Lesiones cutáneas en la mano de una paciente trasplantada / Cutaneous lesions on the hand of a transplant recipient

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Eritema necrolítico migratorio: clave diagnóstica en el síndrome del glucagonoma / Necrolytic migratory erythema: A diagnostic clue in glucagonoma syndrome

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Rituximab for the treatment of lupus erythematosus panniculitis.

Lupus erythematosus panniculitis (LEP) or lupus profundus (LP) is a clinical variant of lupus eryhematosus that involves the deep dermis and the subcutaneous fat and is associated with tender subcutaneous nodules or plaques, with occasional ulceration, atrophy, and scarring. The management of this entity can be difficult because of the lack of response to conventional treatments, such as systemic steroids and antimalarials. The two patients of this study presented LP refractory to several therapies that demonstrated a remarkable improve to the infusion of the anti-CD20 monoclonal antibody, rituximab at a dosage of 375 mg/m²/week. After the first infusion, painful lesions had resolved without the appearance of new lesions. Rituximab may be an effective treatment for patients with LP when other therapies are ineffective. To date, there is only one case report of LEP treated with rituximab in the literature.

Two cases of cutaneous chronic graft versus host disease in treatment with psoralen plus ultraviolet-A-bath photochemotherapy.

BACKGROUND: Up to 50% of patients undergoing allogenic stem cell transplantation or bone marrow transplantation (BMT) can develop acute or chronic graft versus host disease (GVHD) as a severe complication. Immunosuppressive therapies may prove not only ineffective but may cause serious adverse effects. GVHD remains a major clinical problem and is often associated with high mortality rates. METHODS: This article presents the cases of a 48-year-old woman and a 23-year-old man, both presenting with severe cutaneous sclerodermic chronic GVHD following allogenic stem cell transplantation. Despite several years of treatment with oral corticosteroids, mycophenolate mofetil, sirolimus and acitretine, the skin lesions had not improved. Both patients were then treated with psoralen plus ultraviolet (PUVA)-bath photochemotherapy three times weekly, following a standardized treatment protocol. RESULTS: After a total accumulated dose of about 90 J/cm2, skin lesions in both patients had improved, showing complete remission in some body areas. Systemic corticosteroid treatment could be gradually reduced in the case of the female patient and skin conditions remained stable during maintenance treatment of PUVA-bath two times weekly and during a mean follow-up period of eight months. CONCLUSION: Oral PUVA therapy has been established as a successful treatment for acute and chronic GVHD, but unfortunately may result in systemic side effects. Psoralen plus ultraviolet-A-bath photochemotherapy provides clinicians with a therapeutic alternative that offers high clinical efficacy and safety. Therefore, PUVA-bath could be included as an alternative in the treatment protocol for chronic cutaneous GVHD.

Clofazimine as elective treatment for granulomatous cheilitis.

Cheilitis granulomatosa (CG) is a rare, idiopathic inflammatory disorder that usually affects young adults and clinically is characterized by diffuse, non-tender, soft to firm swelling of one or both lips. A variant of granulomatous cheilitis is Melkersson-Rosenthal syndrome when associated with facial paralysis and furrowed tongue. Several treatments have been used with variable success. We report 3 cases of GC treated with oral clofazimine 100 to 200 mg daily for 3 to 6 months obtaining regression of lesions in all treated cases. Hyperpigmentation and elevation of liver enzymes were observed as side effects.

Granuloma necrosante extravascular de Churg-Strauss / Extravascular necrotizing granuloma of Churg-Strauss

Las manifestaciones cutáneas de la granulomatosis alérgica de Churg-Strauss (síndrome de Churg-Strauss) se presentan en dos tercios de los pacientes. Presentamos el caso de una mujer de 37 años con un granuloma necrosante extravascular de Churg-Strauss como manifestación cutánea de la granulomatosis alérgica de Churg-Strauss. Aunque la presentación clínica era típica, fueron precisas tres biopsias cutáneas para su diagnóstico. El granuloma necrosante extravascular de Churg-Strauss consiste en un granuloma en empalizada con degeneración basófila del colágeno, infiltración por neutrófilos y leucocitoclasia. Aunque inicialmente descrito como específico de la enfermedad de Churg-Strauss, se ha observado en otras vasculitis sistémicas y en diversas enfermedades del tejido conectivo y linfoproliferativas. (AU)

Infección sistémica por Trichosporon asahii / Disseminated infection due to Trichosporon asahii

La tricosporinosis es una fungemia cada vez más frecuente en pacientes con neoplasias hematológicas; es producida por el Trichosporon asahii cuyo habitat es el suelo, agua y excreciones animales, aunque también forma parte de la flora normal de la piel y aparato respiratorio. En situaciones de neutropenia produce una diseminación con afectación pulmonar y renal de evolución fatal; en el 10 al 50% de los casos aparecen lesiones cutáneas. Se describe un caso de infección sistémica por Trichosporon asahii en una paciente de 40 años afecta de una leucemia mieloide aguda, de curso inicial poco sintomático y de una evolución rápida y fatal, con afectación cutánea, pulmonar y renal. La valoración clínica y el estudio histopatológico con cultivo de la piel fueron diagnósticas. Esta infrecuente fungemia debe tenerse en cuenta al valorar pacientes con neoplasias hematológicas en situación de neutropenia. Sólo el diagnóstico precoz puede evitar una diseminación mortal (AU)