ABSTRACT
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.
Subject(s)
Lasers, Dye/therapeutic use , Lichenoid Eruptions/therapy , Low-Level Light Therapy/instrumentation , Purpura/therapy , Biopsy , Female , Humans , Lichenoid Eruptions/diagnosis , Lichenoid Eruptions/pathology , Middle Aged , Purpura/diagnosis , Purpura/pathology , Skin/pathology , Skin/radiation effects , Treatment OutcomeABSTRACT
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.
Subject(s)
Cocaine-Related Disorders/complications , Cocaine , Glomerulonephritis , Levamisole , Purpura , Vasculitis , Adjuvants, Pharmaceutic/adverse effects , Adjuvants, Pharmaceutic/pharmacology , Adult , Autoantibodies/blood , Cocaine/pharmacology , Colombia , Dopamine Uptake Inhibitors/pharmacology , Drug Contamination , Female , Glomerulonephritis/chemically induced , Glomerulonephritis/diagnosis , Glomerulonephritis/immunology , Glomerulonephritis/therapy , Humans , Levamisole/adverse effects , Levamisole/pharmacology , Male , Necrosis , Patient Care Management/methods , Purpura/chemically induced , Purpura/diagnosis , Purpura/immunology , Purpura/therapy , Skin/pathology , Treatment Outcome , Vasculitis/chemically induced , Vasculitis/diagnosis , Vasculitis/immunology , Vasculitis/therapyABSTRACT
Objetivos: Revisäo da doença de Schamberg enfocando os aspectos imunológicos e terapêuticos. Métodos: A doença de Schamberg, o líquen aureus, e as purpúras de Majocchi, Gougerot-Blum, e ectematosa de Doucas-Kapetanakis, pertencem ao espectro das chamads púrpuras simples. As lesöes purpúricas pigmentadas säo geralmente assintomáticas e näo têm etiopatogenia estabelecida. Resultados: A púrpura de Schamberg apresenta um quadro histopatológico de inflamaçäo com hemorragia, sem necrose fibrinóide (capilarite ou pervasculite). A agressäo vascular, com estravazamentode hemácias e conseqüente deposiçäo de hemossiderina, é secundária a reaçöes imunológicas mediadas por linfócitos T na regiäo capilar dérmica. As citocinas e oa umento da expressäo das moléculas de adesäo endoteliais promovem a aderência células T -queratinócitos. A ativaçäo precoce destes receptores de adesäo endoteliais, determina a organizaçäo do infiltrado inflamatório pericapilar. Alteraçöes sutis no sistema coagulaçäo-fibrinólise foram descritas. Näo há terapia definida. A pentoxifilina, diminuindo a adesäo de células T às células endoteliais e aos queratinócitos, foi útil no controle das lesöes de alguns casos. Há um potencial futuro para a utilizaçäo de outras estratégias terapêuticas antiinflamatórias. Conclusöes: A púrpura de Schamberg é uma capilarite com a participaçäo de linfócitos T, queratinócitos, citocinas e moléculas de adesäo. Requer terapia antiinflamatória potente.
Subject(s)
Humans , Purpura/therapy , Pigmentation Disorders/immunology , Pigmentation Disorders/therapy , Anti-Inflammatory Agents/pharmacokineticsABSTRACT
Se describe el caso de una lactante menor la cual presentó púrpura fulminante (PF) y coagulación intravascular diseminada (CID) a los dos meses de edad como manifestación de una probable deficiencia homocigota de proteína C (PC). La paciente presentaba además hemorragia del vítreo, sinequia extensa y desprendimiento de retina en el ojo izquierdo. Fue manejada con plasma fresco congelado(PFC) y heparina con buena evolución. En una ocasión, un intento por disminuir la frecuencia de las transfusiones con PFC a un régimen menor de dos veces al día condujo a un nuevo episodio de PF, el cual se controló al reinstituir el régimen de PFC a dos veces al día. Se instituyó tratamiento con warfarina, lo que permitió una supresión paulatina de la terapia con PFC: Se discuten los aspectos relevantes de la deficiencia de PC y se hace la revisión del tema
Subject(s)
Infant , Humans , Female , Protein C/analysis , Protein C/deficiency , Purpura/diagnosis , Purpura/therapy , Skin Diseases/diagnosis , Skin Diseases/physiopathologyABSTRACT
La discusión clínica se basará en el análisis de las principales causas de hepatoesplenomegalia en el período neonatal y en lactactes menores de tres meses de edad. Intentaré excluír rápidamente las afecciones menos frecuentes que cursan con hepatoesplenomegalia, para luego circunscribirme a los diagnósticos diferenciales más probables.
Subject(s)
Humans , Infant, Newborn , Infant , Purpura/diagnosis , Purpura/therapy , Splenomegaly/diagnosis , Splenomegaly/etiology , Primary Myelofibrosis/diagnosis , Hepatomegaly/diagnosis , Hepatomegaly/etiology , Neoplasms/diagnosis , Neoplasms/therapy , Leukemia, Monocytic, Acute/diagnosisABSTRACT
La discusión clínica se basará en el análisis de las principales causas de hepatoesplenomegalia en el período neonatal y en lactactes menores de tres meses de edad. Intentaré excluír rápidamente las afecciones menos frecuentes que cursan con hepatoesplenomegalia, para luego circunscribirme a los diagnósticos diferenciales más probables.(AU)
Subject(s)
Humans , Infant , Infant, Newborn , Neoplasms/diagnosis , Neoplasms/therapy , Purpura/diagnosis , Purpura/therapy , Leukemia, Monocytic, Acute/diagnosis , Primary Myelofibrosis/diagnosis , Hepatomegaly/diagnosis , Hepatomegaly/etiology , Splenomegaly/diagnosis , Splenomegaly/etiologyABSTRACT
This report summarizes the documented cases of homozygous protein C deficiency in the United States and Europe. Procedures for diagnosing and treating this disorder (both initially and over the long term) have been compiled by a working party on homozygous protein C deficiency of the Subcommittee on Protein C of the International Committee on Thrombosis and Haemostasis. Homozygous protein C deficiency is an autosomal recessive disorder that usually manifests itself by purpura fulminans and, less commonly, by massive large vein thrombosis; severe diffuse intravascular coagulation also develops in these infants, and there is evidence of intrauterine thrombosis. For confirmation of homozygous protein C deficiency in a neonate with purpura fulminans or massive venous thrombosis, the infant should have undetectable protein C activity and both parents should be heterozygous for protein C deficiency. At the onset of symptoms, the initial treatment should be plasma (8 to 12 ml/kg every 12 hours) until all lesions have healed. Two modalities for long-term treatment are accepted as useful in these children: oral anticoagulant therapy or protein C replacement (fresh frozen plasma or prothrombin complex concentrate). Liver transplantation has been performed in only one child, with success. Oral anticoagulation (vitamin K antagonists, maintaining the prothrombin time from one and one-half to two times control values or at the International Normalized Ratio of 2.5 to 4.4) is our recommendation of choice for long-term treatment. With appropriate care, these children are able to be free of coagulopathy and live relatively normal lives.
Subject(s)
Protein C Deficiency , Purpura/therapy , Anticoagulants/therapeutic use , Blood Coagulation Factors/therapeutic use , Child, Preschool , Humans , Infant , Infant, Newborn , Plasma , Purpura/diagnosis , Purpura/geneticsSubject(s)
Megakaryocytes/pathology , Purpura/chemically induced , Adolescent , Adult , Combined Modality Therapy , Diazepam/adverse effects , Ethanol/adverse effects , Female , Humans , Insecticides/adverse effects , Male , Meprobamate/adverse effects , Middle Aged , Oxyphenbutazone/adverse effects , Purpura/pathology , Purpura/therapy , Pyrazoles/adverse effects , Quinine/adverse effects , Sulfasalazine/adverse effects , Sulfonamides/adverse effects , Tolbutamide/adverse effectsSubject(s)
Humans , Plasmapheresis , Blood/metabolism , Blood Proteins/metabolism , Anemia, Hemolytic, Autoimmune/therapy , Purpura/therapy , Lupus Erythematosus, Systemic/therapy , Myasthenia Gravis/therapy , Purpura, Thrombocytopenic/therapy , ABO Blood-Group System , Antibodies , Neoplasms/therapy , Arthritis, Rheumatoid/therapyABSTRACT
We have seen six examples of autoerythrocyte sensitization in children that allow a definition of the disorder in the pediatric age group. The typical skin lesion is a painful, erythematous bruise that starts after minor trauma or surgery and often involves an area away from the injury site. The skin manifestations can be debilitating, and reappear unpredictably for an indefinite period. Somatic symptoms can be associated with the bruising. Usually the children have a disturbed psychological background. Characteristically, multiple detailed investigations are performed before the diagnosis is made. An intradermal injection of autologous red cells may or may not give a "positive" ecchymotic reaction. Pediatric patients seem to respond well to psychotherapy, which is usually the only effective form of treatment.