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 FactorsABSTRACT
BACKGROUND: Reticular erythematous mucinosis (REM syndrome) is a rare cutaneous disease that predominantly affects the chest and upper back area of middle-aged women. Although antimalarial treatment is generally considered the most effective approach, only a few case reports exist on its use in REM syndrome. OBSERVATIONS: A total of 11 patients with REM syndrome (10 women and 1 man), mean age, 44 years (age range, 37-54 years), were included in this retrospective analysis. Ten of the 11 patients were cigarette smokers (91%), and 6 had concomitant autoimmune diseases (55%). Since no clinical score exists for REM syndrome, we used the validated Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) to evaluate the efficacy of antimalarial treatment. Overall, a significant decrease in the clinical score was observed from a median of 4 (range, 2-8) before initiation of treatment to 0 (range, 0-4) after 3 months of antimalarial therapy and to 0 (range, 0-4) after 12 months of therapy (P < .001). Two patients withdrew from the study owing to adverse gastrointestinal tract effects (nausea and vomiting); 2 relapsed after finishing their antimalarial regimens; 3 patients were free of disease 2 years after the end of treatment; and 4 patients were lost to follow-up. CONCLUSION: Antimalarial agents significantly improve or completely clear the skin lesions of patients with REM syndrome and should be considered as a first-line therapy for this rare disease.
Subject(s)
Antimalarials/therapeutic use , Erythema/drug therapy , Mucinoses/drug therapy , Adult , Antimalarials/adverse effects , Chloroquine/adverse effects , Chloroquine/therapeutic use , Comorbidity , Erythema/epidemiology , Erythema/pathology , Female , Humans , Male , Middle Aged , Mucinoses/epidemiology , Mucinoses/pathology , Retrospective Studies , Smoking/epidemiology , Steroids/therapeutic useABSTRACT
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Subject(s)
Humans , Female , Adult , Skin Diseases, Vesiculobullous/complications , Skin Diseases, Vesiculobullous/diagnosis , Skin Diseases, Vesiculobullous/physiopathology , Mucinoses/complications , Mucinoses/diagnosis , Mucinoses/classification , Mucinoses/epidemiologyABSTRACT
Species specific diseases, e.g. idiopathic mucinosis of the Chinese Shar Pei are reported by use of 31 clinical cases and 140 biopsy samples. Idiopathic mucinosis is one of the most frequent diseases of this increasing breed in Germany, too. The disease is diagnosed by use of histologic examination of skin biopsies. Therapy is performed by application of corticosteroids and symptomatic treatment.
Subject(s)
Dog Diseases/pathology , Mucinoses/veterinary , Skin/pathology , Adrenal Cortex Hormones/therapeutic use , Age Factors , Animals , Biopsy , Dog Diseases/epidemiology , Dog Diseases/therapy , Dogs , Female , Germany/epidemiology , Inbreeding , Male , Mucinoses/epidemiology , Mucinoses/pathology , Mucinoses/therapy , Species SpecificityABSTRACT
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.
