Cutaneous mucinosis in mixed connective tissue disease
CASE 1: A female patient, 44-years-old, diagnosed with MCTD (Kasukawa's\nclassification criteria) for eight years. She had Raynaud's phenomenon, swollen hands,\npolyarthritis, photosensitivity, esophageal ectasia, leukopenia, lymphopenia,\npredominantly axonal peripheral sensorimotor polyneuropathy, sensorineural hearing loss\nand anterior uveitis. Anti-RNP antibodies titers were 1/1200 (haemaglutination). The\npatient remained stable for over one year taking azathioprine (1.5 mg/ kg / day) and\nwhen joint activity initiated was treated with prednisone 10 mg/ day and leflunomida,\nwith improvement (). A month later she\nnoticed papules on the neck, back and face, which were biopsied. The histopathology\nanalysis revealed a mild lymphocytic infiltrate in lymphocytic perivascular and\nperiadnexal areas in the superficial dermis, with some melanophages and collagen fibers\ndissociation (). The PAS showed focal\nincrease of the basal layer of the epidermis. The deposition of mucin was confirmed by\nAlcian-Blue staining. The epidermis was normal. Immunofluorescence\nshowed no deposition of C3, anti-IgM, anti-IgG or anti-IgA antibodies. Histopathological\nfindings were therefore consistent with cutaneous papular mucinosis. The appearance of\nthis skin condition occurred independently of other manifestations of MCTD. The dose of\nazathioprine was increased to 2mg/kg/day. This led to an improvement of the lesions.