Brentuximab vedotin in the treatment of cutaneous T-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry.

BACKGROUND: Brentuximab vedotin (BV) has been approved for CD30-expressing cutaneous T-cell lymphoma (CTCL) after at least one previous systemic treatment. However, real clinical practice is still limited. OBJECTIVES: To evaluate the response and tolerance of BV in a cohort of patients with CTCL. METHODS: We analysed CTCL patients treated with BV from the Spanish Primary Cutaneous Lymphoma Registry (RELCP). RESULTS: Sixty-seven patients were included. There were 26 females and the mean age at diagnosis was 59 years. Forty-eight were mycosis fungoides (MF), 7 Sézary syndrome (SS) and 12 CD30+ lymphoproliferative disorders (CD30 LPD). Mean follow-up was 18 months. Thirty patients (45%) showed at least 10% of CD30+ cells among the total lymphocytic infiltrate. The median number of BV infusions received was 7. The overall response rate (ORR) was 67% (63% in MF, 71% in SS and 84% in CD30 LPD). Ten of 14 patients with folliculotropic MF (FMF) achieved complete or partial response (ORR 71%). The median time to response was 2.8 months. During follow-up, 36 cases (54%) experienced cutaneous relapse or progression. The median progression free survival (PFS) was 10.3 months. The most frequent adverse event was peripheral neuropathy (PN) (57%), in most patients (85%), grades 1 or 2. CONCLUSIONS: These results confirm the efficacy and safety of BV in patients with advanced-stage MF, and CD30 LPD. In addition, patients with FMF and SS also showed a favourable response. Our data suggest that BV retreatment is effective in a proportion of cases.

Primary cutaneous lymphomas in children: A prospective study from the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry.

BACKGROUND/OBJECTIVES: Primary cutaneous lymphomas are rare in pediatric patients. The clinical and histopathological manifestations may differ from those in adults. Due to their low frequency and the insidious clinical picture, the diagnosis is usually delayed. The Spanish Primary Cutaneous Lymphoma Registry was initiated in 2016 as a multicenter registry that would allow better insight into the epidemiological, clinical, histopathological, and treatment response characteristics of patients with primary cutaneous lymphomas. METHODS: We conducted a prospective observational cohort study of primary cutaneous lymphomas in pediatric patients participating in the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry. RESULTS: At the time of the analysis, 10 patients under 18 years of age out of 799 all-age cases (1.25%) had been included in the registry (7 males, 3 females). The mean age at diagnosis was 9.7 years (SD: 4.8). Seven (70%) had mycosis fungoides, 2 of them had the folliculotropic variant; and 3 (30%) had primary cutaneous marginal zone B-cell lymphoma. CONCLUSIONS: Primary cutaneous lymphomas are extremely rare in pediatric patients and usually have a good prognosis. Therefore, a high level of suspicion is necessary for the diagnosis. We suggest management by experienced physicians and follow-up into adulthood.

Pseudolymphomatous folliculitis.

Pseudolymphomatous folliculitis is a benign entity that is included within pseudolymphomas. Because of its rapid clinical growth and suspicious histology it has to be differentiated from malignant entities. Given its low frequency, the dermatoscopic characteristics of this entity are not well-characterized and have been described only once previously. We present a middle-aged woman with a facial erythematous plaque of 6 months' evolution, with dermatoscopy in which follicular plugs on an erythematous base were appreciated. The histology showed a dense lymphocytic infiltrate with folliculotropism and follicular alteration, with numerous peripheral histiocytes positive for S100 and CD1a. The lesion partially disappeared after the biopsy, and completely after topical treatment.

Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma of mature cytotoxic T cells. Initially, patients with SPTCL were treated with doxorubicin-based polychemotherapy. OBJECTIVE: To analyze clinical, biologic, immunophenotypical, molecular, imaging, treatment, and outcome data reflecting the current state of knowledge. METHODS: A retrospective multicenter study of 16 patients with SPTCL that was diagnosed between 1996 and 2016. RESULTS: The female-to-male ratio was 1.7. The median age at diagnosis was 46.5 years. Patients presented with multiple nodular or plaque-like lesions preferentially affecting the legs and/or trunk. Histopathology typically showed a lobular panniculitis with individual adipocytes surrounded by atypical lymphocytes, usually with a CD3+, CD4-, CD8+, CD56-, TIA1 cytotoxic granule associated RNA binding protein 1-positive phenotype and high proliferation rate. SPTCL was associated with autoimmune diseases in 25% of patients, and with the development of hemophagocytic syndrome in 18% of patients. Oral steroids alone or in combination with low-dose methotrexate or cyclosporine A were the most common initial treatment, achieving a complete response in 85% of the treated patients. The median follow-up time was 14 months. The 5-year disease-specific survival rate was 85.7%. LIMITATIONS: This was a retrospective study. CONCLUSIONS: SPTCL has an excellent prognosis. Immunosuppressive agents can be considered for first-line treatment.

Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type, With Spontaneous Regression After Biopsy.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) represents approximately 20% of cutaneous B lymphomas with an intermediate prognosis. Spontaneous regression is uncommon; there are only 2 published cases. An 83-year-old woman presented 2 orange erythematous nodules on the back of her right leg with an elastic consistency, infiltrated, painful to the touch, and of an 8-month evolution. A histological examination revealed a dense cellular dermo-hypodermic infiltrate sparing the papillary dermis, composed of large cells with immunoblast and centroblast morphology and frequent mitosis. Immunohistochemical studies showed positivity for CD20, CD79, Bcl2, Bcl6, MUM1, Fox-P1, and IgM with Ki67 >95%. Rearrangement of heavy IgH chains was monoclonal. The extension study was negative, establishing a diagnosis of PCDLBCL-LT, T2aN0M0. Three months after biopsy, the patient's lesions regressed spontaneously. New biopsies were taken that revealed a mild diffused dermo-hypodermic cellular infiltrate compounded by small-sized T lymphocytes, with predominance of CD8. Despite its self-limited character, treatment with radiotherapy was done, remaining asymptomatic after 1 year follow-up. There are 2 published cases of PCDLBCL-LT with spontaneous regression. The cause of this unusual autoinvolutional phenomenon is unknown; it may be an immune response against tumor cells through a traumatic or infectious mechanism.

Does the new staging system proposed for mycosis fungoides and Sézary syndrome provide reliable agreement for T1 and T2 disease?

BACKGROUND: Patch or plaque stages in mycosis fungoides (MF) have different prognoses. The recent staging system proposed for MF discriminates between patches and plaques based upon clinical features. OBJECTIVE: To estimate interdermatologist agreement on the morphological evaluation of MF lesions. METHODS: Twenty-four patients with MF were enrolled. Two dermatologists evaluated every lesion face to face and independently with respect to the patch-plaque status. Cohen's κ was used to determine the rate of agreement. RESULTS: Agreement was 67% with respect to the patch or plaque status [95% confidence interval (CI) = 49-85%; p < 0.001]. Current systemic treatment (56%; p = 0.01) was associated with lower agreement. Younger age at diagnosis [odds ratio (OR) 1.03 (95% CI 1.02-1.05)], younger age at enrolment [OR 1.03 (95% CI 1.02-1.04)] and time on systemic treatment [OR 1.02 (95% CI 1.01-1.04)] were independent risk factors for disagreement (p < 0.001). CONCLUSION: The new system for MF staging carries a significant risk of disagreement regarding patch and plaque subsets.

Pediatric dermatology consultations: a retrospective analysis of inpatient consultations referred to the dermatology service.

To analyze the care provided by consultant dermatologists to hospitalized pediatric patients, we retrospectively studied the records of inpatient pediatric consultation requests received by a hospital dermatology department between 2000 and 2009. The diagnoses were recorded according to the International Classification of Diseases, Ninth Revision (ICD-9). In a high percentage of cases, the problem was addressed in a single visit, and few additional tests were ordered. The most common diagnostic groups were diseases of the skin and subcutaneous tissue (ICD-9: 680-709). The most frequent specific diagnosis was atopic dermatitis (ICD-9: 691.8). Our series shows that the role of dermatologists as consultants for pediatric inpatients is similar to their role in outpatient consultations.

Subcutaneous panniculitis-like T-cell lymphoma, lupus erythematosus profundus, and overlapping cases: molecular characterization through the study of 208 genes.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic cutaneous lymphoma. Differential diagnosis with lupus erythematosus panniculitis (LEP) can be challenging and overlapping cases have been described. In this study, we investigate whether gene expression profiling may or not identify markers that can be used to improve our understanding of the disease and to make a precise differential diagnosis. SPTCL, LEP, and overlapping cases were analyzed using a customized NanoString platform including 208 genes related to T-cell differentiation, stromal signatures, oncogenes, and tumor suppressor genes. Gene expression unsupervised analysis of the samples differentiated SPTCL from LEP samples. Most overlapping cases were clustered with LEP cases. Differentially expressed genes were observed when comparing SPTCL with LEP cases; and overlapping with LEP cases. Gene set enrichment analysis recognized gene sets defining each group. In conclusion, SPTCL and LEP have distinctive molecular profiles and the molecular background of overlapping cases more closely resembles LEP.

Dermatologists in hospital wards: an 8-year study of dermatology consultations.

BACKGROUND: The value of dermatologists as consultants is increasing. OBJECTIVE: To evaluate the activity of dermatologists as inpatient consultants. METHODS: Retrospective study of consultations to Dermatology of inpatients, visits per consultation, referral service, procedures performed, delay until visit and diagnoses based on ICD-9. RESULTS: 3,144 requests generated 4,824 visits, 200 biopsies, 107 cultures and other procedures. The mean delay between request and visit was 0.75 days. The requesting service was recorded in 3,097 cases: 21.5% by Internal Medicine, 11.4% by Pediatrics, 8.3% by Neurology and 6.2% by the Infectious Diseases Unit. Follow-up was recommended in 12.4% by the Dermatology Service and in 8% by a dermatologist. Reliable diagnoses were recorded for 2,832 consultations. The most frequent diagnostic groups were diseases of the skin and subcutaneous tissue (ICD-9: 680-709) in 58%, infectious and parasitic diseases (ICD-9: 001-139) in 20%, and neoplasm (ICD-9: 140-239) in 5.7%. The most frequent diagnoses were contact dermatitis (ICD-9: 692.X) in 8.9%, drug reactions (ICD-9: 693.0) in 7.4%, candidiasis (ICD-9:112.X) in 7.1% and seborrheic dermatitis (ICD-9: 690) in 5.3%. CONCLUSIONS: Most diagnoses were of a specific cutaneous disease and the dermatologist would be in the best position to evaluate those that inpatients acquire.

Povidone-iodine induced post-surgical irritant contact dermatitis localized outside of the surgical incision area. Report of 27 cases and a literature review.

BACKGROUND: Povidone-iodine solution is an antiseptic that is used worldwide as surgical paint and is considered to have a low irritant potential. Post-surgical severe irritant dermatitis has been described after the misuse of this antiseptic in the surgical setting. METHODS: Between January 2011 and June 2013, 27 consecutive patients with post-surgical contact dermatitis localized outside of the surgical incision area were evaluated. Thirteen patients were also available for patch testing. RESULTS: All patients developed dermatitis the day after the surgical procedure. Povidone-iodine solution was the only liquid in contact with the skin of our patients. Most typical lesions were distributed in a double lumbar parallel pattern, but they were also found in a random pattern or in areas where a protective pad or an occlusive medical device was glued to the skin. The patch test results with povidone-iodine were negative. CONCLUSIONS: Povidone-iodine-induced post-surgical dermatitis may be a severe complication after prolonged surgical procedures. As stated in the literature and based on the observation that povidone-iodine-induced contact irritant dermatitis occurred in areas of pooling or occlusion, we speculate that povidone-iodine together with occlusion were the causes of the dermatitis epidemic that occurred in our surgical setting. Povidone-iodine dermatitis is a problem that is easily preventable through the implementation of minimal routine changes to adequately dry the solution in contact with the skin.

Treatment of stage Ia and Ib mycosis fungoides with psoralen UVA monotherapy: an observational study in tertiary hospitals in the Canary Islands.

BACKGROUND: Mycosis fungoides (MF) is the most common form of primary cutaneous T cell lymphoma. Psoralen combined with ultraviolet A (PUVA) is a first-line treatment for early-stage disease. OBJECTIVES: This study was conducted to assess the clinical effectiveness of and tolerance to PUVA monotherapy in MF. METHODS: We retrospectively reviewed the files of patients who received PUVA for stage I disease. The study included 31 patients, of whom 32% presented with stage Ia and 67% with stage Ib disease, and 68% presented with patch and 32% with plaque disease. All patients received treatment three times per week. RESULTS: Complete response (CR) was achieved in 71% of patients. The median cumulative dose of UVA at CR was 211.7 J/cm(2) . There was a significant difference in median cumulative dose at CR between patients with plaque and patch disease, respectively, but not between patients with stage Ia and Ib disease. Median disease-free survival (DFS) was 230 weeks. Patients with patch disease achieved longer DFS than those with plaque disease (P = 0.004), although DFS was similar in stage Ia and Ib patients. Of the patients who received maintenance therapy, 58% relapsed. Univariate analysis showed patch disease to be a predictive factor for CR, but no predictors of relapse were identified. A total of 71% of patients developed clinical adverse reactions. CONCLUSIONS: Psoralen with UVA is a safe and effective treatment for early-stage MF. Patch disease responds more favorably than plaque disease and is associated with a longer period of DFS. Maintenance treatment does not appear to reduce recurrence. Current evidence suggests that the proposed revision to the classification of MF, which takes into account the extent and type of disease, more accurately predicts response to PUVA.

Contact urticaria due to phenoxyethanol in an aftershave.

The occurrence of pruritus immediately after application of an aftershave product is usually due to irritant contact dermatitis. We report a case of contact urticaria in a male patient, produced by an aftershave product containing phenoxyethanol.

[Neonatal lupus erythematosus: 4 cases and clinical review]. / Lupus eritematoso neonatal: 4 casos y revisión clínica.

Neonatal lupus erythematosus (NLE) is an infrequent disease in newborns caused by the transplacental passage of maternal Anti-Ro/SSA, Anti-La/SSB and/or Anti-U1 RNP antibodies. The most common manifestations are cutaneous and cardiac. We carried out a retrospective study of cases of NLE diagnosed in the last 10 years at the Hospital Universitario Insular in Gran Canaria. Complete data was obtained for 4 patients. Three cases had circulating Anti-Ro antibodies in the mother and in the newborns, while in the fourth case they were Anti-RNP. Two mothers were diagnosed with systemic lupus, one with mixed connective tissue disease and the other with leucocytoclastic vasculitis. The skin lesions consisted of urticaria-like and desquamative lesions. One patient presented with ulceration. The histological study of the urticaria-like lesions showed a non-specific perivascular infiltrate; the desquamative lesions were consistent with subacute lupus erythematosus.