Sweet Syndrome as a Herpetiform Mimic: A Diagnostic Challenge.

ABSTRACT: The authors present a singular case of Sweet syndrome (acute febrile neutrophilic dermatosis) manifesting with an unusual herpetiform clinical presentation, underscoring the imperative for its inclusion in differential diagnoses of herpetic infections. A 26-year-old female patient with a systemic lupus erythematosus history presented with facial edema, hyperthermia, cephalalgia, and polyarticular pain. Dermatological examination revealed clustered, vesicle-like papules on erythematous, edematous skin, mimicking herpetic infection. Elevated acute-phase reactants and urine anomalies were noted. Histopathology confirmed Sweet syndrome, characterized by superficial and deep neutrophilic dermatitis, karyorrhexis, and papillary dermal edema. The patient responded to corticosteroid therapy and a brief antibiotic course, resolving both systemic and cutaneous symptoms. This case is remarkable for its atypical herpetiform presentation, a clinical rarity in Sweet syndrome, challenging the conventional diagnostic process. It emphasizes the necessity of considering Sweet syndrome in differential diagnoses when encountering herpetiform lesions, particularly in patients with autoimmune backgrounds. This case contributes significantly to the understanding of Sweet syndrome's clinical variability and highlights the critical role of thorough clinicopathological evaluation in achieving accurate diagnosis in complex dermatological disorders.

Pseudolymphomatous Cutaneous Angiosarcoma Presenting With Persistent Firm Facial Edema in a Patient With Systemic Sclerosis.

ABSTRACT: Pseudolymphomatous cutaneous angiosarcoma (cAS) is a rare subtype characterized by a prominent lymphocytic infiltrate, posing diagnostic challenges due to its resemblance to lymphoid neoplastic processes. We present a novel case highlighting the clinical and histopathological features, notably its association with persistent firm facial edema in a patient with systemic sclerosis (SSc). A 47-year-old woman with a 21-year history of SSc presented with firm palpebral edema evolving to involve the entire face and cervical region over six months. Diagnostic imaging revealed inflammatory changes in orbital regions, supradiaphragmatic lymphadenopathies, and lytic lesions. Skin biopsy demonstrated a diffuse neoplasm with vascular channels and solid areas, accompanied by dense lymphocytic proliferation. Pseudolymphomatous cutaneous angiosarcoma, a rare malignant neoplasm, exhibits variable clinical presentations and rapid progression. Histologically, it manifests as irregularly shaped vascular channels lined by prominent endothelial cells. Immunohistochemistry, particularly markers such as v-ets erythroblastosis virus E26 oncogene homolog (avian) (ERG), aids in diagnosis. Notably, this case marks the first presentation of cAS with persistent facial edema in SSc, highlighting the association between SSc and cancer risk. This case underscores the diagnostic challenges posed by cAS and emphasizes the importance of early detection for optimal patient outcomes. Further understanding of its association with autoimmune disorders such as SSc is crucial for comprehensive management strategies.

Granulomatous Panniculitis as an Initial Manifestation of Sjögren Syndrome.

ABSTRACT: A 65-year-old woman presented with unexplained weight loss, recurrent fever, and a dermatosis with painful nodules on the extremities. Biopsies showed focal lobular panniculitis with neutrophilic microgranulomas. Comprehensive investigations ruled out infection and hematologic and solid organ neoplasms. Laboratory results showed anti-Ro/SSA and anti-La/SSB antibody positivity, and elevated inflammatory markers. Dry mouth and eye were confirmed. The diagnosis of Sjögren syndrome with cutaneous panniculitis was established. Prednisone treatment with 30 mg/d resulted in remission of fever and pain improvement. This case emphasizes Sjögren syndrome as an autoimmune disease with multiple cutaneous manifestations and highlights its association with granulomatous panniculitis.

Extracellular Vesicles from Human Plasma Show a Distinctive Proteome and miRNome Profile in Patients with Severe Cutaneous Adverse Reactions.

Cutaneous drug-induced reactions are immune-mediated responses that can lead to life-threatening diseases such as drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome, and toxic epidermal necrolysis, collectively known as severe cutaneous adverse reactions (SCARs). Unfortunately, they cannot be predicted during drug development, and, at present, a prognostic biomarker is not available nor are validated in vitro assays for diagnosis. Thus, by using proteomic and microarray miRNA analysis, the cargo of extracellular vesicles obtained from SCARs patients was analyzed and correlated with the severity of the reaction. Confirmatory assays using Western blot and qRT-PCR were performed to validate findings, and bioinformatic tools were used to establish the correlation between protein and miRNAs expression between groups. The proteomic analysis showed an increase in the amount of pro-inflammatory proteins, von Willebrand factor, and C-reactive protein and a decrease in anti-inflammatory and protective proteins in the SCARs group compared with the control group. Additionally, histone protein H2A was enriched in DRESS patients. APO1 and SERPINA4 proteins, highly increased in the control group but absent in the SCARs group, are the target of several overexpressed miRNAs, suggesting that the regulation of these proteins might involve gene silencing and protein repressing mechanisms in the severe patients. According with previous reports showing its presence in plasma and T-cells, microRNA miR-18 was upregulated in extracellular vesicles obtained from the most severe patients. Determination of the unique cargo associated with different disease conditions will help to understand the pathophysiology of these complex reactions and might help to develop novel biomarkers for life-threatening iatrogenic cutaneous disease.

Toenail abnormalities in rheumatoid arthritis patients are associated with radiographic damage and impact disability: a cross sectional study nested within a cohort.

OBJECTIVES: Cutaneous involvement is an extra-articular manifestation of rheumatoid arthritis (RA). This includes nail abnormalities, which are often overlooked. We described nail findings in RA patients currently attending an early arthritis cohort (n=145), and associated them with disease activity and/or damage, as well as patient-reported outcomes. METHODS: A standardised nail examination was performed in 122 patients (84.1% of the cohort), concomitant to the rheumatic assessment. Disability, quality of life and perceived nail-related health were also assessed. Nail findings and their location were recorded and classified according to standardised definitions. Logistic and linear regression models were used to investigate predictors of nail findings and to identify the impact of toenail findings on disability, which was evaluated with the HAQ. Patients consented to participate. RESULTS: Patients were primarily middle-aged females, with median follow-up of 9 years, and had disease under control. Most patients (62.3%) had at least one nail finding and these patients scored lower their nail-related health. The median (IQR) of findings/abnormalities per patient was 3 (2-5) and the number of nails affected per patient was 10 (2-12). Age (OR: 1.04, 95%CI: 1.007-1.074) and erosive disease (OR: 2.26, 95%CI: 1.1-5.1) were associated with nail findings. Toenail involvement was consistently associated with HAQ score out of normal range (OR=3.4, 95%CI=1.24-9.35, p=0.02). There was a linear association between the number of toenails affected and the HAQ score. CONCLUSIONS: Nail abnormalities are common and heterogeneous findings in RA patients; they are associated with erosive damage and impact disability.

Biosimilar medicines for psoriasis. Positioning of the Mexican Academy of Dermatology.

The development and marketing of biosimilars opens a new scenario in the treatment of many pathologies, including psoriasis. This article reflects the position of the Mexican Academy of Dermatology (AMD) on the use of biosimilar medicines for the treatment of psoriasis in Mexico. In summary, the AMD estates that there is sufficient evidence to accept comparability of pharmacokinetics and pharmacodynamics of some biosimilar medicines to adalimumab, infliximab and etanercept; this evidence does not sufficiently support interchangeability or indication extrapolation. It is essential to establish a close pharmacovigilance not only to guarantee compliance with the Cofepris rules in Mexico, but also to facilitate the effective monitoring of the adverse effects of biosimilar medicines. Although the goal of biotechnological drugs is to achieve substantial savings for patients and public institutions, no economic criteria should prevail over rigorous scientific criteria that guarantee maximum therapeutic efficacy and optimum safety for patients.

El desarrollo y comercialización de biocomparables abre un nuevo escenario en el tratamiento de muchas patologías, entre ellas la psoriasis. El presente artículo recoge la postura de la Academia Mexicana de Dermatología (AMD) respecto al uso de medicamentos biocomparables para el tratamiento de la psoriasis en México. En resumen, la AMD establece que existe suficiente evidencia para aceptar la comparabilidad farmacocinética y farmacodinámica entre algunos medicamentos biocomparables al adalimumab, el infliximab y el etanercept; esta evidencia no sustenta suficientemente su intercambiabilidad ni la extrapolación de indicaciones; es fundamental establecer una farmacovigilancia estrecha no solo para garantizar el cumplimiento de las reglas de la Comisión Federal para la Protección contra Riesgos Sanitarios en México, sino para facilitar un seguimiento efectivo de los efectos adversos de los medicamentos biocomparables. Si bien la meta de los medicamentos biotecnológicos es lograr un ahorro sustancial para los pacientes y las instituciones públicas, los criterios económicos no deben anteponerse a criterios científicos rigurosos que garanticen la máxima eficacia terapéutica y la óptima seguridad para los pacientes.

Inflammatory chemokine profiles and their correlations with effector CD4 T cell and regulatory cell subpopulations in cutaneous lupus erythematosus.

We compared the chemokine/receptor expression in skin biopsies of discoid (SLE/DLE) and subacute lupus (SLE/SCLE) and correlated it with tissue and circulating effector CD4 T cells/regulatory cells. Skin biopsies and peripheral blood from 9 active SLE/DLE patients, 9 SLE/SCLE patients, 5 control SLE patients without cutaneous lesions and 10 control healthy donors were included. Clinical skin activity was measured by Cutaneous Lupus Erythematosus Disease Area and Severity Index scoring, and systemic activity was measured by a modified SLEDAI-2K excluding the cutaneous items. Pain and pruritus were evaluated by a 10-point visual analogue scale. To determine the frequencies of CXCL-10/CXCR3-, CCL2/CCR2-, CCL17/CCR4-, CCL20/CCR6-, CCL27/CCR10-, CXCL8/CXCR1-, CXCL13/CXCR5-, IL-22-, CD4/IL-17A-, CD4/IL-4-, CD4/IFN-γ-, CD123/IDO-, CD25/Foxp3-, and CD20/IL-10-expressing cells, double immunostaining procedures were performed. Circulating CD4+/CD161-/IL-22+, CD4+/CD161+/IL-17+, CD4+/CD25-/IL-4+, CD4+/CD25-/IFN-γ+, CD4+/CD25hi/Foxp3+, CD3+/CD19+/CD38hi/IL-10+, and CD123+/CD196+/IDO + cells were analyzed by flow cytometry. RESULTS: In the tissue, CXCL10, CXCR5, and CCL20 expression and IL-22+, CD4+/IL-17+, CD4+/IFN-γ + and CD123+/IDO + cell percentages were increased in SLE/DLE versus SLE/SCLE. Circulating CD4+/CD161-/IL-22+, CD4+/CD161+/IL-17+, CD4+/CD25-/IFN-γ+, CD19+/CD38hi/IL-10 + and CD123+/CD196+/IDO + cell percentages were higher in SLE/DLE versus SLE/SCLE. In the tissue, we found positive correlations between CXCR3 and CD4+/IL-17 + cells; CCR2 and CD4+/IFN-γ + cells; and CCR10 and CD123+/IDO + cells in the SLE/DLE patients and between CXCL13 and CD20+/IL-10 + cells in the SLE/SCLE patients. In the peripheral blood, we determined positive correlations between CXCR5 and CD4+/CD25-/IFN-γ + cells; CCL17 and CD4+/CD161-/IL-22 + cells; and CCL17 and CD4+/CD161+/IL-17 + cells in the SLE/DLE patients and between CXCR5 and CD3+/CD19+/CD38hi/IL-10 + cells; CCR2 and CD4+/CD25hi/Foxp3 + cells; and CXCR1 and CD4+/CD25hi/Foxp3 + cells in the SLE/SCLE patients. Positive correlations between the pain score and the expression of CCL2 and CCR6 expression were found in the SLE/SCLE patients. In conclusion, the correlations between the expression of chemokines/receptors and subpopulations of effector/regulatory cells showed differential responses among the cutaneous pathologies.

MicroRNA Expression in Cutaneous Lupus: A New Window to Understand Its Pathogenesis.

BACKGROUND: The role of miRNAs in the pathogenesis of cutaneous lupus has not been studied. OBJECTIVE: It was to assess the levels of a selected panel of circulating miRNAs that could be involved in the regulation of the immune response, inflammation, and fibrosis in cutaneous lupus. METHODS: It was a cross-sectional study. We included 22 patients with subacute (SCLE) and 20 with discoid (DLE) lesions, and 19 healthy donors (HD). qRT-PCR for miRNA analysis, flow cytometry in peripheral blood, and skin immunohistochemistry were performed to determine the distribution of CD4 T cells and regulatory cells and their correlation with circulating miRNAs. RESULTS: miR-150, miR-1246, miR-21, miR-23b, and miR-146 levels were downregulated in SCLE vs. HD. miR-150, miR-1246, and miR-21 levels were downregulated in DLE vs. HD. miR-150, miR-1246, and miR-21 levels were downregulated in DLE γ + with miR-1246 in SCLE, whereas CD123+/CD196+/IDO+ cells were positively associated with miR-150 in DLE. In the tissue, CD4+/IL-4+ and CD20+/IL-10+ cells were positively associated with miR-21 and CD4+/IFN-γ + with miR-1246 in SCLE, whereas CD123+/CD196+/IDO+ cells were positively associated with miR-150 in DLE. In the tissue, CD4+/IL-4+ and CD20+/IL-10+ cells were positively associated with miR-21 and CD4+/IFN-ß, thyroid hormone, and cancer signaling pathways were shared between miR-21, miR-31, miR-23b, miR-146a, miR-1246, and miR-150. CONCLUSIONS: A downregulation of miR-150, miR-1246, and miR-21 in both CLE varieties vs. HD. miR-150, miR-1246, and miR-21 levels were downregulated in DLE.

Up-Regulation of T-Cell Activation MicroRNAs in Drug-Specific CD4+ T-Cells from Hypersensitive Patients.

Dysregulation in the expression of microRNAs (miRNAs), single-stranded RNAs which regulate gene expression, has been associated with diseases such as Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), although their cellular origin has not been explored. Thus, the focus of this work was to study expression patterns of reported miRNAs involved in T-cell activation following drug-specific stimulation in peripheral blood mononuclear cells (PBMCs) and drug-specific CD4+ T-cell clones (TCC) from patients with different cutaneous manifestations of delayed-type drug hypersensitivity reactions. CD4+ T-cells from hypersensitive patients were stimulated to proliferate, secreted cytokines (IFN-γ and IL-22), cytolytic molecules (Granzyme B) and up-regulate miRNAs 24 to 48 h after drug exposure. Carbamazepine-specific CD4+ T-cells that proliferated to the greatest extent and secreted the highest levels of IFN-γ showed an up-regulation of miR-18a and miR-155. In contrast, piperacillin-specific CD4+ T-cells displaying high expression of miR-9 and miR-21 showed an association with the extent of proliferation, but not IFN-γ secretion. MiR-155 up-regulation was detected in PBMCs from all hypersensitive patients 24 h after drug treatment, while miR-18a and miR-21 expression was up-regulated after 48 h. These findings demonstrate that miRNAs are expressed during drug-specific CD4+ T-cell activation and shows a new regulation path for drug hypersensitivity reactions.

Nutritional Deficiency-Related Oral and Cutaneous Lesions in an Obese Patient.

The cutaneous and oral lesions related to nutritional deficiencies are scarcely reported. Micronutrient deficiencies may significantly affect mouth mucosa and skin, causing great morbidity. We report an extraordinary case with detailed clinical and microscopic findings affecting the oral cavity and skin. Physicians must be familiar with these manifestations to suspect the diagnosis.

Pemphigus foliaceus with circinated plaques and neutrophil pustules.

Pemphigus is a group of autoimmune intraepidermal bullous diseases; being pemphigus foliaceus (PF) and pemphigus vulgaris (PV) the most common subtypes. Pustular variants are scarcely reported for both PV and PF. The purpose of this manuscript was to describe the clinical, microscopic and immunologic findings of an atypical case of PF presenting with pustules, including a review of the literature. PF is described as blisters and because this entity is rare, it is not known for the general medical community that they are other clinical features that can be seen as this one we present here with pustules.

Cytokines and Effector/Regulatory Cells Characterization in the Physiopathology of Cutaneous Lupus Erythematous: A Cross-Sectional Study.

We compared the presence of diverse cytokines and regulatory T and B cells in skin biopsies of discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). We included 19 patients with DLE, 13 with SCLE, 8 healthy controls, and 5 patients with hypertrophic scars. We assessed the CLASI activity score. To determine IL-22-producing cells and the subpopulation of CD4(+)/IL-17A(+)-, CD4(+)/IL-4(+)-, and CD4(+)/IFN-γ (+)-expressing T cells, CD123(+)/IDO(+) pDCs, CD25(+)/Foxp3(+) Tregs, and CD20(+)/IL-10(+)-producing B cells, an immunostaining procedure was performed. Also intracellular IL-22, IL-17, IL-4, IFN-γ, and Foxp3 in CD4 T cells, IL-10 in B cells, and IDO in pDCs were analyzed by flow cytometry in peripheral blood. The main cellular participation in both lupus groups was IL-17- and IL-22-producing cell responses both at skin and at peripheral blood but prevailed in DLE. The CLASI activity scores negatively correlated with Th22 subpopulation and positively correlated with CD25(+)/Foxp3(+) Treg cells. In conclusion a proinflammatory and regulatory imbalance coexists in cutaneous lupus, both responses being more intense in DLE.

Focal Epithelial Hyperplasia in Adult Patients With HIV Infection: Clearance With Topical Imiquimod.

Case 1 A 41-year-old man with human immunodeficiency virus (HIV) 1 diagnosed 16 years prior to his consultation was referred for an 8-month history of multiple painless lumps in his mouth. He had A2 status (CD4 cell count of 273 cells/mm3 and viral load of 43,000 copies/L) and was taking treatment with lamivudine/zidovudine (combivir) and efavirenz. Physical examination showed multiple small mucosal-colored and lobulated papulonodules located in the palate and lower gingiva and a whitish verrucous plaque on the lower labial mucosa (Figure 1a). The lesions were diagnosed clinically as focal epithelial hyperplasia (FEH) and further confirmed by classical histopathological findings (Figure 1b). He had previously received unspecified treatment; thus, topical 5% imiquimod cream was initiated every night. Mild erosion and ulceration developed in the upper labial mucosa, which were managed with lubrication (petrolatum ointment). After 2 weeks, all of the small lesions disappeared and the largest plaque resolved 1 week later (Figure 1c). A small residual mass in the area of biopsy, suggesting a scar, remained on the lower lip. The area was removed surgically and corresponded to fibrosis histologically, with no evidence of human papillomavirus (HPV) infection. CD4 cell count (694 cells/mm3) and viral load (<40 copies/L) did not show remarkable changes after imiquimod administration. No serious side effects were observed and the patient has remained free of disease after 1 year of follow-up.

[Leukemia cutis: clinical features of 27 mexican patients and a review of the literature]. / Leucemia cutis (LC): características clínicas de 27 pacientes mexicanos y una breve revisión de la literatura.

BACKGROUND: Leukemia Cutis (LC) consists in neoplastic leukocytic infiltration of the skin and is strongly associated with the presence of extramedullary disease and poor prognosis. However, there are few studies in the literature regarding this entity. We perform a retrospective study of 27 mexican patients in order to analyze the clinical features and prognosis of LC in Mexico, and a brief review of the literature. METHODS: Cases diagnosed as LC by skin biopsy were selected from the database of the Department of Dermatology of National Institute of Medical Science and Nutrition Salvador Zubirán. Cases were searched between the dates of January 1993 and December 2013. RESULTS: Twenty-seven cases which were histologically confirmed with cutaneous leukemic infiltrate were included. Of these patients 60% were male and the mean age at diagnosis was 42 yr (19 to 80 yr). The predominant tipe of LC was acute myeloid leukemia (AML) with 48% of the cases. Nodular neoformations were the main clinical manifestation with 63% of the cases. The mean interval between the diagnosis of LC and death was 10 months (CI 95%). CONCLUSIONS: The presence of LC is a marker of poor prognosis and can precede the relapse of systemic leukemia. Cutaneous infiltration may be the first or the only sign of progression, so doctors should be familiar with the clinical manifestations of this disease.

Pachydermoperiostosis, a unique entity with distinctive clinical features.

Pachydermoperiostosis, which occurs more frequently in men, is a rare entity with distinctive clinical features and an insidious onset.. We report the case of a 30-year-old man with a one-year history of acropachy, arthralgias, hiperhidrosis, and progressive skin thickening of the face and scalp. The radiological findings were consistent with periostosis and the histopathological analysis from a facial skin biopsy showed a pandermal increase in the thickness and number of collagen bundles. The pathogenesis of PDP is currently unknown, although an increased secretion of prostaglandin E2 (PGE2), which stimulates the overexpression of vascular endothelial growth factor (VEGF), has been suggested as a major factor. No specific treatment exists; however, in most cases, the disease tends to stabilize over time.

Pain and pruritus in cutaneous lupus: their association with dermatologic quality of life and disease activity.

OBJECTIVES: To evaluate the association of pain and pruritus with Dermatologic Quality of Life (QoL) and cutaneous disease activity in patients with 1) specific cutaneous lupus erythematosus (CLE) lesions, 2) non-specific CLE lesions and 3) both types of CLE lesions. METHODS: We evaluated 42 consecutive SLE patients with at least one active lesion attributed to lupus. Pain and pruritus were evaluated using a visual analogue scale, dermatologic QoL with the DLQI, clinical activity with the CLASI score and systemic activity with the SLEDAI-2K. RESULTS: The patients were predominantly females, mean age 34.2±11.2 years and median SLE duration of 7 years. Sixteen patients (38%) had specific lesions, 12 (28.5%) non-specific lesions and 14 patients (33%) both lesions. Patients with both lesions had the highest CLASI activity scores (median 17) (p<0.0001), all the cases of severe activity (p=0.002) and higher (worst) DLQI scores (median 11.5, p=0.04). The overall median pain score was 5 (0-9). Patients with non-specific or the combination of both CLE lesions had more pain (p<0.008). Pain correlated with the DLQI (τ=0.38, p=0.001) and the CLASI activity score (τ=0.47, p=0.002). Pain was more intense in vasculitis and bullous lesions followed by oral ulcers. Pruritus score did not differ among groups (median 6) and did not correlate with the DLQI or the CLASI activity score. CONCLUSIONS: We identified pain as a factor that correlated with dermatologic QoL and cutaneous activity. In this sense, this feature needs to be considered as part of the treatment targets in lupus.

Amicrobial pustulosis of the folds associated with autoimmune disorders: systemic lupus erythematosus case series and first report on the association with autoimmune hepatitis.

Amicrobial pustulosis of the folds (APF) associated with autoimmune disorders is an infrequent entity characterized by the recurrent appearance of follicular and nonfollicular sterile pustules in the context of autoimmune disease. Most reports on APF suggest systemic lupus erythematosus (SLE) as the major immunological associated disorder but the association with autoimmune hepatitis (AH) has not been previously documented. We describe the clinical and histological characteristics of 5 patients with APF: 4 with SLE and 1 with AH. As APF is an exclusion diagnosis, in order to establish an opportune diagnosis and treatment, physicians should be aware of patients with any autoimmune disease who develop a pustular dermatosis for which cultures and stains are negative. We propose the inclusion of anti-liver kidney microsome antibodies in the minor criteria for APF diagnosis.

[Anti-MDA5 dermatomyositis. Literature review]. / Dermatomiositis anti-MDA5. Revisión de la literatura.

Dermatomyositis positive anti-melanoma differentiation-associated gene 5 (anti-MDA5 DM) is a rare disease that represents less than 2%. The prevalence of anti-MDA5 DM ranges from 7 to 60%, with higher prevalence in Asian (11-60%) and women. The clinical picture may be variable and is accompanied by the typical features of dermatomyositis, such as periorbital heliotrope (blue-purple) rash with edema, erythematous rash on the face, or the anterior chest (in a V-sign), and back and shoulders (in a shawl sign), violaceous papules or plaques located on the dorsal part of the metacarpophalangeal or interphalangeal joints, which are pathognomonic by definition; yet, one of the most striking signs is the painful ulceration skin that is found in 82% of cases, which is deep and in punching holes or showing hyperkeratotic crusts. For diagnosis is necessary the typical DM rashes (Gottron's papules or Gottron's sign and heliotrope rash), along with either an "interface dermatitis" skin pathology or evidence of myositis or a MSA (myositis-specific autoantibodies). Immunoprecipitation is the gold standard method to detect MSA. Combinations of glucocorticoids and immunosuppressants are used for treatment; besides, it is necessary the detection of rapidly progressive interstitial disease (RP-ILD) with a high-resolution CT because of its high association with fatal prognosis.

La dermatomiositis positiva contra el gen 5 asociado a la diferenciación de melanoma (DM anti-MDA5) es una enfermedad rara que representa menos del 2%. La prevalencia de DM anti-MDA5 varía de 7 a 60%, con mayor prevalencia en asiáticos (11-60%) y mujeres. El cuadro clínico es muy variado y se acompaña por las características típicas de dermatomiositis, como la eritema en heliotropo, con edema, exantema eritematoso en la cara o la parte anterior del tórax (signo de V) y en la espalda y los hombros (signo del chal), las pápulas de Gottron en la parte dorsal de las articulaciones metacarpofalángicas o interfalángicas, que son patognomónicas por definición, pero uno de los signos más llamativos es la ulceración cutánea dolorosa que se encuentra hasta en un 82% de los casos, es profunda y en sacabocados muestran costras hiperqueratósicas. Para el diagnóstico son necesarias las erupciones típicas de la DM (pápulas de Gottron o signo de Gottron y erupción de heliotropo), junto con una patología cutánea de "dermatitis de interfase" o evidencia de miositis o un MSA (autoanticuerpos específicos de miositis). La inmunoprecipitación es el método de referencia para detectar MSA. Para su tratamiento se usan combinaciones de glucocorticoides e inmunosupresores; ademas, es necesaria la detección de enfermedad intersticial rápidamente progresiva (RP-ILD) con tomografía computarizada de alta resolución por su alta asociación con un pronóstico fatal.

Clinical atrophy patterns of filiform papillae of the tongue and their relationship with the serum levels of iron, vitamin B12 and folic acid in patients with systemic diseases.

BACKGROUND: The purpose of the study was to evaluate the clinical patterns of atrophy of the filiform papillae (FP) of the tongue and their relationship with the serum levels of iron and vitamin B12 among patients with systemic diseases, in a tertiary care center. METHODS: A cross-sectional, analytical, research study was designed. A systematic tongue examination was performed to evaluate the presence and clinical patterns of FP atrophy. We collected epidemiologic, clinical, and laboratory data. Statistical analysis included χ2 test, Fisher's exact test, Kruskal-Wallis test, and a logistic regression analysis. RESULTS: A total of 87 patients (83.9% females) were included [median age = 55 (range 20-89) years]. Endocrinopathy (60.9%) was the most frequent comorbidity. We found atrophy of the FP in 90.8% of the patients; the atrophy was mild in 83.5% of the cases, and severe in 16.5%. The most common atrophic patterns were as follows: focalized in 64 (73.6%) cases, "U"-shaped pattern in 60 (69%), and generalized in 30 (34.5%). Geographic tongue and median rhomboid glossitis were observed in 12 (13.8%) and 11 (12.6%) subjects, respectively. Lower titers of serum iron were detected in cases with focal (median = 71 vs. 110 mcg/dl) and generalized (median = 55 vs. 78 mcg/dl) FP atrophy (P = 0.03 and P = 0.009, respectively), than their counterparts. The presence of symptomatology was related to the focal pattern of atrophy (P = 0.038). CONCLUSIONS: A high frequency of filiform papillary atrophy of the tongue was observed in patients with comorbidities. Some atrophic patterns of the tongue were significantly associated with certain medical conditions.

Nail Changes in People Living with Human Immunodeficiency Virus: Observational and Cross-Sectional Study in a Third-Level Hospital.

Introduction: Nail changes in people living with human immunodeficiency virus (HIV) have been scarcely reported. The aim of this study was to establish the frequency and characteristics of nail alterations observed in adults with HIV infection in a third-level hospital in Mexico. Method: Observational and cross-sectional study carried out in 205 patients receiving care at the HIV/AIDS Clinic of the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán (INCMNSZ) in Mexico City. We performed a nail and iconographic assessment of both hands and toenails. We collected information of demographic and clinical variables, as well as drugs use, and antiretroviral treatment used by the participants through a questionnaire and from medical records. We performed direct cytological examinations and nail mycological cultures in participants with symptoms of onychomycosis. Results: The participants were predominantly male patients (91.2%), with a mean age of 41 (range 21-78) years, under antiretroviral therapy (91.2%), with a suppressed viral load (78.5%) and mean CD4+ lymphocyte count of 379.5 (range 20-1,162) cells/µL. Fitzpatrick's IV phototype was prevailing in the studied population (70%). Nail changes were documented in 72.2% of the patients; being pigmentary changes (37.1%) and trauma (30.7%) the most frequent. Onychomycosis was observed in 26.3%; with total dystrophic onychomycosis as the most frequent clinical variant (68.5%). We obtained fungal isolates in 59.3% of participants and Candida parapsilosis was the most frequent of these (37.5%). Conclusions: We observed a high prevalence of nail changes with very diverse etiology, as well as a variety of nondermatophytic yeasts and molds isolates associated with cases with onychomycosis. These findings reinforce and confirm the need for routine nail examination and stress the importance of medical personnel working with people living with HIV to have broad knowledge of nail pathology.