Adult purpura fulminans associated with staphylococcal infection and administration of colony-stimulating factors.

Purpura fulminans (PF) is a rare syndrome of progressive haemorragic necrosis due to disseminated intravascular coagulation (DIC) and dermal vascular thrombosis leading to purpura and tissue necrosis. PF is more often associated with either a benign infection or a severe sepsis. Rarely, it has been related to drug intake. We report the case of a 24-year-old female patient who suffered from staphylococcal sepsis and pancytopenia, for which she was treated with antibiotics, granulocyte-colony stimulating factor (G-CSF) and granulocyte/macrophage CSF (GM-CSF). Two days after the last GM-CSF dose, she developed widespread necrotic plaques with erythematous borders and purpura in the breast, arms and legs. Coagulation tests indicated DIC and a skin biopsy showed fibrin thrombi in the superficial dermal vessels. The patient totally recovered after removal of the necrotic tissues and application of skin autografts. Although staphylococcal infection was most probably involved in the development of PF, a role of CSF cannot be excluded in this case.

Dermatosis autoinfligida: presentación de un caso y revisión / Self-inflicted dermatosis: presentation of a case and review

Las dermatosis autoinfligidas representan un motivo habitual de consulta, así como un desafío diagnóstico y terapéutico. Se caracterizan por la autoprovocación de lesiones cutáneas de manera consciente o inconsciente en el contexto de trastornos psiquiátricos complejos. Comunicamos el caso de una paciente que consultó al Servicio de Dermatología por úlceras irregulares y sobreinfectadas localizadas en rostro, de 2 meses de evolución. El caso representó una dificultad diagnóstica ya que solo a partir del interrogatorio exhaustivo y los exámenes complementarios, que descartaron otras patologías, se arribó al diagnóstico de úlceras autoprovocadas en contexto de patología psiquiátrica que requirió manejo interdisciplinario. (AU)

Self-inflicted dermatoses are a frequent cause for consultation and represent a diagnostic and therapeutic challenge. They are characterized by the conscious or unconscious self-generation of cutaneous lesions in the context of complex psychiatric disorders. We report the case of a patient who consulted at the dermatology department with two months history of irregular and superinfected ulcers located on the face. This case represented a diagnostic difficulty since only from an exhaustive interrogation and complementary examinations, which ruled out other pathologies, we arrived at the diagnosis of self-inflicted ulcers in the context of psychiatric pathology that required interdisciplinary management. (AU)

Outcomes in 11 patients with dermatofibrosarcoma protuberans treated with Mohs micrographic surgery.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon intermediate-grade fibrohistiocytic sarcoma. It occurs most often in adults aged 20-50 years and is associated with local invasion and a high recurrence rate. Uncontrolled local disease or metastases may result in death. Treatment has involved wide excision, Mohs micrographic surgery (MMS) and other approaches. The purpose of the current study was to review our experience with MMS in the treatment of patients with DFSP over the past six years. METHODS: We carried out a retrospective chart review for all patients treated with MMS at the Hospital Italiano de Buenos Aires during a six-year period to October 2009. Patient data included age and sex, site, and prior surgical treatment (if any) of the tumor and details of the Mohs procedure. RESULTS: Eleven patients were treated for DFSP. Four (36.4%) patients had been previously treated with a standard wide excision. Three (27.3%) of the 11 tumors were located on the back, four (36.4%) on the upper extremity, one (9.1%) on the lower extremity, and three (27.3%) on the trunk. Mean lesion size at presentation was 5.16 cm(2), and mean defect size was 12.65 cm(2), yielding a difference of 7.49 cm(2) and a ratio (defect size/lesion size) of 2.45. No tumors recurred after MMS. CONCLUSIONS: The recurrence potential of DFSP is directly related to the extent of resection. Mohs micrographic surgery with continuous histological margin control allows for maximum tissue preservation and low recurrence rates and is rapidly emerging as a first-line treatment modality for this condition.

Desafíos terapéuticos en la enfermedad injerto contra huésped / Therapeutic challenges in graft versus host disease

La enfermedad injerto contra huésped es una entidad en la cual las células inmunológicas competentes de un tejido injertado reconocen y dañan antígenos presentes en el receptor del trasplante, que es incapaz de defenderse de ellas. Es una complicación frecuente del trasplante alogénico de médula ósea, y con menor frecuencia se produce luego de trasplantes de órganos sólidos o transfusiones de hemoderivados no irradiados. Se comunica el caso de una paciente de sexo femenino de 23 años, con leucemia linfoblástica aguda.y trasplante alogénico de médula ósea, que presentó una enfermedad injerto contra huésped con compromiso cutáneo y gastrointestinal dependiente de corticoides, con mejoría de los signos y síntomas cutáneos luego del tratamiento con infliximab y fotoféresis extracorpórea. (AU)

Graft versus host disease is an entity in which competent grafted immune cells recognize and damage tissue antigens present in the transplant recipient, who is unable to defend from them. It is one of the most serious complications in patients undergoing allogeneic bone marrow transplantation, although less frequently it may be associated with solid organ transplants or transfusions of not irradiated blood products. We report the case of a 23 year-old patient with acute lymphoblastic leukemia and allogeneic bone marrow transplantation, that presented graft versus host disease with skin and gastrointestinal involvement, dependent on corticosteroids, that showed improvement in signs and skin symptoms after treatment with infliximab and extracorporeal photopheresis. (AU)

Serie parasitosis en dermatología. Miasis: diferentes formas de presentación clínica / Parasitic series in dermatology. Myiasis: different forms of clinical presentation

La miasis es una infestación por larvas en desarrollo de una gran variedad de moscas del orden Díptera. Según el sitio de invasión, se clasifica en miasis cavitarias, que pueden deberse a invasión de cavidades naturales o de heridas, y miasis forunculoide, cuando atraviesa piel indemne. Esta infestación presenta una distribución mundial, con variaciones estacionales en relación con la latitud geográfica y el ciclo de vida de distintas especies de moscas. Presentamos una serie de tres casos de pacientes con distintas formas clínicas de manifestación de miasis. (AU)

Myiasis is the tissue infestation by a variety of Diptera order larvae flies. According to the invasion site, they are classified in myiasis of cavities, which can be because of an invasion of natural cavities or wounds, and furuncular myiasis, when they invade through intact skin. This infestation has a worldwide distribution, with seasonal variations in relation to the geographic latitude and the life cycle of different species of flies. We present three cases of patients with different clinical forms of presentation of myiasis. (AU)

Nuevas posibilidades terapéuticas en melanoma metastásico / Novel therapeutics possibilities for metastatic melanoma

El melanoma ha experimentado un aumento constante en su tasa de incidencia en las últimas cinco décadas a nivel mundial. El pronóstico del paciente con melanoma se relaciona con el estadio de la enfermedad al momento del diagnóstico, con una sobrevida global media de 6,2 meses en pacientes con melanoma metastásico. El avance en las investigaciones sobre la biología y el comportamiento tumoral permitió el desarrollo de nuevas terapias con distintos mecanismos de acción y mayor eficacia. En esta revisión se abordan las terapias biológicas en melanoma metastásico, su mecanismo de acción y principales resultados en ensayos clínicos. (AU)

Melanoma has experienced a consistent increase in incidence over the past five decades worldwide. The prognosis of patients with melanoma is related to the stage of disease at diagnosis, with a median overall survival of 6.2 months in metastatic melanoma. Progress in research on tumor biology allowed the development of new therapies with different mechanisms of action and greater efficiency. In this review, biologic therapies in metastatic melanoma, its mechanism of action and main results in clinical trials are discussed. (AU)

Síndrome de Laugier-Hunziker / Laugier-Hunziker syndrome

El síndrome de Laugier-Hunziker es un trastorno pigmentario adquirido poco frecuente, caracterizado por presentar lesiones hiperpigmentadas cutaneomucosas idiopáticas que pueden asociarse a melanoniquia longitudinal. A pesar de ser considerado una enfermedad benigna sin manifestaciones sistémicas ni potencial maligno, es clave realizar el diagnóstico diferencial con otros trastornos pigmentarios, en especial con el síndrome de Peutz-Jeghers. Se presenta aquí el caso de un paciente con este síndrome poco frecuente. (AU)

Laugier-Hunziker syndrome is a rare acquired pigmentary disorder that is characterized by idiopathic mucocutaneous pigmentation that may be associated with longitudinal melanonychia. Although this syndrome is considered a benign disease with no systemic manifestations or malignant potential, it is important to rule out other mucocutaneous pigmentary disorders, especially Peutz-Jeghers syndrome. We report the case of a patient with this unusual syndrome. (AU)

Serie parasitosis en dermatología: escabiosis / Series parasitosis in dermatology: scabies

La escabiosis es una ectoparasitosis pruriginosa producida por el ácaro Sarcoptes scabiei, variedad hominis, específica del ser humano. Si bien su distribución es universal, con frecuencia es subdiagnosticada por asociarla únicamente a hacinamiento y malos hábitos de higiene. Se transmite por contacto directo con una persona afectada o a través de fómites, por lo que es muy común el contagio de los convivientes. Presentamos un caso de escabiosis en una paciente anciana evaluada por prurito generalizado. (AU)

Scabies is a human specific pruritic ectoparasitosis produced by the mite Sarcoptes scabiei var. hominis. Although it has a worldwide distribution, it is often underdiagnosed because it is only associated with overcrowding and poor hygiene. It is transmitted by a direct contact with an affected person or through fomites. The transmission to cohabitants is very common. We present a case of scabies in an elderly patient with generalized pruritus. (AU)

Disseminated strongyloidiasis in immunocompromised patients--report of three cases.

BACKGROUND: Strongyloides stercoralis is an intestinal nematode of humans. The characteristic cutaneous manifestation of strongyloidiasis is larva currens. Patients with suppressed immunity can develop a severe disseminated strongyloidiasis involving wide spread of the larvae to extraintestinal organs, outside the usual migration pattern. Patients with cell-mediated immunodeficiency and on corticosteroid therapy appear to be at highest risk for the development of this highly fatal entity. METHODS: We present three patients with disseminated strongyloidiasis. All patients were immunocompromised and were undergoing corticosteroid therapy. Physical examination revealed a rapidly progressive purpuric petechial eruption with a reticulated pattern, mainly over the abdomen. RESULTS: The histopathologic findings of the skin biopsies revealed a purpuric lymphomonocytic vasculitis that compromised the superficial dermis. In one skin biopsy, a larva was identified. None of our patients had eosinophilia. Strongyloides stercoralis was isolated in the fluid of the three patients, either before or after they died. CONCLUSION: The risk of disseminated strongyloidiasis in patients with impaired cell-mediated immunity is unknown; however, given the poor prognosis of disseminated strongyloidiasis, consideration should be given to the screening of patients at increased risk of infection.

Use of sodium thiosulfate in the treatment of calciphylaxis.

Calciphylaxis is an infrequent but severe entity found in chronic dialysis patients. Its clinical pattern consists of tissue ischemia with itchy and painful subcutaneous nodules and plaques, most often located on the abdomen, buttocks, thighs and/or legs. These injuries evolve to extensive superficial necrosis of the skin overlying the panniculitis, with ulceration, overinfection and consequent sepsis. Current treatment modalities used to counteract this pathology are not entirely effective. A new treatment reported for calciphylaxis, is the use of intravenous sodium thiosulfate. This inorganic salt is already used in the treatment of intoxication caused by cyanide, in patients with calcific nephrolithiasis and tumoral calcinosis, with very good and safe results. We herewith report a case of calciphylaxis that was cured using intravenous sodium thiosulphate treatment.

Lymphocutaneous nocardiosis and cutaneous pheohyphomycosis in a liver transplant recipient.

BACKGROUND: Infections are the leading cause of morbidity and mortality in transplanted patients. The increasing number of immunocompromised patients has not only augmented infections by specific pathogens, but also by opportunistic microbial agents. METHODS: A mixed cutaneous infection caused by Nocardia brasiliensis and Exophiala jeanselmei is reported in a liver transplant patient. RESULTS: The cutaneous lesions were painful nodules which drained purulent material. They were located on the right lower limb, with lymphadenopathies in the groin. CONCLUSIONS: The patient was treated with itraconazole (600 mg/day) plus trimethoprim (1600 mg/day)-sulfamethoxazole (320 mg/day) for 8 weeks, with complete remission of the lesions.

Penfigoide ampollar por fármacos: entidad subdiagnosticada? / Drug-Induced Bullous Pemphigoid: an Underdiagnosaed Entity?

El penfigoide ampollar (PA) es una enfermedad infrecuente, de curso crónico y benigno, que aparece en personas de edad avanzada y se caracteriza por la presencia de ampollas subepidérmicas.En términos generales, el diagnóstico de las enfermedades ampollares se basa en las manifestaciones clínicas, los hallazgos histopatológicos y la inmunofluorescencia directa. Si bien la ausencia de alguno de estos métodos puede dificultar el mismo, una adecuada correlación clínico-patológica permite, en la mayoría de los casos, arribar al diagnóstico y realizar el tratamiento apropiado. El PA puede ser causado por fármacos y produce cuadros clínicos similares al PA idiopático. A continuación se presentan dos casos con diagnóstico de penfigoide ampollar por fármacos.

Bullous Pemphigoid is a chronic, infrequent benign disease of the elderly, characterized by the presenceof subepidermal bullae. Diagnosis is based on clinical, histopathological and direct immunofluorescencefindings. Though the absence of any of them hampers the diagnosis, a correct clinico-pathologic correlation is necessary to make the appropriate treatment. Drug induced-BullousPemphigoid presents with identical clinical features as those of the Idiopathic Bullous Pemphigoid.We present two patients with drug-induced Bullous Pemphigoid.

Expresiones cutáneas de candidiasis sistémica en pacientes inmunosuprimidos / Cutaneous expression of systemic candidiasis in immunosuppressed patients

La candidiasis sistémica constituye una importante causa de morbimortalidad en pacientes internados en centros de alta complejidad. Las expresiones cutáneas de la infección son múltiples y se observan en un pequeño porcentaje de pacientes. Se presentan cinco casos de candidiasis sistémica con compromiso cutáneo en pacientes inmunosuprimidos: un paciente trasplantado de médula ósea, dos pacientes con leucemia mieloide aguda, un caso de sepsis bacteriana y un paciente con linfoma linfocítico. Todos los pacientes presentaron fiebre persistente y lesiones cutáneas que se biopsiaron para estudio histológico y cultivo. La histopatología mostró seudohifas en tres de los casos. En los cultivos de piel se identificó a Candida tropicalis, Candida Krusei y Candida albicans en dos pacientes. El incremento de cepas resistentes hace necesario el empleo de nuevos antifúngicos como las equinocandinas para el tratamiento de esta entidad.

Systemic candidiasis is a signifi cant cause of morbidity and mortality in immunosuppressed hospitalized patients. Skin signs of systemic candi-diasis are numerous although they can be observed in a small percent-age of patients. We report fi ve cases of systemic candidiasis with cutaneous manifestations in immunosuppressed patients: one case of bone- marrow transplantation, two patients with acute myeloid leukaemia, one with bacterial sepsis and one patient with lymphocitic lymphoma. The patients presented persistent fever and cutaneous lesions. Skin biopsy for histopathological study and cultures were carried out. The histological examination showed yeasts in three of the cases. Skin culture revealed: Candida tropicalis, Candida krusei and Candida albicansin two cases. Due to the increase of resistant species new drugs such as echinocandins are currently available for the treatment of this entity.

Cutaneous necrosis by calcific uremic arteriolopathy.

BACKGROUND: Calcific uremic arteriolopathy is a rare and serious disorder characterized by systemic medial calcification of the arteries and tissue ischemia. Most often it is found in patients with chronic renal failure on dialysis and in renal transplant recipients with secondary hyperparathyroidism. METHODS: We report six patients with end-stage renal disease [five on hemodialysis (one with a nonfunctioning renal graft) and one on peritoneal dialysis] who developed painful livedo reticularis and skin necrosis of the limbs. All had secondary hyperparathyroidism and elevated calcium-phosphorus product. Our patients presented with the following clinical features: white race (six patients), hypoalbuminemia (three patients), diabetes (one patient), and obesity (four patients). RESULTS: Subtotal parathyroidectomy was performed in three cases. Despite this procedure, two patients died; one patient survived and his lesions healed. One patient was treated with aggressive wound care and hemodialysis (with low dialysate calcium concentration and Renagel phosphorus binders) and one patient received only local wound care, both with improvement of their lesions. In one case, no therapy was performed because the patient died immediately after diagnosis. CONCLUSIONS: The three patients who survived (Cases 4, 5 and 6) had distal lesions, normal serum albumin, and an early diagnosis. There was a relationship between the outcome of the patients and these factors, rather than the type of treatment received.

Calcinosis cutis following liver transplantation in a pediatric patient.

We report the occurrence of calcinosis cutis in a 3-year-old girl after liver transplantation. The cutaneous lesions consisted of 5 mm white papules on an erythematous base in linear and rosette configurations that developed in the abdominal and lumbar areas 10 days after transplantation. The patient had received calcium chloride solution intravenously during surgery. We excluded other causes of ectopic calcification such as hyperparathyroidism, renal failure, and extravasation of calcium solution. We discuss the etiology of calcinosis cutis after liver transplantation. This sequence of events has not been previously described in pediatric patients.

Enfermedad de morbihan / Morbihan´s disease

La enfermedad de Morbihan (morbus Morbihan) es una entidad poco frecuente, que se caracteriza por la aparición recurrente, a nivel del tercio medio y superior del rostro, de eritema y edema, que conducen a una induración desfigurante. Actualmente se plantea que la urticaria de contacto inmunológica desempeña un rol crucial en la patogenia. Presentamos un paciente de 65 años con enfermedad de Morbihan de 2 años de evolución.

Morbihan’ s disease (morbus Morbihan) is an infrecuent entity, characterized by the appearance of relapsing erithema and edema on the center and upper aspect of the face, leading to disfi guring induration.Nowadays, immunologic contact urticaria plays a crucial role in the pa-togenesis. We present a 65 year-old patient with Morbihan’ s disease, with its onset 2 years ago.

Síndrome de Sweet asociado a neoplasias / Sweet's syndrome associated with neoplasms

El síndrome de Sweet fue descrito en el año 1964 por Robert Douglas Sweet, como una entidad a la cual denominó dermatosis neutrofílica febril y aguda. Se caracteriza por cinco rasgos principales: 1) aparición brusca de placas eritemato-dolorosas en cara, cuello y extremidades; 2) fiebre; 3) leucocitosis polimorfonuclear; 4) denso infiltrado dérmico a predominio neutrofilico; 5) rápida respuesta al tratamiento esteroideo. Se puede clasificar en cinco grupos: idiopático, parainflamatorio, paraneoplásico, secundario a drogas y asociado a embarazo. En el 20 por ciento de los casos se asocia a enfermedades malignas, representando las hematológicas el 85 por ciento y los tumores sólidos el 15 por ciento restante. Se presenta una serie de siete casos de síndrome de Sweet asociado a neoplasias, diagnosticados durante el período 2002-2006, de los cuales seis correspondieron a enfermedades oncohematológicas y el restante a tumores sólidos. Como comentario de dicha casuística, se hace hincapié en la importancia del diagnóstico de este síndrome, debido a que puede anunciar la recaída del tumor o la progresión de la enfermedad de base. De esta manera, mediante el uso de métodos de diagnóstico y tratamiento oportunos, se lograría mejorar la calida de vida de estos pacientes. También debe tenerse en cuenta, que los pacientes oncológicos reciben múltiples medicaciones (factor estimulante de colonias), que pueden estar implicadas en la aparición de esta entidad, debiendo ser las mismas descartadas como posibles causas.

Sweet's syndrome was described in 1964 by Robert Douglas Sweet, as an entity he named acute febrile neutrophilic dermatosis. It is characterized by five main features: 1) sudden appearance of erythematous and tender plaques on the face, neck and extremities; 2) fever; 3) polymorphonuclear leukocytes; 4) predominantly neutrophilic dense infiltrate in the dermis, and 5) rapid response to steroid therapy. Sweet's syndrome can be classified into five groups: idiopathic, parainflammatory, paraneoplastic, drug-induced, and pregnancy-related. Twenty percent of cases are associated with malignancies; 85 percent out of them involve hematologic alignancies and the remaining 15 percent, solid tumors. A series of seven cases of Sweet's syndrome associated with neoplasms which were diagnosed from 2002 to 2006 is presented. Six cases were related to oncohematologic diseases and one to solid tumors. These results highlight the importance of the diagnosis of the syndrome, since it may predict tumor relapse or underlying disease progression. The timely use of diagnostic and treatment methods may improve the quality of life of these patients. The fact that oncology patients take multiple medications (a colony-stimulating factor) which may be associated with the onset of this entity must also be considered in excluding possible causes.