Skin-nontumor - Interstitial granulomatous dermatitis with arthritis

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Granulomatous but non-infectious disorders

Interstitial granulomatous dermatitis with arthritis

Reviewer: Mowafak Hamodat MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns (Canada) (see Reviewers page)
Revised: 26 October 2011, last major update June 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Definition
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● Uncommon; first described in 1993
● Linear inflammatory indurations on lateral aspects of trunk (“rope sign”), also plaques or papules associated with systemic connective tissue disease
● Usually in women
● Arthritis is usually symmetric and involves fingers, wrists, elbows and shoulders
● Associated with positive rheumatoid factor (not always), autoantibodies and elevated erythrocyte sedimentation rate

Treatment
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● Systemic corticosteroids
● Dapson was useful in patients with rheumatoid papules

Case reports
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● 34 year old woman with SLE and a skin rash (Case of Week #221)
● 54 year old woman with arthritis (Dermatol Online J 2009;15:22)
● Disease without rope sign (Hum Pathol 2004;35:892)

Clinical images
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Plaque on thigh

Micro description
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● Diffuse dense inflammatory infiltrate of histiocytes within reticular dermis, focal degenerated collagen and elastic fibers, palisading histiocytes
● Variable neutrophils, eosinophils, lymphocytes, mucin and vasculitis
● Less mucin than granuloma annulare
● Palisading of histiocytes and lymphocytes around a central zone of numerous neutrophils

Micro images
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Superficial and deep perivascular and interstitial infiltrate of lymphocytes, eosinophils and histiocytes; histiocytes are scattered between collagen bundles and associated with mucin deposits

Positive stains
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● Direct immunofluorescence (DIF) may demonstrate C3, IgM and fibrinogen in dermal blood vessels

Differential diagnosis
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● Granulomatous drug reactions: vacuolar interface changes, often epidermotrophism of lymphocytes; resolves when drug is stopped
● Rheumatoid dermatosis: extensive homogeneous necrobiosis with numerous giant cells and stromal fibrosis
● Interstitial granuloma annulare: mid dermal necrobiotic collagen center surrounded by palisading histiocytes, with fibroblasts and lymphocytes; occasional foreign body giant cells, vasculitis, mucin; may need multiple sections to find necrobiotic collagen
● Necrotizing granuloma: may be due to infectious microorganism

Additional references
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● Hum Pathol 2004;35:779 (editorial)

End of Skin-nontumor > Granulomatous but non-infectious disorders > Interstitial granulomatous dermatitis with arthritis

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