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Granulomatous interstitial Dermatitis with arthritisM30.1
Synonym(s)
HistoryThis section has been translated automatically.
Dykman, 1965; Ackerman AB, 1997
DefinitionThis section has been translated automatically.
Very rare vasculitic clinical picture occurring in association with diseases in which circulating immune complexes play a pathogenetic role. Clinically, symmetrical, red, usually asymptomatic erythema, papules or plaques are predominantly localized on the lateral trunk, also in a linear orientation (rope signs or "rope signals"). There are clinical similarities to urticarial vasculitis.
EtiopathogenesisThis section has been translated automatically.
The pathogenesis of the disease is not yet clear. Some authors assign it to the spectrum of interstitial granuloma anulare (Rose C 2017). Other authors consider it to be a late stage of vasculitis.
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
Eminently chronic, bilateral, symmetrical, up to about 3.0-20 cm long and 0.5-1.0 cm wide, coarse, painless, band-like erythema, also reddish papules and plaques (also rope signals) in the posterior or anterior axillary line, the chest, the buttocks are described as characteristic. These may also spread to the adjacent extremities.
Anular patterns are also found(erythema anulare-like).
Not infrequently, the changes occur together with rheumatoid arthritis.
HistologyThis section has been translated automatically.
Dense, band-shaped histiocytic infiltrate in the middle reticular dermis. Furthermore, there are "flame figure" type (but not to be confused with these), so-called "floating signs", mostly basophilic areas consisting of degenerated collagen. These are surrounded by rosette-like arranged, partly also pleomorphic histiocytes (some mitoses can also be detected). Furthermore, there are discrete perivascular lymphocytic infiltrates and some neutrophil and eosinophilic leukocytes.
| Accentuates around post-capillary venules and larger vessels in the skin and subcutis |
| Capillaries omitted or less strongly involved |
| perivascular leukocytoclasia |
| Damage to endothelial cells |
| Fibrin in/around vessel walls |
| Perivascular extravasation of erythrocytes |
| No/mild edema in the papillary dermis |
| Collagen degeneration with slight basophilic necrotic lesions of variable extent, surrounded by palisade granulomas |
| No significant number of eosinophils |
| No plasma cells or fibrosclerosis |
Differential diagnosisThis section has been translated automatically.
Clinical:
Lyme disease: Cutaneous Lyme disease
(possibly with interstitial granulomatous dermatitis). Clinical-serological clarification!Atrophodermia progressiva idiopathica (Pasini-Pierini)
: In this case, a similar clinical picture may be present in terms of color. Atypical would be a strand-like arrangement of the erythema. Always detectable is a "pseudoatrophy" of the surface. (disseminatum): Clinically atypical forms of granuloma anulare may cause difficulties in clinical differentiation (if they can be differentiated at all).: Early (premycotic) forms of CTCL may present an analogous clinical picture with a sparsely scaling, asymptomatic red, planar plaque. Histologic:Lyme disease: Rare manifestation of cutaneous Lyme disease
with interstitial granulomatous dermatitis. In this case, accompanying plasma cellular components are usually found. Clinical workup!Morphea: The early stage of morphea
may (although less frequently) have a histiocytic accompanying component, which gives way to dermatosclerosis at a later stage.Granuloma anulare: In interstitial (incomplete)granuloma
anulare, additional "mucin deposits" are detected.: An eminently chronic clinical picture, which shows signs of vasculitis especially in the early stages. Later stages may make differentiation difficult.Granulomatous cutaneous T-cell lymphoma
(granulomatous mycosis fungoides): CD4+ small- to medium-sized T-cell proliferation predominates. Usually numerous eosinphilic granulocytes are present. Here, the detection of a T-cell clone is an important component in the lymphoma diagnosis.
Complication(s)This section has been translated automatically.
Possible causes include rheumatoid arthritis, Hashimoto's thyroiditis, diabetes mellitus, vitiligo, systemic lupus erythematosus.
TherapyThis section has been translated automatically.
Treatment of the underlying disease. Good success was achieved in individual cases with Infliximab.
Note(s)This section has been translated automatically.
Interstitial granulomatous dermatitis is a rare disease whose entity is still controversial. The histological changes are unspecific. It is probably a reaction pattern that occurs in the context of different diseases, such as systemic vasculitides, collagenoses, rheumatoid arthritis, lymphoproliferative diseases, subacute bacterial endocarditis and chronic active hepatitis. These diseases have in common the presence of circulating immune complexes.
In 1951, Churg and Strauss described analogous changes in 13 patients with vasculitis as "allergic granulomas" in the skin ("Churg-Strauss granulomas").
Case report(s)This section has been translated automatically.
59-year-old patient who observed the appearance of skin-colored to reddish, partially ulcerated papules on both elbows for several years. One sister was diagnosed with chronic polyarthritis.
For about 3 months, additional appearance of reddish, painless strands on the lateral trunk and in the area of the posterior axillary line and proximal upper arms. In the meantime, these faded away and left streak-like hyperpigmentation. Furthermore, the patient complained of intermittent joint pain of the finger, foot and knee joints for years.
Clinical: Flat, partly skin-colored partly reddish, firm, partly ulcerated and crusted, partly confluent papules up to 2.0 cm in size on the elbows; about 30 cm long and 0.6 cm wide, reddish, rough, painless plaque in the posterior axillary line with spreading to the proximal upper arm. A slightly shorter and less prominent similar strand appeared in the left posterior axillary line.
Laboratory findings: LDH: 320 U/l (normal range:120-240 U/l), CRP: 7.5 mg/dl (normal range:< 0.5 mg/dl), iron: 10 mmol/l (normal range:14-32). Sm-AK: positive; nRNP-Ak: positive. Other routine laboratory parameters such as rheumatoid factor, ANA, ds-DNA-AK, SSA, SSB, borrelia serology showed no pathological findings.
LiteratureThis section has been translated automatically.
- Dykmann CJ et al (1965) Linear sucutaneous bands in rheumatoid arthritis. Ann Intern Med 63: 134-140
- Gottlieb GJ et al (1995) Interstitial granulomatous dermatitis with cutaneous cords and arthritis:linear subcutaneous bands in rheumatoid arthritis revisited. Dermatopathol Pract Concept 1: 3-69.
- Harpster EF et al (1989) Linear granuloma annulare. J Am Acad Dermatol 29:1138-1141.
- Jorizzo JL et al (1983) Dermatologic conditions reported in patients with rheumatoid arthritis. J Am Acad Dermatol 8: 439-457.
- Ratzinger G et al (2015) The vasculitis wheel-an algorithmic approach to cutaneous vasculitides. J Dtsch Dermatol Ges 13: 1092-1118.
- Rose C et al.(2017) Granulomatous reaction pattern of the skin: Interstitial granulomatous dermatitis - lymphoma - vasculitis. Dermatologist 68:553-559.
- Sanchez JL et al (1990) Rheumatoid neutrophilic dermatitis. J Am Acad Dermatol 22: 922-925.
- Tomasini C et al (2002) Interstitial granulomatous dermatitis with plaques. J Am Acad Dermatol 46: 892-899