Self-healing juvenile cutaneous mucinosis: Clinical and histopathologic findings of 9 patients: The relevance of long-term follow-up.

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.

Reticular erythematous mucinosis (REM) with telangiectasias associated with essential thrombocytosis and lung carcinoma.

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.

Positive photobiological investigation in reticular erythematous mucinosis syndrome.

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.

Self-healing juvenile cutaneous mucinosis.

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.

Mucinosis eritematosa reticular: a proposito de dos casos / Reticular eritematous mucinosis: a propouse of too cases

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

Self-healing cutaneous mucinosis.

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.

Papular mucinosis and human immunodeficiency virus infection.

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.

Mucinosis pápulo-nodular asociada a Lupus Eritematoso Sistémico con poliserositis, esplenomegalia y adenomegalias

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.