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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 2: erythematous-violaceous indurated mildly pruritic and painless lesionson trunk
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f03: Case 2: erythematous-violaceous indurated mildly pruritic and painless lesionson trunk

Mentions: CASE 2: A 42-year-old male, diagnosed with MCTD (Kasukawa's classificationcriteria) for eight years. He had Raynaud's phenomenon, hand swelling, pleurisy,lymphadenopathy, arthritis, myopathy and anti-RNP antibodies titers 1/6400(haemaglutination. The patient remained clinically stable for more than seven monthswith prednisone 15mg/day and azathioprine 2.0 mg/ kg / day when he presentederythematous-violaceous indurated mildly pruritic and painless lesions in the face,trunk and upper limbs (Figure 3). As there were noother signs of MCTD disease activity, the dose of prednisone was reduced to 7.5 mg/ dayand azathioprine was discontinued. Histopathological analysis of papules of the trunkshowed epidermal parakeratosis and hyperorthokeratosis. Perivascular, interstitial andperiadnexal lymphocytic inflammatory infiltrate appeared in the dermis (predominantlythe former). The alcian-blue and the PAS staining revealed interstitialmucinosis in the reticular dermis. Chloroquine diphosphate was introduced (250 mg/ day)and the prednisone dose was increased to 20 mg/ day, with improvement of thelesions.


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 2: erythematous-violaceous indurated mildly pruritic and painless lesionson trunk
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f03: Case 2: erythematous-violaceous indurated mildly pruritic and painless lesionson trunk
Mentions: CASE 2: A 42-year-old male, diagnosed with MCTD (Kasukawa's classificationcriteria) for eight years. He had Raynaud's phenomenon, hand swelling, pleurisy,lymphadenopathy, arthritis, myopathy and anti-RNP antibodies titers 1/6400(haemaglutination. The patient remained clinically stable for more than seven monthswith prednisone 15mg/day and azathioprine 2.0 mg/ kg / day when he presentederythematous-violaceous indurated mildly pruritic and painless lesions in the face,trunk and upper limbs (Figure 3). As there were noother signs of MCTD disease activity, the dose of prednisone was reduced to 7.5 mg/ dayand azathioprine was discontinued. Histopathological analysis of papules of the trunkshowed epidermal parakeratosis and hyperorthokeratosis. Perivascular, interstitial andperiadnexal lymphocytic inflammatory infiltrate appeared in the dermis (predominantlythe former). The alcian-blue and the PAS staining revealed interstitialmucinosis in the reticular dermis. Chloroquine diphosphate was introduced (250 mg/ day)and the prednisone dose was increased to 20 mg/ day, with improvement of thelesions.

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