RESULTS: Median age was 31 years (interquartile range, 27-38 years) with a male-to-female ratio of 5:1. Eighty-three percent of the patients had retiform purpura affecting the limbs, buttocks, face, or abdomen; 73% had ear necrosis, 50% cutaneous ulcers, 17% genital necrosis, 13% oral ulcers, and 10% digital necrosis. Cutaneous involvement was classified according to the frequency of the compromised corporal area, and purpuric lesions were stratified in 4 grades of severity. Anti-neutrophil cytoplasmic autoantibodies were positive in 85% of the cases, lupus anticoagulant in 73%, and antinuclear autoantibodies in 57%; rheumatoid factor was negative in all cases. We found nephritis in 17 cases (57%). Prednisolone was used in most of the patients (70%), with other immunosuppressive agents being used in a lower percentage. Improvement was observed in 93% of the patients, but symptoms recurred in 40%, attributed to relapses in consumption. End-stage chronic renal disease developed in 10% of the cases, and 1 patient died. CONCLUSIONS: Because of rising cocaine consumption and levamisole adulteration frequency, levamisole-adulterated cocaine-induced vasculitis/vasculopathy is becoming more common. Detailed characterization of skin involvement coupled with multiple antibody positivity is essential for a diagnosis. Renal involvement is frequent, clinically and histologically heterogeneous, and potentially serious.

What is that rash?

CASE HISTORY: A healthy 15-month-old girl presented to the emergency department with a 24-hour history of fever and rash. The initial blanching rash developed into non-blanching areas with associated leg swelling. She had received no recent medications, had no known drug allergies and no unwell contacts.On examination, she was feverish at 38.6°C, capillary refill time was <2 s with warm peripheries, heart rate 169 bpm and blood pressure 94/59 mm Hg. A palpable purpuric rash was evident on all four limbs and face (figure 1) although the trunk was spared. Her legs were tense and oedematous to the knee.edpract;103/1/25/EDPRACT2016311782F1F1EDPRACT2016311782F1Figure 1Rash at presentation.Initial investigations: Haemoglobin level: 131 g/L, white cell count: 16.6×109/L, neutrophils: 11.1×109/L and platelets: 407×109/LCoagulation screen: normalC reactive protein level: 20 mg/LLactate level: 1.7 mmol/LIntravenous ceftriaxone was commenced following blood culture and meningococcal PCR. The following day, while remaining systemically well, she developed a vesicular rash on her trunk and back (figure 2).edpract;103/1/25/EDPRACT2016311782F2F2EDPRACT2016311782F2Figure 2Vesicular rash. QUESTIONS: What is the diagnosis? Henoch-Schonlein purpura (HSP)Meningococcal septicaemiaAcute haemorrhagic oedema of infancy (AHOI)Vasculitic urticariaGianotti-Crosti syndromeWhat further investigation is required? Check viral serology including Epstein-Barr virus and hepatitis B virusComplement levels and autoimmune screenSkin biopsyLumbar puncture and audiologyNo further investigationHow should this child be managed? Complete 7 days of ceftriaxone treatmentOral aciclovirOral steroidsRegular follow-up with urinalysis and blood pressure monitoringStop antibiotics if cultures were negative at 48 hours and dischargeAnswers are on page▪▪.

Laser acupuncture improves skin color deformation by cupping: A pilot study.

Negative pressure of cupping induces skin deformation such as ecchymosis and purpura in a circular shape. Thus, there is a desire to treat skin before depigmentation and scarring occurs. Therefore, we introduce laser therapy, a widely used technique treat pigmentation in dermatology. Various parameters of laser therapy can be applied, so to determine the optimal exposure parameters that do not damage the surrounding tissues, the subjects were divided into four groups: a non-stimulation group and three laser groups (4 J/cm2 group, 6 J/cm2 group, and 8 J/cm2 group). We selected the wavelength and output of laser as follows: 660nm and 50mW. The 40 were divided into four groups of 10. In the first experiment, we measured skin temperature using Digital infrared thermography in order to observe whether the laser could cause heat damage. In the second experiment, each group received the assigned laser therapy protocol every 24 hours for 72 hours. We obtained a skin image using a cross polarization technique. Previous studies have shown that a*and E.I (erythema index) represent the degree of skin erythema (hemoglobin content). M.I (melanin index) indicates the degree of skin pigmentation (melanin content). Hence, skin color information was analyzed with the a*, erythema index (E.I), and melanin index (M.I) for 72 hours. None of the laser exposure parameters led to skin damage by heating or energy dissipation. The results of a*, E.I, and M.I of all groups showed the different recovery rates towards the normal skin color information before cupping. As energy density increases, the result of a* and E.I showed the fast recovery rate. There was no significant different between M.I at non-stimulation group and M.I at 4 J/cm². Therefore, the least energy density as 6 J/cm² is need for the recovery of melanin content. The a*, E.I, and M.I at 8 J/cm² group rather than other groups were significantly recovered to normal skin color. In conclusion, the laser therapy (energy density: 8 J/cm²) has a significant recovery of the skin erythema and skin pigmentation except to skin damage.

[Case report: 40-year-old patient with massive bleeding undergoing cesarean section]. / Kasuistik - 40-jährige Patientin mit Massivblutung im Rahmen einer Sectio caesarea.

We report a case of a 40-year-old patient suffering major bleeding when undergoing cesarean section for delivery with placenta previa. After surgery the patient suffered from severe bleeding several times and again underwent surgery with abdominal packing. After an abdominal compartment syndrome and an ARDS a post transfusion purpura was found to be the cause of the repeated bleeding and could successfully be treated with a cycle of plasmapheresis.

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.

Treatment of purpura with lasers and light sources.

The potential for bruising is a frequent concern for patients undergoing minimally invasive procedures, particularly injection-based soft tissue fillers and botulinum toxin type A. Decreasing the risk of this side effect with good technique and careful patient selection is key, but interventions that quicken the resolution of bruising are also helpful. Many practitioners have employed the theory of selective photothermolysis, using laser and light devices, to target hemoglobin and its breakdown products to speed time to bruise resolution. 585-595 nm pulsed dye, pulsed 532 nm and 1064 nm long-pulsed neodymium-doped yttrium aluminum garnet (Nd:YAG) lasers and intense pulsed light may be utilized with best results achieved when treatment is performed 1-2 days after the appearance of purpura. Specific therapy recommendations, side effects and contraindications will be addressed in this review.

How to spot cocaine-induced pseudovasculitis.

The prevalence of cocaine-induced pseudovasculitis (CIP) causing cutaneous destruction is increasing, and plastic surgeons need to be aware of this condition because they are a part of the multidisciplinary treatment team. Differentiation of CIP from a true autoimmune vasculitis can be exceedingly challenging, and misdiagnosis with ensuing treatment may be fatal. This article is a succinct review of CIP, guided by a clinical case of 30% total body surface area skin necrosis, to familiarize the reader with this syndrome. Diagnostic aids include history of cocaine use, localized disease manifestation to skin or mucosa, discordance of antineutrophil cytoplasmic antibody and target antibody patterns typical for true vasculitis, and testing for antihuman neutrophil elastase and levamisole. Treatment is primarily supportive, and wound care, with regard to dressings and surgery, is a cross between to that of burns and meningococcemia patients.

An approach to the patient with retiform purpura.

Retiform purpura consists of branching purpuric lesions caused by a complete blockage of blood flow in the dermal and subcutaneous vasculature. The differential diagnosis for retiform purpura is broad, including vasculitides of the small and medium vessels as well as microvascular occlusion due to thrombotic, infectious, and embolic phenomena. Determining the etiology of this important dermatologic sign can be a diagnostic challenge; however, an organized approach can improve the speed and accuracy of diagnosis and identify an effective treatment. This review focuses on early recognition, evaluation, and treatment of hospitalized patients with retiform purpura. Specifically, vasculitis, protein C and S deficiencies, heparin necrosis, warfarin necrosis, antiphospholipid antibody syndrome, disseminated intravascular coagulation, cryoglobulinemia, calciphylaxis, and cholesterol embolization syndrome will be discussed in detail. These conditions are commonly seen in consultative dermatology and can have multiorgan involvement, complicated laboratory evaluation, and long-term therapeutic implications.

A case of lichen aureus successfully treated with 595 nm wavelength pulsed-dye laser.

BACKGROUND: Lichen aureus (LA) is a variant of pigmented purpuric dermatosis (PPDs) that typically presents with the acute onset of a solitary, unilateral, purple to rust-yellow colored lichenoid patch or plaque on lower extremities. Treatment remains challenging and is based on anecdotal case reports often with poor results. AIMS: Describe a case of LA successfully treated with 595 nm wavelength pulsed-dye laser (PDL). PATIENT/METHOD: A 46-year-old woman with segmental LA was treated using a 595 nm PDL at a uniform spot size of 10 mm, with pulse durations of 10 milliseconds and fluence of 6 J/cm2. The patient had received previous treatments with no improvement. RESULTS: Clearance was archived after three sessions with PDL. Sessions were performed at intervals of 4 weeks, with no serious adverse events nor recurrence. CONCLUSION: We hypothesize the favorable clinical outcome with PDL is due to the affinity of the wavelength for oxyhemoglobin (allowing uniform vessel penetration and energy delivery to fragile capillaries and intraluminal blood) and to its anti-inflammatory profile. PDL seems to be an alternative for patients with progressive LA that have failed other therapies.

Drug-induced cutaneous vasculitis and anticoagulant-related cutaneous adverse reactions: insights in pathogenesis, clinical presentation, and treatment.

Drug-induced vasculitis and anticoagulant-related skin reactions are commonly encountered in the inpatient and outpatient settings. The spectrum of clinical presentation is broad and ranges from focal, skin-limited disease, to more extensive cutaneous and soft tissue necrosis, to potentially fatal systemic involvement. The prompt recognition of these adverse events can have a significant impact on patient morbidity and mortality. We highlight the key features of the clinical presentation with an emphasis on primary lesion morphology, distribution, and epidemiology of purpuric drug reactions. The proposed pathophysiology, histologic findings, and therapeutic interventions of these potentially life-threatening diseases are discussed.

Giant placental chorioangioma followed by circulatory failure of the newborn and infantile hemangioma: Case report.

Placental chorioangioma (CA) is a benign placental tumor. No specific treatment is required for asymptomatic cases. We report a female infant born to a mother with giant placental CA. However fetal growth was normal and, fetal hydrops was not detected by ultrasound examination until delivery, she had hydrops, subgaleal hematoma, thrombocytopenia, hemolytic anemia, respiratory distress and circulatory failure after birth. She was successfully treated without any neurological sequelae. At 2 months of age, infantile hemangioma appeared in her lower lip. The present case suggested that giant placental CA might cause postnatal problems and be associated with the development of infantile hemangioma.