A Descriptive Study of 103 Primary Cutaneous B-Cell Lymphomas: Clinical and Pathological Characteristics and Treatment from the Spanish Lymphoma Oncology Group (GOTEL).

Primary cutaneous B-cell lymphomas (PCBCLs) are B-cell lymphomas that can occur in the skin without evidence of extracutaneous involvement. The 2005 WHO/EORTC classification of cutaneous lymphomas and its 2018 update have distinguished three main categories based on clinicopathological, immunohistochemical, and genetic characteristics: primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle centre lymphoma (PCFCL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT). PCMZL and PCFCL are clinically indolent, while PCDLBCL-LT is an aggressive lymphoma. Due to its low incidence and lack of prospective studies, it is difficult to establish a standard treatment for each subgroup. The objective of our study was to describe the clinical and pathological characteristics of 103 patients with cutaneous B-cell lymphoma from 12 centres belonging to the Spanish Lymphoma Oncology Group. The median age was 53 years (40-65). According to skin extension, 62% had single-site lymphoma, 17% had regional lymphoma, and 20% had multifocal lymphoma. Histology: 66% had PCMZL, 26% had PCFCL, and 8% had PCDLBCL-LT. Twenty-three percent of the patients were treated exclusively with surgery, 26% with radiotherapy only, 21% with surgery plus radiotherapy, 10% with polychemotherapy, and 5% with rituximab monotherapy. Overall, 96% of patients achieved a complete response, and 44% subsequently relapsed, most of them relapsing either locally or regionally. The 10-year OS was 94.5% for the entire cohort, 98% for the PCMZL cohort, 95% for the PCFCL cohort, and 85.7% for the PCDLBCL-LT cohort. Our data are comparable to those of other published series, except for the high frequency of PCMZL. The expected heterogeneity in therapeutic management has been observed.

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.

Erythema multiforme to amoxicillin with concurrent infection by Epstein-Barr virus.

BACKGROUND: The incidence of rashes following the intake of aminopenicillins during an acute episode of infectious mononucleosis is high, but severe cutaneous reactions as erythema multiforme or Stevens-Johnson syndrome are rare manifestations in childhood. MATERIAL AND METHODS: We report the case of a 7 year old girl that developed a generalized purpuric rash with target shaped areas, 9 days after starting treatment with amoxicillin-clavulanic acid. Laboratory investigation revealed a significant increase of Epstein Barr virus (EBV) specific IgM antibody. After skin biopse she was diagnosed as erythema multiforme syndrome. Prick, intradermal and patch tests were performed with penicilloylpolylysine, minor determinant mixture, benzylpenicillin, ampicillin, amoxicillin, cefazoline and cefotaxime, the 24 hours reading was positive for aminopenicillins. Patch tests were also positive only for aminopenicillins, other betalactams were negative. CONCLUSIONS: The interaction between an infectious agent (EBV) and amoxicillin could precipitate the severe skin reaction. Patch test and delayed intradermal reading with amoxicilllin were an useful tool for the diagnosis of the etiological agent in this reaction. The negative response to other beta-lactams, suggests that the aminobenzyl group of the side chain of amoxicillin plays a predominant role in this reaction.

[Amyopathic dermatomyositis associated with a recurrence of breast cancer]. / Dermatomiositis amiopática asociada a recurrencia de cáncer de mama.

The term "amyopathic dermatomyositis", or dermatomyositis "sine myositis" is used to describe those patients who present with the skin manifestations typical of dermatomyositis, but with no evidence of inflammatory myopathy. Amyopathic dermatomyositis may be associated with an underlying neoplasm, the same as with classic dermatomyositis. We present the case of a 59-year-old female patient, with cutaneous findings typical of dermatomyositis, with no proximal muscle weakness and with normal serum muscle enzymes, which stayed in a normal range throughout the later follow-up period, although the electromyogram performed six months later showed alterations with a myopathic pattern. These skin symptoms raised the suspicion of an occult neoplasm, and a recurrence of the patient's breast cancer, apparently inactive for many years, was finally found. The association of amyopathic dermatomyositis with a recurrence of breast cancer is exceptional.

Skin tumors in patients aged 90 years and older.

BACKGROUND: The aging of the population in the developed world is an accepted fact. Consequently, the number of patients aged 90 years or over with cutaneous cancer will increase in coming years. OBJECTIVE: The objective was to determine the nature of skin lesions amenable to dermatologic surgery in patients aged 90 years or more. METHODS: We studied the clinical histories of 52 outpatients in their 90 s, who had had histopathologic studies made of their skin tumors when attended in the Hospital General de Alicante (Spain) between January 1999 and July 2002. We studied sex, age, type of tumor, site, associated disorders, regular medications, type of anesthesia, and type of treatment given. RESULTS: The average age of the patients was 92.4 years. Thirty-six patients were women and 16 were men. Altogether the 52 patients had a total of 72 lesions. The most frequent diagnosis was basal cell carcinoma with 36 lesions, followed by 20 squamous cell carcinomas. The overall ratio of basal cell carcinoma to squamous cell carcinoma was 1.8. Patients had an average of 1.5 comorbid medical conditions and were taking an average of 2.3 regular medications. CONCLUSION: Dermatologists often attend patients aged 90 years or over with nonmelanoma skin cancer. The most important decision is as to what is the best management of these patients.

Subungal abcesses secondary to paclitaxel.

Various cutaneous side effects, including nail changes, have been associated with taxane chemotherapeutic agents, but usually docetaxel has been implicated. We report a patient with acute paronychia due to paclitaxel administered for treatment of breast cancer.

Interstitial granulomatous dermatitis with plaques and arthritis.

Interstitial granulomatous dermatitis (IGD) is a histopathological disorder characterised by an infiltration of the reticular dermis with a predominance of interstitial and palisadic histiocytes with a few areas of degenerating collagen bundles associated with a variable number of polynuclear neutrophils and eosinophils. There are several clinical conditions with a pattern of IGD. The linear form associated with arthritis was the first variety described. There is also a second form, which presents with plaques. This variety may be associated with arthritis, use of certain drugs or the presence of different systemic disorders. We report a case of IGD with plaques and arthritis. We discuss the differential clinical and histological diagnosis with other inflammatory skin lesions, which may be associated with joint disorders and collagen degeneration. We believe that it should be considered in patients presenting with arthritis and skin lesions.