Treatment of steroid-refractory chronic graft-versus-host disease with imatinib: Real-life experience of the Spanish group of hematopoietic transplantation (GETH).

Treatment of steroid-refractory chronic graft-versus-host disease (cGVHD) is a challenge. Here, we describe a retrospective analysis of 66 patients with steroid-refractory cGVHD treated with imatinib (starting dose of 100 mg in 70% of patients; maximum dose of 100-200 mg in 74%). Most patients had multi-organ involvement (≥2 organs, 83%), with the most affected being skin (85%), oral mucosa (55%), eyes (42%), and lungs (33%). The overall response rate was 41% (21 partial and three complete responses). The organ with the best response rate was the skin (46%), followed by gastrointestinal tract (43%), liver (41%), the oral mucosa (36%), eyes (29%), and lungs (18%). Imatinib led to steroid tapering in 17/38 patients. Twenty-five (38%) patients experienced imatinib-related adverse events, comprising extra-hematologic toxicity (n = 24, 36%) and hematologic toxicity (n = 6, 9%). No cases of grade 4-5 toxicity were reported. The main causes of imatinib discontinuation were treatment failure (52%) and toxicity (9%). After a median follow-up of 41 months, the 3-year overall survival was 81%, with no difference between imatinib responders and non-responders. These real-life results show that imatinib is safe and has moderate efficacy in patients with heavily pre-treated cutaneous sclerotic cGVHD; however, activity against lung cGVHD is very limited.

Rehabilitation Interventions in the Multidisciplinary Management of Patients With Sclerotic Graft-Versus-Host Disease of the Skin and Fascia.

Graft-versus-host disease (GVHD) is a multisystemic disorder that affects 30%-80% of patients who undergo allogeneic hematopoietic stem cell transplantation 10%-15% of GVHD patients develop sclerotic features affecting the skin or deeper tissues, leading to functional limitations and poor quality of life. There is limited literature regarding the indications and efficacy of specific rehabilitative interventions in sclerotic GVHD (sclGVHD). In this article, we summarize the current evidence supporting rehabilitation intervention in sclGVHD and offer our approach to the multidisciplinary management of this disease. In addition, we review techniques that have been employed in other sclerotic skin diseases (eg, iontophoresis, extracorporeal shock waves, botulinum toxin A, adipose derived stromal vascular fraction), but that require further validation in the sclGVHD setting. Ultimately, optimal care for this complex disease requires a multidisciplinary approach that includes a rehabilitation and adaptive program tailored to each patient's needs.

Trichomegaly induced by topical tacrolimus for the treatment of periorbital vitiligo: A brief report of a new adverse effect.

Trichomegaly is a known adverse effect of systemic administration of cyclosporine but is less commonly associated with systemic tacrolimus or with topical calcineruin inhibitors. In this report, we describe the first case, to our knowledge, of trichomegaly due to long-term use of topical tacrolimus for periocular vitiligo.

Epidermal consumption in benign and malignant melanocytic neoplasms.

Consumption of the epidermis associated with effacement of the rete ridge pattern has been cited as a useful criterion in the diagnosis of melanoma, but the significance of consumption in the absence of rete ridge effacement is unknown. We evaluated 701 melanocytic neoplasms for presence and 'grade' of consumption by melanocytic nests relative to diagnosis, body location, gender and age. We defined 1+ consumption as collections of melanocytes occupying greater than two thirds of the viable epidermis, with or without loss of the rete ridge pattern. Nests extending to the bottom of, within, and through the granular layer were graded 2+, 3+ and 4+, respectively. Consumption was more frequent and higher grades were found in melanomas followed by Spitz nevi compared with conventional melanocytic nevi (p < 0.001). Melanomas with higher Breslow thickness showed higher grades (p < 0.05). In conventional nevi, consumption occurred most frequently in back (13.7%), acral (11.9%) and scalp (9.8%) locations. Consumption without the requirement for rete ridge effacement occurs more frequently and at higher grades in melanoma. Higher grades correlate with higher Breslow thickness. Consumption is also common in Spitz nevi and occurs at lower grades in conventional (non-Spitz) nevi, especially on the back, the scalp and at acral sites.

Ingenol mebutate gel treatment for actinic cheilitis: report of four cases.

Actinic cheilitis (AC) are premalignant lesions that have an increased risk of malignant transformation. Their treatment, therefore, is essential to prevent carcinogenesis. However, optimal therapy is not well established and different modalities yield variable results. Ingenol mebutate gel has recently been approved by the US Food and Drug Administration for topical treatment of actinic keratosis, with high clearance rates. On the basis of these findings, we report our experience with this drug for the treatment of AC.

Mid-dermal elastolysis: report of a case and literature review.

Mid-dermal elastolysis (MDE) is an uncommon and probably underdiagnosed disorder of the elastic tissue. Clinical suspicion and histopathological confirmation are essential for the diagnosis. We report the case of a young woman who presented with this disorder after an inflammatory process during pregnancy and we review the main characteristics of this rare entity.

Debut de hepatitis crónica autoinmune como prúrigo nodular / Autoimmune chronic hepatitis presenting as prurigo nodularis

No disponible

Calcifilaxia no urémica en una paciente con artritis reumatoide / Non-uremic calciphylaxis in a female patient with rheumatoid arthritis

No disponible