Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren's Syndrome.

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare condition attributed to plasma cell proliferation and the deposition of immunoglobulin light chains in the skin without association with systemic amyloidosis or hematological dyscrasias. It is not uncommon for patients diagnosed with PLCNA to also suffer from other auto-immune connective tissue diseases, with Sjögren's syndrome (SjS) showing the strongest association. This article provides a literature review and descriptive analysis to better understand the unique relationship between these two entities. To date, 34 patients with PLCNA and SjS have been reported in a total of 26 articles. The co-existence of PLCNA and SjS has been reported, especially in female patients in their seventh decade of life with nodular lesions on the trunk and/or lower extremities. Acral and facial localization, which is a typical localization of PLCNA in the absence of SjS, seems to be much more unusual in patients with associated SjS.

Localized Cutaneous Nodular Amyloidosis in a Patient with Sjögren's Syndrome.

Primary localized cutaneous nodular amyloidosis (PLCNA) is included in the primary forms of cutaneous amyloidosis along with macular and lichenoid amyloidosis. It is a rare disease attributed to plasma cell proliferation and deposition of immunoglobulin light chains in the skin. We present the case of a 75-year-old woman with a personal history of Sjogren's syndrome (SjS), who consulted for asymptomatic yellowish, waxy nodules on the left leg. Dermoscopy of the lesions showed a smooth, structureless, yellowish surface with hemorrhagic areas and few telangiectatic vessels. Histopathology revealed an atrophic epidermis and deposits of amorphous eosinophilic material in the dermis with a positive Congo red stain. The diagnosis of nodular amyloidosis was made. Periodic reevaluation was indicated after the exclusion of systemic amyloidosis. PLCNA is often associated with autoimmune connective tissue diseases, and up to 25% of all PLCNA cases occur in patients with SjS. Therefore, in addition to ruling out systemic amyloidosis, screening for possible underlying SjS should be performed when the diagnosis of PLCNA is confirmed.

Monkeypox outbreak in Spain: clinical and epidemiological findings in a prospective cross-sectional study of 185 cases.

BACKGROUND: Since May 2022, a new outbreak of monkeypox has been reported in several countries, including Spain. The clinical and epidemiological characteristics of the cases in this outbreak may differ from those in earlier reports. OBJECTIVES: To document the clinical and epidemiological characteristics of cases of monkeypox in the current outbreak. METHODS: We conducted a prospective cross-sectional study in multiple medical facilities in Spain to describe the cases of monkeypox in the 2022 outbreak. RESULTS: In total, 185 patients were included. Most cases started with primarily localized homogeneous papules, not pustules, in the probable area of inoculation, which could be cutaneous or mucous, including single lesions. Generalized small pustules appeared later in some of them. Heterogeneous lesions occurred during this generalized phase. All patients had systemic symptoms. Less common lesions included mucosal ulcers (including pharyngeal ulcers and proctitis) and monkeypox whitlows. Four patients were hospitalized, none died. Smallpox vaccination and well-controlled HIV disease were not associated with markers of severity. Contact during sex is the most likely mechanism of transmission. In this outbreak, cases have been described in men who have sex with men and are strongly associated with high-risk sexual behaviours. Seventy-six per cent of the patients had other sexually transmitted diseases upon screening. CONCLUSIONS: The clinical findings in this outbreak differ from previous findings and highly suggest contact transmission and initiation at the entry site. The characterization of the epidemiology of this outbreak has implications for control. What is already known about this topic? Monkeypox eruption is described as consisting of pustules. The roles of HIV and previous smallpox vaccination in the prognosis are unknown. The transmission route was initially described as respiratory droplets and was later suggested to be via sexual contact. What does this study add? Initial lesions at the probable inoculation area were homogeneous and papular (pseudopustules). Generalized small pustules appeared later in some of them. Heterogeneous lesions occurred during this generalized phase. All patients had systemic symptoms. Less common signs included mucosal ulcers (including pharyngeal ulcers and proctitis) and monkeypox whitlows. Well-controlled HIV and previous smallpox vaccination were not associated with severity. No patient died. The data support the hypothesis of transmission via contact during sex. Although this might change, the outbreak is currently limited mostly to men who have sex with men, with high-risk factors for sexually transmitted diseases.

Primary Cutaneous CD4 Small/Medium T-Cell Lymphoproliferative Disorder Following COVID-19 Vaccination-What Do We Know about Lymphoproliferative Disorders and Cutaneous Lymphomas after COVID-19 Vaccination? A Report of an Atypical Case and a Review of the Literature.

The association between Primary cutaneous CD4 small/medium T-cell lymphoproliferative disorder (PCSM-TCLPD) and COVID-19 immunization has been sparsely documented in the medical literature. Reviewing the literature, albeit infrequently, we can find cases of the recurrence and new onset of lymphoproliferative processes and cutaneous lymphomas following the COVID-19 vaccine. Many of the entities we encounter are classified as cutaneous lymphoproliferative disorders. The prevailing hypothesis suggests that the predominant cutaneous reactions to SARS-CoV-2 vaccines may stem from T-cell-mediated immune activation responses to vaccine components, notably messenger RNA (mRNA). Specifically, it is posited that the presence of cutaneous lymphoid infiltrates may be linked to immune system stimulation, supported by the absence, to date, of instances of primary cutaneous B-cell lymphoma following mRNA vaccination. Within this context, it is imperative to underscore that the etiological association between PCSM-TCLPD and COVID-19 vaccination should not discourage vaccination efforts. Instead, it underscores the necessity for continuous surveillance, in-depth investigation, and comprehensive follow-up studies to delineate the specific attributes and underlying mechanisms of such cutaneous manifestations post vaccination.

Efficacy and Satisfaction of Low Doses UVA1 Phototherapy: A Spanish Experience from a Single Centre.

BACKGROUND: UVA1 phototherapy is a treatment used for multiple dermatological conditions. The optimal therapeutic regimens and dosing of UVA1 are a matter of debate. The dosages used vary widely between published studies and there are no evidence-based protocols that provide data on dosage, duration, or the role of maintenance therapy. The purpose of this study is to evaluate the experience in our medical center regarding treatment with UVA1, as well as the degree of patient satisfaction with the treatment according to their pathology. METHODS: We present a retrospective evaluation of outcomes, treatment tolerability, and satisfaction in adult patients using a low dose of UVA1 phototherapy, administered in our dermatologic service between 2019 and 2022. RESULTS: A total of 78 patients were treated with UVA1, of whom 46 patients (59%) were over 18 years old, completed treatment, and gave their consent. The overall objective clinical response rate was 91.30% (42/46), achieving a complete response in 17 (36.96%) patients, partial response in 25 (54.34%), and no response in 4 (8.70%). The complete response rates recorded were high in morphea, scleredema, or chronic hand eczema. In terms of the level of satisfaction objectively measured by TSQM-9 version 1.4, highlighting high scores obtained in mastocytosis, systemic sclerosis, morphea, scleredema, chronic hand eczema, or prurigo nodularis (over 65 points). CONCLUSIONS: We present a review of treatment with UVA1 phototherapy at low doses with good response in a wide variety of dermatological pathologies.

Dimethyl Fumarate as Therapeutic Alternative in Moderate-to-Severe Psoriasis: Our Experience.

Purpose: Dimethyl fumarate (DMF) is an oral formulation approved for the treatment of moderate-to-severe psoriasis in adult patients requiring systemic therapy. Here, we describe our clinical experience with DMF for moderate-to-severe psoriasis in Spain. Patients and Methods: This is a retrospective study including 30 adult patients with moderate-to-severe psoriasis under treatment with DMF between September 2018 and January 2020. Patients were treated with DMF as per its Summary of Product Characteristics and the median duration of treatment was 15 weeks (4-55 weeks). Psoriasis Area and Severity Index (PASI) and body surface area (BSA) severity scales were evaluated from baseline to week 36 and adverse events (AEs) developed during treatment were described. Results: The efficacy of DMF was assessed at week 8 and at week 36 (n = 5), both PASI and BSA were 0. At week 24, median PASI showed a decrease in both the last observation carried forward (LOCF; n = 23) and the observed cases (OC) (n = 10): from 10 to 6 and from 10 to 1.5, respectively. Median BSA also showed a decrease from 19 to 10 in LOCF and from 17 to 3 in OC. The most frequent AEs were diarrhoea (40.0%), flushing (13.3%) and lymphopenia (3.3%). In 47.1% patients, AEs have been solved by adjusting the DMF dose. Treatment discontinuation rate due to AEs was 43.3%. Conclusion: Our clinical experience indicates that DMF could be an effective and safe treatment for moderate-to-severe psoriasis in adult patients.

Acral lesions in a pediatric population during the COVID-19 pandemic: a case series of 36 patients from a single hospital in Spain.

BACKGROUND: To describe the cutaneous acral findings in a pediatric population noticed during this pandemic. METHODS: A retrospective descriptive study was performed collecting data on 36 patients under 14 years old, presenting suspicious acral skin manifestations for coronavirus disease 2019 (COVID-19). RESULTS: Patients were mostly male (63.8%). The mean age was 11.11 years. 66.67% of patients showed erythematous papules, and 44.44% purpuric macules. Feet were affected in 97.22% of patients and hands in 5.55%. Lesions were asymptomatic in 50% of patients. 30.55% of patients showed extracutaneous findings, preceding skin lesions in 12.62 days. Seven patients underwent specific severe acute coronavirus 2 (SARS-CoV-2) testing; all of these patients tested negative. CONCLUSIONS: The association between these symptoms and SARS-CoV-2 remains unclear. We recommend using these manifestations as a sign of SARS-CoV-2 infection in children. This could lead to the examination of asymptomatic and mildly symptomatic children so that contagions may be avoided.