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| Author, year, diagnosis | Demographics | Clinical description | Pathology (skin biopsy) | Associated condition(s) | Therapy and course |
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Johnson et al., 1973 [8]
DM with lichen myxedematosus | 35 African American males | Pruritic papular rash on upper and lower extremities, face, chest, and back. With perifollicular papules on forearms, hands, back, face and lower extremities, dysphagia and proximal muscle weakness of shoulders and pelvic girdle, and nail fold telangiectasia | Fragmented collagen with fibroblast proliferation and mucinous changes extending from the epidermis into the dermis with perivascular lymphocytosis and occasional histiocytes | None | 80 mg oral prednisone daily with good response |
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Igarashi et al., 1985 [9]
DM with cutaneous mucinosis | 67 Japanese males | Poikilodermatous lesions on face, chest, and extremities | Skin bx: frayed and fragmented collagen bundles with mucinous material in between bundles when stained with alcian blue | Gastric cancer | Unknown |
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Requena et al., 1990 [3]
DM with mucinosis | 66 females | Erythematous, indurated plaque on hypogastric region of abdomen with irregular borders, heliotrope rash, violaceous rash on face, and Gottron’s papules. | Amorphous mucinous material in epidermis and dermis which stained with alcian blue. The mucinous deposition caused thickening of the dermis and separation of collagen fibers | None | 80 mg oral prednisone and 120 mg oral azathioprine daily for 4 weeks with slow taper improved malaise and weakness however only partial improvement of the abdominal mucinous plaque |
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Kaufmann et al., 1998 [1]
Plaque like mucinosis | Case 1: 65 females | Case 1: erythematous plaques on extensor surface of upper extremities and left hip, muscle weakness of shoulder, and pelvic girdle | Case 1: separation of collagen bundles with perivascular lymphoplasmacytic infiltrate with abundant mucin deposition in the papillary and reticular dermis on staining with alcian blue | Case 1: none | Case 1: 80 mg oral prednisone daily, and 100 mg azathioprine daily with clinical response within 3 weeks, full resolution at 1 year |
| Case 2: 37 females | Case 2: erythematous plaques over lateral surface of thighs bilaterally, weakness in shoulder and pelvic girdle, and clinical picture developed after fever arthralgia and malaise | Case 2: skin biopsy-separation of collagen vascular bundles with superficial and deep perivascular lymphoplasmacytic infiltrate of dermis with mucin between separated collagen bundles on Alcian blue staining | Case 2: obesity, history of autoimmune thyroiditis, preceded by viral prodrome. No malignancy detected | Case 2: 2 days of IVIG and high dose prednisone and then azathioprine, with excellent response |
Del Pozo et al., 2001 [2]
DM with cutaneous mucinosis | Case 1: 53 females | Case 1: small violaceous papules on upper extremities and chest; proximal muscle weakness | Case 1: skin biopsy showed hyperkeratosis, colloid bodies, and edema at the dermoepidermal junction with moderate perivascular lymphocytic infiltrate. Collagen bundles separated by mucin deposition | Case 1: ovarian adenocarcinoma | Case 1: recurrence despite prednisone, hydroxychloroquine, methotrexate, and azathioprine. Ovarian carcinoma treated with surgery, paclitaxel and cisplatin, no comment on status of DM or cutaneous findings after treatment |
| Case 2: 44 females | Case 2: flesh coloured papules across flexural creases of palms and fingers | Case 2: skin biopsy-epidermal atrophy with perivascular lymphocytic infiltrate and moderate mucin deposition between collagen fibers | Case 2: none | Case 2: mucinosis developed after treatment for DM with 30 mg prednisone po and 250 mg hydroxychloroquine daily, lesions did not improve with this treatment |
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Tan et al., 2003 [7]
DM with cutaneous mucinosis | 65 Chinese males | Nontender erythematous plaques on neck, upper back and extensor surfaces of forearms bilaterally.
Proximal muscle weakness developed 3 months after initial presentation | Mucin deposition between collagen bundles with surrounding superficial perivascular lymphocytic infiltrate without epidermal changes | Nasopharyngeal carcinoma | Skin lesions resolved after 2 months of radiation therapy |
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Chen et al., 2005 [10]
Dermatomyositis with mucinosis and intestinal vasculopathy | 21 Taiwanese females | Erythematous indurated mass on lower abdomen, labia majora and inner thigh with malar rash and periungual telangiectasia, heliotrope rash, Gottron’s papules, proximal muscle weakness, and dysphagia | Atrophic epidermis with vacuolar alteration of basal keratinocytes, interstitial mucin deposition, and perivascular lymphocytic infiltrate of dermis and subcutaneous tissue | None | Prednisolone 60 mg/d IV with resolution of CPK however developed dysphagia and pulse steroid therapy initiated. Patient suffered complications and ultimately died after a long hospital course |
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Edward et al., 2007 [11]
Amyopathic DM with mucinosis | 31-year-old female | Puritic rash on flexor surface of forearms and chest, violaceous rash of face, Gottron’s papules, and nail fold telangiectasia | Dermal accumulation of mucin without inflammatory infiltrate; no evidence of inflammatory myopathy | None | Improved with oral steroids |
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Wang, 2011 [12]
DM with lichen myxedematosus | 60-year-old male | Violaceous erythema on face, neck, and chest with flesh coloured papules on arms; muscle weakness of shoulders and dysphagia | Separation of collagen bundles with lymphocytic infiltrate of dermis and mucin deposition between collagen bundles demonstrated with alcian blue stain | None | Oral prednisone 40 mg daily with good response |
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