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3.
J Am Acad Dermatol ; 78(6): 1164-1170, 2018 Jun.
Article in English | MEDLINE | ID: mdl-29066274

ABSTRACT

BACKGROUND: Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder, and its pathogenesis and long-term prognosis are unknown. OBJECTIVE: To elucidate the clinical and histopathologic characteristics, pathogenesis, and outcome in patients with SHJCM. METHODS: Retrospective study of 9 patients with SHCJM. To complement initial findings, data collection forms were sent to the referring physicians. RESULTS: All patients had an acute onset of firm nodules. Of the 9 patients, 6 presented initially with waxy papules on the dorsum of the hands; 5 suffered from periorbital edema, and 6 had a febrile prodrome. Histopathologic assessment of the papules revealed dermal mucin deposition, whereas the nodules showed proliferative fasciitis-like features or nonspecific chronic lobular panniculitis. Laboratory studies elicited evidence of active viral infection in 2 patients (human herpes virus 6 and rotavirus). Seven cases had spontaneous resolution within 6 months, and 2 patients with incomplete resolution showed subsequent transition to fibroblastic rheumatism and an autoinflammatory rheumatologic disease, respectively. LIMITATIONS: This was a retrospective study with incomplete data from referring physicians. CONCLUSIONS: Although spontaneous complete regression is expected, patients with SHJCM need long-term follow-up because of the possible development of dematorheumatolgic conditions. The pathogenetic role of microbial agents deserves further investigation.


Subject(s)
Mucinoses/pathology , Mucinoses/physiopathology , Remission, Spontaneous , Skin Diseases, Papulosquamous/pathology , Skin Diseases, Papulosquamous/physiopathology , Age Factors , Biopsy, Needle , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Immunohistochemistry , Incidence , Infant , Male , Monitoring, Physiologic/methods , Mucinoses/epidemiology , Retrospective Studies , Risk Assessment , Sampling Studies , Severity of Illness Index , Sex Factors , Skin Diseases, Papulosquamous/epidemiology , Time Factors
5.
Actas dermo-sifiliogr. (Ed. impr.) ; 100(10): 857-860, dic. 2009.
Article in Spanish | IBECS | ID: ibc-77439

ABSTRACT

En este artículo se efectuó una amplia descripción clínico-patológica y cronológica de las manifestaciones cutáneas del síndrome tóxico por aceite de colza adulterado o síndrome tóxico por aceite. Esta nueva enfermedad se produjo en España en 1981, debida a la ingesta de aceite de colza destinado a uso industrial, que había sido coloreado con anilinas y posteriormente decolorado de forma fraudulenta y vendido como aceite de oliva. En total se produjeron unos 20.000 casos y unos 400 fallecimientos. Existía un predominio por el sexo femenino, sobre todo en la fase tardía. En la fase aguda las manifestaciones cutáneas predominantes fueron exantemas toxoalérgicos, con un patrón dermatopatológico de erupción alérgica urticariforme. Alrededor de un 25% de los casos desarrolló posteriormente un aspecto edematoso de la piel, con trastornos de la pigmentación, que demostró estar relacionado con mucinosis dérmica. Finalmente se produjo un cuadro esclerodermiforme peculiar, que tendió a mejorar de forma espontánea. La presencia constante de mastocitos en todas las biopsias y el desarrollo de mastocitosis en varios pacientes sugirieron un papel importante del mastocito en la patogenia del cuadro, que luego ha sido ratificada en otros procesos esclerodermiformes. En 1989 apareció en EE.UU. el síndrome mialgia-eosinofilia, relacionado con sustancias tóxicas presentes en suplementos alimentarios de triptófano y que compartía muchos aspectos con el síndrome tóxico por aceite. Esto corroboró la existencia de mucinosis y cuadros esclerodermiformes de origen tóxico (AU)


This article offered an extensive description of the clinical and pathological features and time-course of the skin manifestations of toxic syndrome caused by denatured rapeseed oil, also known as toxic oil syndrome. This new condition occurred in Spain in 1981 and was due to the ingestion of rapeseed oil intended for industrial use that had been denatured with anilines and subsequently refined and sold fraudulently as olive oil. In total, 20 000 cases and 400 deaths were reported. The disease affected mainly women, particularly in the late stages. In the acute phase, the predominant skin manifestations were toxic-allergic rashes reminiscent of allergic urticaria in the dermatopathologic study. In approximately 25% of cases, the patients’ skin subsequently took on an edematous appearance, with pigmentary abnormalities shown to be related to cutaneous mucinosis. Finally, a characteristic sclerodermatous condition would develop that tended to improve spontaneously. The constant presence of mast cells in all biopsies and the development of mastocytosis in several patients pointed to an important role for these cells in the pathogenesis of the condition. This was subsequently confirmed in other sclerodermatous processes. In 1989, eosinophilia-myalgia syndrome caused by toxins present in tryptophan food supplements was reported in the United States. This syndrome resembled toxic oil syndrome in many ways and demonstrated that mucinosis and toxic sclerodermatous processes do exist (AU)


Subject(s)
Humans , Foodborne Diseases/physiopathology , Oils/toxicity , Mastocytosis/physiopathology , Exanthema/physiopathology , Mucinoses/physiopathology , Scleroderma, Diffuse/physiopathology , Fasciitis/physiopathology , Eosinophilia-Myalgia Syndrome/physiopathology
7.
Eur J Dermatol ; 15(3): 179-81, 2005.
Article in English | MEDLINE | ID: mdl-15908303

ABSTRACT

The cutaneous mucinoses are a heterogeneous group of diseases in which mucin accumulates in the skin. Reticular erythematous mucinosis (REM) is an infrequent variant. We present a 48-year-old man with essential thrombocytosis and REM lesions with atypical telangiectasias on his chest, who developed a non-small cell lung carcinoma. We discuss the unusual clinical finding of telangiectasias over REM lesions and the association with essential thrombocytosis and lung carcinoma.


Subject(s)
Carcinoma, Non-Small-Cell Lung/pathology , Lung Neoplasms/pathology , Mucinoses/pathology , Paraneoplastic Syndromes/pathology , Telangiectasis/pathology , Thrombocythemia, Essential/pathology , Biopsy, Needle , Carcinoma, Non-Small-Cell Lung/physiopathology , Humans , Immunohistochemistry , Lung Neoplasms/physiopathology , Male , Middle Aged , Mucinoses/physiopathology , Paraneoplastic Syndromes/diagnosis , Prognosis , Risk Assessment , Telangiectasis/physiopathology , Thrombocythemia, Essential/physiopathology
8.
Photodermatol Photoimmunol Photomed ; 20(5): 235-8, 2004 Oct.
Article in English | MEDLINE | ID: mdl-15379872

ABSTRACT

BACKGROUND: Reticular erythematous mucinosis (REM) syndrome is a rare disorder. Its clinical course is cyclic with remissions and exacerbations. In this disease, photosensitivity has previously been noticed but rarely demonstrated. We report three new cases with positive photobiological investigation. CASE REPORTS: Three patients (two males, one female) with a mean age 47 years were seen with reticular erythematous papules on the upper chest and or back. After sun exposure, the lesions were exacerbated. Skin biopsies showed dermal lymphocytic perivascular infiltration with mucin deposition between collagen bundles. Direct immunofluorescence was negative. Antinuclear antibodies were absent. In cabin, ultraviolet (UV)A exposure reproduced clinically and histologically REM lesions in our cases. UVA and UVB provocating phototests were negative. In all patients treatment with oral antimalarials and external photoprotection was effective. CONCLUSIONS: In our patients, we confirm the photosensitive feature of REM syndrome by provocative irradiation in UVA cabin. The mechanism of triggering is actually unclear. It is supposed that UV radiation, heat, and perspiration are necessary to reveal this affection.


Subject(s)
Mucinoses/physiopathology , Photosensitivity Disorders/physiopathology , Sunlight/adverse effects , Adult , Aged , Biopsy , Collagen , Erythema/physiopathology , Female , Humans , Lymphocytes/pathology , Male , Middle Aged , Mucinoses/pathology , Mucins , Photobiology , Photosensitivity Disorders/pathology , Syndrome , Ultraviolet Rays/adverse effects
9.
J Am Acad Dermatol ; 50(5 Suppl): S97-100, 2004 May.
Article in English | MEDLINE | ID: mdl-15097940

ABSTRACT

Self-healing juvenile cutaneous mucinosis is an extremely uncommon disorder characterized by the acute eruption of multiple papules and subcutaneous nodules. In this report, we describe one of the youngest patients reported to date with self-healing juvenile cutaneous mucinosis and further define several of the histologic and clinical characteristics unique to the disorder. Awareness of this disease is important because, despite an ominous presentation, all reported cases have resolved spontaneously. Therefore, aggressive therapy should be avoided.


Subject(s)
Mucinoses , Skin Diseases , Child, Preschool , Humans , Male , Mucinoses/pathology , Mucinoses/physiopathology , Remission, Spontaneous , Skin/pathology , Skin Diseases/pathology , Skin Diseases/physiopathology , Subcutaneous Tissue/pathology
10.
Rev. argent. dermatol ; 80(3): 130-5, jul.-sept. 1999. ilus
Article in Spanish | LILACS | ID: lil-258688

ABSTRACT

Se presentan dos pacientes con mucinosis eritematosa reticular (M.E.R.) en edad media de la vida. Realizamos una revisión de la literatura, para actualizar conceptos de la clínica y patología


Subject(s)
Male , Female , Adult , Middle Aged , Hydroxychloroquine/therapeutic use , Mucinoses/classification , Mucinoses/diagnosis , Mucinoses/pathology , Mucinoses/physiopathology , Mucinoses/therapy , Biopsy , Hydroxychloroquine/administration & dosage
11.
Rev. argent. dermatol ; 80(3): 130-5, jul.-sept. 1999. ilus
Article in Spanish | BINACIS | ID: bin-12837

ABSTRACT

Se presentan dos pacientes con mucinosis eritematosa reticular (M.E.R.) en edad media de la vida. Realizamos una revisión de la literatura, para actualizar conceptos de la clínica y patología(AU)


Subject(s)
Male , Female , Adult , Middle Aged , Mucinoses/classification , Mucinoses/diagnosis , Mucinoses/physiopathology , Mucinoses/pathology , Mucinoses/therapy , Hydroxychloroquine/therapeutic use , Biopsy , Hydroxychloroquine/administration & dosage
12.
Dermatology ; 192(3): 268-70, 1996.
Article in English | MEDLINE | ID: mdl-8726646

ABSTRACT

Self-healing juvenile cutaneous mucinosis is a new entity distinct from previously described forms of cutaneous mucinosis. We present a new case in a 26-year-old female patient who began abruptly with a papular eruption, involving the scalp, face, neck and trunk, accompanied by periarticular papules on the hands and arthralgias. No associated disease could be detected and the lesions resolved spontaneously within a few months. This is the first case described in an adult patient.


Subject(s)
Mucinoses/physiopathology , Adult , Age of Onset , Arthralgia/etiology , Female , Humans , Mucinoses/pathology , Remission, Spontaneous
13.
Cutis ; 55(2): 113-4, 1995 Feb.
Article in English | MEDLINE | ID: mdl-7729156

ABSTRACT

Cutaneous mucin deposition occurs both as an isolated phenomenon and in patients with various systemic disorders. Among these are endocrinopathies (eg, hypo- and hyperthyroidism), malignancy (mycosis fungoides), connective tissue disorders (lupus erythematosus), and infectious diseases (scleredema associated with upper respiratory tract infection). We present a case of papular mucinosis in a patient infected with the human immunodeficiency virus. This case represents the third report of these disorders coexisting.


Subject(s)
HIV Infections/complications , Mucinoses/complications , Mucinoses/diagnosis , Adult , Biopsy , Humans , Male , Mucinoses/physiopathology
14.
Acta méd. colomb ; 17(6): 459-63, nov.-dic. 1992. ilus, tab
Article in Spanish | LILACS | ID: lil-183247

ABSTRACT

Describimos el caso de un paciente de 24 años, con un cuadro clínico de 33 meses de evolución de un lupus eritomatoso sistémico asociado a lesiones pápulo-nodulares en miembros superiores e inferiores en el tronco. La biopsia de piel de las lesiones papulo-nodulares demostró acúmulos de mucina y abundantes mastocitos en la dermis intercalados con tejido colágeno. Revisamos la literatura y sólo se han informado 18 casos, ninguno en Latinoamérica. A diferencia de los casos descritos en la literatura médica, no hay informe de poliserositis, adenomegalias y esplenomegalia asociados a la mucinosis pápulo-nodular como se describe en este paciente. Las lesiones cutáneas se manifestaron con la actividad lúpica y la respuesta a la prednisona fue adecuada.


Subject(s)
Humans , Female , Adult , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/physiopathology , Mucinoses/classification , Mucinoses/complications , Mucinoses/diagnosis , Mucinoses/drug therapy , Mucinoses/epidemiology , Mucinoses/etiology , Mucinoses/physiopathology , Mucinoses/therapy , Mucinosis, Follicular/complications , Mucinosis, Follicular/diagnosis , Mucinosis, Follicular/drug therapy , Mucinosis, Follicular/epidemiology , Mucinosis, Follicular/etiology , Mucinosis, Follicular/physiopathology , Mucinosis, Follicular/therapy , Splenomegaly/complications , Splenomegaly/etiology , Splenomegaly/physiopathology
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