Requirements for Accessing New Dermatology Drugs in Spain: Results of the EQUIDAD Study. / Condicionantes de acceso a nuevos medicamentos dermatológicos en España: resultados del proyecto EQUIDAD.

BACKGROUND: Although the Spanish Ministry of Health prepares national therapeutic positioning reports (TPRs) and drug reimbursement policies, each of the country's 17 autonomous communities (ACs) is responsible for health care services and prescription requirements in its territory. The aim of the EQUIDAD study was to describe and explore potential differences in prescription requirements for new dermatology drugs across the autonomous communities. MATERIAL AND METHODS: Cross-sectional study conducted in April and May, 2023. Two dermatologists with management responsibilities from each autonomous community reported on territorial and more local prescription requirements for drugs covered by national TPRs issued between 2016 and 2022. RESULTS: Thirty-three researchers from 17 autonomous communities participated. The data submitted revealed between-community inequities in access to new drugs. Overall, 64.7% of the regions imposed additional prescription requirements to those mentioned in the TPRs for psoriasis. This percentage was lower for atopic dermatitis (35.3%) and melanoma (11.8%). The most common requirement for accessing a new drug was a previous prescription for another drug. Differences and additional requirements were also detected at the local level (i.e., differences between hospitals within the same autonomous community). CONCLUSIONS: Spain's autonomous communities have multiple regional and local prescription requirements that are not aligned with national TPR recommendations. These differences result in inequitable access to new drugs for both patients and practitioners across Spain.

[Translated article] Requirements for Accessing New Dermatology Drugs in Spain: Results of the EQUIDAD Study.

BACKGROUND: Although the Spanish Ministry of Health prepares national therapeutic positioning reports (TPRs) and drug reimbursement policies, each of the country's 17 autonomous communities (ACs) is responsible for health care services and prescription requirements in its territory. The aim of the EQUIDAD study was to describe and explore potential differences in prescription requirements for new dermatology drugs across the autonomous communities. MATERIAL AND METHODS: Cross-sectional study conducted in April and May, 2023. Two dermatologists with management responsibilities from each autonomous community reported on territorial and more local prescription requirements for drugs covered by national TPRs issued between 2016 and 2022. RESULTS: Thirty-three researchers from 17 autonomous communities participated. The data submitted revealed between-community inequities in access to new drugs. Overall, 64.7% of the regions imposed additional prescription requirements to those mentioned in the TPRs for psoriasis. This percentage was lower for atopic dermatitis (35.3%) and melanoma (11.8%). The most common requirement for accessing a new drug was a previous prescription for another drug. Differences and additional requirements were also detected at the local level (i.e., differences between hospitals within the same autonomous community). CONCLUSIONS: Spain's autonomous communities have multiple regional and local prescription requirements that are not aligned with national TPR recommendations. These differences result in inequitable access to new drugs for both patients and practitioners across Spain.

Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years. / Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.

Expert Recommendations on Treating Psoriasis in Special Circumstances (Part II). / Recomendaciones de expertos para el tratamiento de la psoriasis en situaciones especiales (II).

BACKGROUND: There is insufficient information on how best to treat moderate to severe psoriasis in difficult clinical circumstances. MATERIAL AND METHODS: We considered 5 areas where there is conflicting or insufficient evidence: pediatric psoriasis, risk of infection in patients being treated with biologics, psoriasis in difficult locations, biologic drug survival, and impact of disease on quality of life. Following discussion of the issues by an expert panel of dermatologists specialized in the management of psoriasis, participants answered a questionnaire survey according to the Delphi method. RESULTS: Consensus was reached on 66 (70.9%) of the 93 items analyzed; the experts agreed with 49 statements and disagreed with 17. It was agreed that body mass index, metabolic comorbidities, and quality of life should be monitored in children with psoriasis. The experts also agreed that the most appropriate systemic treatment for this age group was methotrexate, while the most appropriate biologic treatment was etanercept. Although it was recognized that the available evidence was inconsistent and difficult to extrapolate, the panel agreed that biologic drug survival could be increased by flexible, individualized dosing regimens, continuous treatment, and combination therapies. Finally, consensus was reached on using the Dermatology Quality of Life Index to assess treatment effectiveness and aid decision-making in clinical practice. CONCLUSIONS: The structured opinion of experts guides decision-making regarding aspects of clinical practice for which there is incomplete or conflicting information.

Paradoxical reaction to antituberculosis therapy in a patient with lupus vulgaris.

Patients receiving treatment for tuberculosis may experience an unexpected deterioration of their disease; this is known as a paradoxical reaction. We present the case of a 59-year-old man with lupus vulgaris who experienced a paradoxical deterioration of cutaneous lesions after starting antituberculosis therapy. The reaction was self-limiting; the lesions gradually improved, and the final outcome was very good. Paradoxical reactions are well-known in patients with human immunodeficiency virus (HIV) infection who start antiretroviral therapy, but they can also occur in non-HIV-infected patients with tuberculosis who start antituberculosis therapy. In the literature reviewed, paradoxical reactions involving skin lesions were described in patients with miliary tuberculosis. The case we report is the first of a paradoxical reaction in lupus vulgaris. The increasing frequency of tuberculosis in Spain could lead to a rise in the number of paradoxical reactions.

The First Year of the AEVD Primary Cutaneous Lymphoma Registry. / Registro de linfomas cutáneos primarios de la AEDV: primer año de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas are uncommon. This article describes the Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV) and reports on the results from the first year. PATIENTS AND METHODS: Disease registry for patients with primary cutaneous lymphoma. The participating hospitals prospectively recorded data on diagnosis, treatment, tests, and disease stage for all patients with primary cutaneous lymphoma. A descriptive analysis was performed. RESULTS: In December 2017, the registry contained data on 639 patients (60% male) from 16 university hospitals. The most common diagnoses, in order of frequency, were mycosis fungoides/Sézary syndrome (MF/SS) (348 cases, 55%), primary cutaneous B-cell lymphoma (CBCL) (184 cases, 29%), primary cutaneous CD30+ T-cell lymphoproliferative disorder (CD30+ CLPD) (70 cases, 11%), and other types of T-cell lymphoma (37 cases, 5%). In total, 105 (16.5%) of the cases recorded were incident cases. The most common diagnosis in the MF/SS group was classic MF (77.3%). Half of the patients with MF had stage IA disease when diagnosed, and the majority were either in partial remission (32.5%) or had stable disease (33.1%). The most widely used treatments were topical corticosteroids (90.8%) and phototherapy. The most common form of primary CBCL was marginal zone lymphoma (50%). Almost all of the patients had cutaneous involvement only and nearly half had stage T1a disease. Most (76.1%) were in complete remission. The main treatments were surgery (55.4%) and radiotherapy (41.9%). The most common diagnosis in patients with CD30+ CLPD was lymphomatoid papulosis (68.8%). Most of the patients (31.4%) had stage T3b disease and half were in complete remission. The most common treatments were topical corticosteroids (68.8%) and systemic chemotherapy (32.9%). CONCLUSION: The characteristics of patients with primary cutaneous lymphoma in Spain do not differ from those described in other series in the literature. The registry will facilitate clinical research by the AEDV's lymphoma group.

[Multiple clustered histiocytofibroma. Apropos of a case with immunohistochemical study]. / Histiocytofibromes multiples groupés. A propos d'un cas avec étude immunohistochimique.

Multiple clustered dermatofibromas constitute an uncommon entity, of which we have collected seven cases in the literature. We observed the case of a 4-year-old girl, who had dermatofibromas confluent in a central plaque of fibrous aspect, located in the lumbar region. The immunohistochemical study showed positive staining for Factor XIIIa antibody, confirming its origin in the dermal dendrocyte.

Pachydermodactyly: report of two cases.

Pachydermodactyly is a rare and benign form of digital fibromatosis with only a few cases described in the literature. We report two cases of pachydermodactyly affecting young men. In one of them a knuckle pad was found, and the fibromatous thickening of the skin affected not only the finger joints but also the dorsum of the hands. This case can be included in the particular clinical form named pachydermodactyly transgrediens.

Complete spontaneous regression of Merkel cell carcinoma: a review of the 10 reported cases.

BACKGROUND: Merkel cell (neuroendocrine) carcinoma (MCC) is a very aggressive primary cutaneous neoplasm occurring most often on the head and neck of the elderly. Complete spontaneous regression (CSR) of MCC was first described in 1986. Since then other cases have been reported bringing the total to 10. OBJECTIVE: To review these 10 cases and obtain long-term follow-up data, to compare them for similarities and differences. METHOD: Each original case report was extensively reviewed and authors contacted in most cases for confirmation and updated information. RESULTS: In no case did MCC recur after CSR was noted, although follow-up information in some cases was short. When CSR occurred, it was swift and dramatic with complete regression of skin and lymph node metastasis in 1-3 months. CONCLUSION: While only 10 cases of CSR is a small number, MCC is itself a rare malignancy with just over 600 reported cases. Today most cases of MCC receive aggressive combined therapy effectively precluding diagnosis of CSR. The nature of regression in these 10 cases may point toward future immunologic therapy just as similar cases of CRS in patients with melanoma have led to advances in the immunologic treatment for that malignancy.

Cutaneous leiomyosarcoma in a child.

Superficial leiomyosarcomas are infrequent tumors that are very rare in childhood. We report on a 12-year-old white boy with a cutaneous leiomyosarcoma of his left groin. Immunohistochemical study revealed positive immunostaining with antibodies to vimentin, desmin, and smooth muscle actin. The tumor was removed with wide surgical margins.

Allergic contact dermatitis from naftifine in a child without cross-reaction to terbinafine.

Allergic contact dermatitis from naftifine has been scarcely described in the English literature, all of them in adults. We report a case of a 12-year-old girl who developed an acute eczema on her neck after application of a naftifine cream. This fact was confirmed by a patch-test study. We did not find a cross-reaction to terbinafine, a structurally linked allylamine.

Sclerosing sweat duct carcinoma mimicking dermatofibroma.

Sclerosing sweat duct carcinoma is an infrequent adnexal tumor, locally aggressive and with a high incidence of local recurrences. Its location is preferably cephalic and its clinical presentation often unspecific. We present a new case with an atypical location and the clinical appearance of a benign cutaneous lesion. Diagnosis was established after a histological and immunohistochemical study. A terminological review was done.

Spontaneous regression of Merkel cell carcinoma of the skin.

We report a 65-year-old man who presented with a Merkel cell carcinoma on his right ear, a small satellite nodule and regional lymph node metastases. No treatment was given because of his generally poor state of health. Spontaneous regression of all the lesions was observed within a month. Merkel cell carcinoma is a potentially aggressive tumour. Only four cases of spontaneous regression have been reported previously.