The use of Goeckerman therapy in managing erythrodermic psoriasis resistant to multiple medications.

Erythrodermic psoriasis is a relatively rare, more dangerous inflammatory variant of psoriasis associated with high morbidity and mortality. It can be exceptionally challenging to manage, defeating even the most experienced dermatologist's arsenal of treatment strategies. Goeckerman therapy, a regimen of ultraviolet B phototherapy and crude coal tar, has demonstrable efficacy in severe and recalcitrant plaque-type psoriasis. However, its utility in erythrodermic psoriasis has not been explored within the dermatology literature. Herein, we present a patient with a long-standing history of erythrodermic psoriasis refractory to eleven treatment modalities including four biologic agents, who had his erythroderma 'turned around' following Goeckerman therapy. 'Turned around' is used to describe dramatically reducing a patient's cutaneous inflammation so that previously recalcitrant disease can now respond to maintenance therapy. The importance of a one to three week 'cool down' period of topical corticosteroid therapy prior to phototherapy or crude coal tar use is highlighted in this case as well. Although Goeckerman therapy is no longer regularly used, it remains one of the most efficacious treatments available for intractable psoriasis, attracting patients from all over the country desperate for symptom relief. This case suggests it may be useful in 'turning around' extremely difficult-to-treat erythrodermic psoriasis as well.

Psoriasis: presentación de un caso y revisión bibliográfica / Psoriasis case presentation and review

Antecedentes: La psoriasis es una enfermedad inmune crónica con manifestaciones dermatológicas y articulares. Puede presentarse a cualquier edad pero ocurre con mayor frecuencia en dos picos: 20-30 años y 50-60 años. Tiene un componente genético fuerte porque aproximadamente el 30% de los pacientes tienen un familiar de primer grado con la enfermedad, y sus hijos pueden de-sarrollar artritis psoriásica sin alteraciones cutáneas. El paciente experimenta pocos periodos de remisión espontanea.Caso clínico: Mujer de 39 años, ama de casa del casco rural de Tela, diagnosticada con psoriasis hace 7 años. Acude a consulta en buen estado general sin secuelas psicológicas, con lesión en placa localizada en codo derecho con área afectada del 4% y eritrodermia psoriásica que cubre la totalidad del abdomen en un 9% de la supericie corporal. Reiere mejoría con los productos naturales. Actualmente con sobrepeso (27 IMC) y con hipertensión sistólica aislada (140/82 mmHg) sugerente de ateroesclerosis. Discusión: Se deben tomar en cuenta los aspectos psico-sociales y metabólicos que afectan la calidad de vida de estos pacientes, para tratarlos de forma inte-gral con un grupo multidisciplinario. El médico general puede manejar la psoriasis en casos concretos como las placas localizadas ≤ 5% del BSA, en los cuales el tratamiento indicado es tópico con corticoesteroides de alta potencia como primera línea. Para todo lo demás, el paciente con psoriasis debe ser evaluado por un dermatólogo.

Diagnosis and pharmacological treatment of chronic actinic dermatitis in the elderly: an update.

Chronic actinic dermatitis (CAD) describes a condition resulting from abnormal photosensitivity; the dermatitis is clinically similar to contact allergic dermatitis. Sun-exposed skin is more commonly affected but the condition can extend to and encompass unexposed skin. CAD is relatively rare but becomes more prevalent in the elderly population. Phototesting, patch testing and laboratory results should be used to help guide diagnosis. In the elderly, it is important to distinguish CAD from drug-induced photosensitivity. Management of the condition requires sunlight avoidance and use of sunscreens, topical emollients and topical corticosteroids. Oral corticosteroids and immunosuppressive therapy such as azathioprine may be indicated but should be used with caution in the elderly.

Carbamazepine induced DRESS syndrome.

We present here an 18-yr-old male who presented with intermittent fever of moderate grade and of 15 days duration, followed by maculopapular erythematous rashes over upper and lower extremities, face, and trunk developing over 10-12 days. He was suffering from recurrent seizures since last 3 months for which he was started on carbamazepine 200mg twice daily for the past 6 weeks. He was febrile on admission. Generalized lymphadenopathy with discreet, non-matted, firm and tender inguinal lymph nodes. Patch test with 1% and 5% solution of carbamazepine was strongly positive.

Documento de consenso sobre la evaluación y el tratamiento de la psoriasis moderada/grave del Grupo Español de Psoriasis* de la Academia Española de Dermatología y Venereología / Consensus Document on the Evaluation and Treatment of Moderate-to-Severe Psoriasis. Spanish Psoriasis Group of the Spanish Academy of Dermatology and Venereology

El tratamiento de la psoriasis se ha visto revolucionado por la introducción de los agentes biológicos, que permiten obtener blanqueamientos y mejorías a largo plazo sin el riesgo de toxicidad que ha venido limitando el tratamiento de estos pacientes con agentes sistémicos clásicos. Se ha acumulado abundante evidencia científica con respecto a la eficacia y seguridad de los agentes biológicos, que ha llevado a revisar el papel del tratamiento sistémico en general y ha permitido establecer nuevos objetivos y estrategias terapéuticas en los pacientes con psoriasis moderada a grave. En este contexto nuevo se hace necesario establecer, de forma consensuada por especialistas expertos y basada en la evidencia científica disponible, criterios de gravedad que justifiquen la indicación de los diferentes tratamientos sistémicos actualmente disponibles para la psoriasis, así como objetivos terapéuticos y parámetros de eficacia, estrategias de tratamiento, cribaje de los pacientes y selección del tratamiento basada en criterios de beneficio/riesgo. El presente documento recoge el consenso del Grupo Español de Psoriasis en estos diferentes aspectos del manejo de la psoriasis moderada a grave (AU)

The treatment of psoriasis has been revolutionized by the introduction of biologic agents; these agents achieve skin clearance and long-term improvement without the risk of toxicity that has limited use of the classic systemic treatments. The role of systemic treatment in the management of psoriasis is being reviewed on the basis of a large volume of scientific evidence on the efficacy and safety of biologic agents, and new therapeutic goals and strategies are being devised for patients with moderate-to-severe psoriasis. This has led to the need to establish severity criteria that will provide the rationale for the indication of the different systemic agents currently available for the treatment of moderate-to-severe psoriasis, as well as therapeutic goals, efficacy measures, therapeutic strategies, screening protocols, and choice of treatment based on the risk-benefit ratio of the different agents. These criteria must be established through consensus by experienced dermatologists and based on available scientific evidence. The present document reflects the consensus of the Spanish Psoriasis Group on these different issues in the management of moderate-to-severe psoriasis (AU)

Eritrodermia psoriásica y psoriasis pustulosa generalizada: tratamiento con etretinato / Psoriatic erythroderma and generalized pustular psoriasis: treatment with etretinate

Se presentan dos formas graves de psoriasis infantil, el primero para remarcar los factores precipitantes tales como el uso de corticoides orales y PUVA en la eritrodermia psoriásica y el segundo por las escasas comunicaciones de psoriasis pustulosa generalizada en niños menores de un año de edad; se comenta el tratamiento exitoso con el etretinato en ambos casos. (AU)

Eritrodermia psoriásica y psoriasis pustulosa generalizada: tratamiento con etretinato / Psoriatic erythroderma and generalized pustular psoriasis: treatment with etretinate

Se presentan dos formas graves de psoriasis infantil, el primero para remarcar los factores precipitantes tales como el uso de corticoides orales y PUVA en la eritrodermia psoriásica y el segundo por las escasas comunicaciones de psoriasis pustulosa generalizada en niños menores de un año de edad; se comenta el tratamiento exitoso con el etretinato en ambos casos.

Ofuji papuloerythroderma in a patient with the acquired immunodeficiency syndrome.

Ofuji papuloerythroderma (OPE) is a distinctive clinical entity of unknown etiology which occasionally may be associated with B-cell and T-cell lymphomas and visceral malignancy. We describe a case of OPE in a male with the acquired immunodeficiency syndrome. To our knowledge, this is the first report of OPE in a patient infected by the human immunodeficiency virus.

Efficacy of ultraviolet B phototherapy for psoriasis in patients infected with human immunodeficiency virus.

To evaluate the efficacy of ultraviolet B (UVB) phototherapy for the treatment of psoriasis in patients infected with human immunodeficiency virus (HIV), the response of 14 patients was compared to that of matched seronegative control individuals. All patients were evaluated prior to treatment (baseline) and after 21 treatments for the extent of total body surface area (TBSA) involvement and the quantification of scale, erythema, and thickness of plaques using a scale of 0 (absent) to 4 (severe). The only concomitant medication allowed was salicylic acid in petrolatum. The cumulative score for scale, erythema, and thickness improved 1.9 +/- 0.5 [mean +/- standard error of mean (SEM)] in the HIV group and 2.4 +/- 0.3 in controls. There was 40.9 +/- 7.3% reduction of TBSA involvement in the former and 38.4 +/- 7.6% reduction in the latter group. None of the differences was statistically significant. There was no statistically significant difference in the response to therapy among various stages of immunosuppression in the HIV group. There was also no deterioration of immune status in this group. UVB phototherapy is an effective treatment for psoriasis in patients infected with HIV. The response is identical to that of matched control individuals.

HTLV-I positive progressive spastic paraparesis (TSP) associated with a lymphoid disorder in three Chilean patients.

We describe the clinical and laboratory features in three Caucasian Chilean patients with tropical spastic paraparesis (TSP) associated with/or preceded by a lymphoproliferative disorder involving cutaneous lesions and localised lymphadenopathy. The neurological symptoms and signs were characteristic of TSP and CSF examination revealed the presence of oligoclonal bands. All three patients had a moderate leucocytosis (10-14 x 10(9)/l) with eosinophilia and a minority (2-4%) of circulating atypical polylobed or ATLL-like lymphocytes. Lymph node histology showed a diffuse pattern of infiltration (1 case) and marked expansion of the paracortical zone with convoluted lymphocytes and immunoblasts (2 cases). Skin biopsy demonstrated a dermal lymphoid infiltration with epidermotropism. Antibodies to HTLV-I were detected in the serum and CSF in the three patients and Southern blot analysis of peripheral blood mononuclear cells showed a monoclonal integration of HTLV-I proviral DNA in one case whereas in the two others the pattern was indicative of low level polyclonal integration. All three patients were treated with prednisolone and one with PUVA with transient partial response on the skin and neurological manifestations. Two patients died months to 5 years from presentation and the other is alive 12 years from diagnosis with active neurological and skin disease. The simultaneous occurrence of HTLV-I associated TSP with smouldering ATLL and a cutaneous ATLL or pre-leukaemic form is discussed.

Etretinate in severe psoriasis of children.

Ten children, five with generalized pustular psoriasis and five with erythrodermic disease, were treated with etretinate and observed for up to seven years. In all those with pustular psoriasis, complete clearing was obtained in three weeks to four months, but in one child maintenance therapy had to be introduced twice, for one year each time. In those with erythrodermic psoriasis, the results were favorable only in two, and in two it was necessary to introduce Re-PUVA. Clinical side effects were slight, and laboratory examinations did not disclose any significant abnormalities. In one child who was treated intermittently for seven years, focal osteoporosis of one tibia was disclosed by radiography. No adverse effects on children's development were noticed.

Pre-Sézary syndrome.

Eighteen patients with erythroderma, recurrent cycles of circulating Sézary cells of less than 1,000 cells/mm3, and a chronic course were followed for a mean time of nearly 5 years and were diagnosed as having pre-Sézary syndrome. Only one patient died, and none developed lymphoproliferative disease. All ten patients who underwent patch testing showed positive results. The elevation of IgE was striking when this group was compared with a group with Sézary syndrome. Most patients achieved partial or complete remission on low-dose chlorambucil and prednisone therapy. Some patients had lymphocytic or lymphomatoid bands on skin biopsy specimens and were like previously reported patients with pre-Sézary syndrome whose condition progressed to Sézary syndrome. A nontoxic chemotherapy or an anti-T cell treatment program can control this chronic erythroderma state.