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Cutaneous mucinosis in mixed connective tissue disease.

Favarato MH, Miranda SS, Caleiro MT, Assad AP, Halpern I, Fuller R - An Bras Dermatol (2013 Jul-Aug)

Bottom Line: It is described in some connective tissue diseases but never in association with mixed connective tissue disease.This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis.Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

View Article: PubMed Central - PubMed

Affiliation: Internal Medicine Department, University of São Paulo, São Paulo(SP),Brazil.

ABSTRACT
Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

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Case 1 - papules on the back
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f01: Case 1 - papules on the back

Mentions: CASE 1: A female patient, 44-years-old, diagnosed with MCTD (Kasukawa'sclassification criteria) for eight years. She had Raynaud's phenomenon, swollen hands,polyarthritis, photosensitivity, esophageal ectasia, leukopenia, lymphopenia,predominantly axonal peripheral sensorimotor polyneuropathy, sensorineural hearing lossand anterior uveitis. Anti-RNP antibodies titers were 1/1200 (haemaglutination). Thepatient remained stable for over one year taking azathioprine (1.5 mg/ kg / day) andwhen joint activity initiated was treated with prednisone 10 mg/ day and leflunomida,with improvement (Figure 1). A month later shenoticed papules on the neck, back and face, which were biopsied. The histopathologyanalysis revealed a mild lymphocytic infiltrate in lymphocytic perivascular andperiadnexal areas in the superficial dermis, with some melanophages and collagen fibersdissociation (Figure 2). The PAS showed focalincrease of the basal layer of the epidermis. The deposition of mucin was confirmed byAlcian-Blue staining. The epidermis was normal. Immunofluorescenceshowed no deposition of C3, anti-IgM, anti-IgG or anti-IgA antibodies. Histopathologicalfindings were therefore consistent with cutaneous papular mucinosis. The appearance ofthis skin condition occurred independently of other manifestations of MCTD. The dose ofazathioprine was increased to 2mg/kg/day. This led to an improvement of the lesions.


Cutaneous mucinosis in mixed connective tissue disease.

Favarato MH, Miranda SS, Caleiro MT, Assad AP, Halpern I, Fuller R - An Bras Dermatol (2013 Jul-Aug)

Case 1 - papules on the back
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3760946&req=5

f01: Case 1 - papules on the back
Mentions: CASE 1: A female patient, 44-years-old, diagnosed with MCTD (Kasukawa'sclassification criteria) for eight years. She had Raynaud's phenomenon, swollen hands,polyarthritis, photosensitivity, esophageal ectasia, leukopenia, lymphopenia,predominantly axonal peripheral sensorimotor polyneuropathy, sensorineural hearing lossand anterior uveitis. Anti-RNP antibodies titers were 1/1200 (haemaglutination). Thepatient remained stable for over one year taking azathioprine (1.5 mg/ kg / day) andwhen joint activity initiated was treated with prednisone 10 mg/ day and leflunomida,with improvement (Figure 1). A month later shenoticed papules on the neck, back and face, which were biopsied. The histopathologyanalysis revealed a mild lymphocytic infiltrate in lymphocytic perivascular andperiadnexal areas in the superficial dermis, with some melanophages and collagen fibersdissociation (Figure 2). The PAS showed focalincrease of the basal layer of the epidermis. The deposition of mucin was confirmed byAlcian-Blue staining. The epidermis was normal. Immunofluorescenceshowed no deposition of C3, anti-IgM, anti-IgG or anti-IgA antibodies. Histopathologicalfindings were therefore consistent with cutaneous papular mucinosis. The appearance ofthis skin condition occurred independently of other manifestations of MCTD. The dose ofazathioprine was increased to 2mg/kg/day. This led to an improvement of the lesions.

Bottom Line: It is described in some connective tissue diseases but never in association with mixed connective tissue disease.This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis.Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

View Article: PubMed Central - PubMed

Affiliation: Internal Medicine Department, University of São Paulo, São Paulo(SP),Brazil.

ABSTRACT
Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

Show MeSH
Related in: MedlinePlus