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1.
Rev Med Chil ; 149(5): 747-757, 2021 May.
Article in Spanish | MEDLINE | ID: mdl-34751328

ABSTRACT

Monoclonal gammopathies of uncertain significance (MGUS) correspond to pre-malignant hematological disorders characterized by the production of a monoclonal protein and infiltration of less than 10% of the bone marrow by plasma cells. Its importance lies in the risk of progression to malignant disorders and in the association with different renal, neurological and skin manifestations. There are pathophysiological mechanisms that support a causal relationship between monoclonal gammopathies (MGs) and different skin diseases, such as type I cryoglobulinemia (CG), primary systemic amyloidosis (PSA) or necrobiotic xanthogranuloma (NXG). However, there is a group of skin diseases associated with MGs whose pathogenesis has not been elucidated. In this context, the role of the dermatologist is crucial in the suspicion of different haematological disorders based on skin manifestations and in the multidisciplinary treatment of these patients. In this article, we carry out an exhaustive review of the literature published in this area and propose a screening algorithm for MGs in patients with specific skin diseases.


Subject(s)
Immunoglobulin Light-chain Amyloidosis , Monoclonal Gammopathy of Undetermined Significance , Paraproteinemias , Skin Diseases , Bone Marrow , Humans , Paraproteinemias/complications , Skin Diseases/etiology
2.
Rev. méd. Chile ; 149(5): 747-757, mayo 2021. tab, ilus
Article in Spanish | LILACS | ID: biblio-1389512

ABSTRACT

Monoclonal gammopathies of uncertain significance (MGUS) correspond to pre-malignant hematological disorders characterized by the production of a monoclonal protein and infiltration of less than 10% of the bone marrow by plasma cells. Its importance lies in the risk of progression to malignant disorders and in the association with different renal, neurological and skin manifestations. There are pathophysiological mechanisms that support a causal relationship between monoclonal gammopathies (MGs) and different skin diseases, such as type I cryoglobulinemia (CG), primary systemic amyloidosis (PSA) or necrobiotic xanthogranuloma (NXG). However, there is a group of skin diseases associated with MGs whose pathogenesis has not been elucidated. In this context, the role of the dermatologist is crucial in the suspicion of different haematological disorders based on skin manifestations and in the multidisciplinary treatment of these patients. In this article, we carry out an exhaustive review of the literature published in this area and propose a screening algorithm for MGs in patients with specific skin diseases.


Subject(s)
Humans , Paraproteinemias/complications , Skin Diseases/etiology , Monoclonal Gammopathy of Undetermined Significance , Immunoglobulin Light-chain Amyloidosis , Bone Marrow
4.
Eur J Dermatol ; 31(6): 813-821, 2021 Dec 01.
Article in English | MEDLINE | ID: mdl-35107072

ABSTRACT

BACKGROUND: The effectiveness of methotrexate (MTX), a first-line treatment for localised scleroderma (morphea), has not been assessed using colour Doppler ultrasonography (CDU). OBJECTIVES: We aimed to ultrasonographically monitor disease activity in patients with morphea treated with MTX, assessing its effectiveness using an Ultrasound Morphea Activity Score (US-MAS). MATERIALS & METHODS: A retrospective cohort of 22 patients was studied between July 2014 and July 2019. The morphea of each patient, treated with MTX, was confirmed by histology and all patients had at least two CDU examinations. The US-MAS is based on published ultrasound signs of disease activity validated by histology. A weight-adjusted average MTX dose (mg/kg/wk) was used to standardize dosage, weight, and time between CDU examinations. The difference in US-MAS between two CDU examinations was determined. Statistical analyses included Wilcoxon and Fisher exact tests, the Spearman correlation coefficient, and risk ratios with 95% confidence intervals. To create two groups, we determined the median of the sample as the cut-off point for MTX dose (0.265 mg/kg/week). Significance was set at p≤0.05; Results: In all cases, CDU examinations showed subclinical signs of activity beyond the visible lesional borders, either in the same or adjacent corporal segments. A negative correlation was found between the change in US-MAS and MTX dose (Spearman coefficient, -0.45; p = 0.035). The group dosed at ≥0.265 mg/kg/wk showed a non-significant change in US-MAS (2-point decrease). No case became inactive. CONCLUSION: MTX is a treatment with a low effectiveness for morphea, causing only slight decreases in ultrasound activity at higher doses.


Subject(s)
Dermatologic Agents/therapeutic use , Immunosuppressive Agents/therapeutic use , Methotrexate/therapeutic use , Scleroderma, Localized/diagnostic imaging , Scleroderma, Localized/drug therapy , Ultrasonography, Doppler, Color , Adolescent , Adult , Child , Dermatologic Agents/administration & dosage , Disease Progression , Female , Humans , Immunosuppressive Agents/administration & dosage , Male , Methotrexate/administration & dosage , Middle Aged , Retrospective Studies , Scleroderma, Localized/pathology , Severity of Illness Index , Treatment Outcome , Young Adult
5.
Clin Cosmet Investig Dermatol ; 12: 373-381, 2019.
Article in English | MEDLINE | ID: mdl-31239742

ABSTRACT

Molluscum contagiosum (MC) is a self-limited infectious dermatosis, frequent in pediatric population, sexually active adults, and immunocompromised individuals. It is caused by molluscum contagiosum virus (MCV) which is a virus of the Poxviridae family. MCV is transmitted mainly by direct contact with infected skin, which can be sexual, non-sexual, or autoinoculation. Clinically, MC presents as firm rounded papules, pink or skin-colored, with a shiny and umbilicated surface. The duration of the lesions is variable, but in most cases, they are self-limited in a period of 6-9 months. The skin lesions may vary in size, shape, and location, which is more frequent in immunosuppressed patients, and could present complications such as eczema and bacterial superinfection. The diagnosis is based on clinical findings. A useful clinical tool is dermoscopy. If the diagnostic doubt persists, confocal microscopy or skin biopsy could be performed. The need for active treatment for MC is controversial; however, there is a consensus that it should be indicated in cases of extensive disease, associated with complications or aesthetic complaints. There are several treatment modalities which include mechanical, chemical, immunomodulatory, and antivirals. The objective of this article is to review the current evidence in etiology, clinical manifestations, diagnosis, and management alternatives of MC.

6.
Indian J Dermatol Venereol Leprol ; 85(3): 287-290, 2019.
Article in English | MEDLINE | ID: mdl-30246704

ABSTRACT

Basaloid follicular hamartoma is a rare, benign and superficial malformation of hair follicles, characterized histologically by epithelial proliferation of basaloid cells with radial disposition. It can be mistaken for basal cell carcinoma. Even though these hamartomas are considered benign lesions, malignant transformation has rarely been reported. We report the case of a 45-year-old healthy woman, with linear, unilateral basaloid follicular hamartoma which developed inflamed papules histologically suggestive of basal cell carcinoma. We believe that identification of local inflammation could be a clinical clue to guide us towards a malignant transformation of basaloid follicular hamartoma.


Subject(s)
Hair Follicle/abnormalities , Hair Follicle/pathology , Hamartoma/diagnosis , Skin Diseases, Genetic/diagnosis , Female , Hair Follicle/surgery , Hamartoma/pathology , Hamartoma/surgery , Humans , Inflammation/diagnosis , Inflammation/pathology , Inflammation/surgery , Middle Aged , Skin Diseases, Genetic/pathology , Skin Diseases, Genetic/surgery
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