Granulomatous interstitial Dermatitis with arthritis M30.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 01.03.2022

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Synonym(s)

Churg-Strauss Granulomas; IGDA; Interstitial granulomatous dermatitis; Interstitial granulomatous dermatitis with arthritis

History
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Dykman, 1965; Ackerman AB, 1997

Definition
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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.

Etiopathogenesis
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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.

Localization
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The trunk, upper extremities and buttocks are mainly affected.

Clinical features
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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.

Histology
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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.

Histopathological algorithm of interstitial granulomatous dermatitis (lowest common denominator: italic, leading symptoms:bold) varies according to Ratzinger et al. 2105
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 diagnosis
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Clinical:

Lyme disease: Cutaneous Lyme disease

(possibly with an interstitial granulomatous dermatitis). Clinical-serological clarification!

Atrophodermia progressiva idiopathica (Pasini-Pierini)

: A similar clinical picture may be present. A stranded arrangement of the erythema is untypical. A "pseudoatrophy" of the surface is always detectable.

Granuloma anulare

(disseminatum): Clinically untypical forms of the granuloma anulare may cause difficulties in the clinical delimitation (if they can be delimited at all).

Cutaneous T-cell lymphoma

: Early (pre-mycoside) forms of CTCL may present an analogous clinical picture with a slightly scaly, symptomless red, flat plaque. Histological:

Lyme disease: Rare manifestation of cutaneous Lyme disease

with interstitial granulomatous dermatitis. In this case there are mostly plasmacellular accompanying components. Clinical clarification!

Morphea: The early stage of morphea

may (although less frequently) have a histiocytic component which gives way to dermatosclerosis in a later stage.

Granuloma anulare

: In the case of interstitial (incomplete) granuloma anulare, "mucin deposits" are also detected.

Erythema elevatum diutinum

: An eminently chronic clinical picture which shows signs of vasculitis, particularly in the early stages. Later stages can make it difficult to differentiate between the two.

Granulomatous cutaneous T-cell lymphoma

(granulomatous mycosis fungoides): CD4+ small to medium T-cell proliferation predominates. Mostly numerous eosinphil granulocytes are present. The detection of a T-cell clone is an important component in the diagnosis of lymphoma.

Complication(s)
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Possible causes include rheumatoid arthritis, Hashimoto's thyroiditis, diabetes mellitus, vitiligo, systemic lupus erythematosus.

Therapy
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Treatment of the underlying disease. Good success was achieved in individual cases with Infliximab.

Note(s)
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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)
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  • 59-year-old patient who had been observing 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 as well as in the area of the posterior axillary line and the proximal upper arms. In the meantime, these strands blistered off and left striped hyperpigmentation. In addition, the patient had been complaining for years about intermittent joint pain in the finger, foot and knee joints.
  • Clinic: Up to 2.0 cm large, flat, partly skin-coloured, partly reddish, firm, partly ulcerated and encrusted, partly confluent papules on the elbows; about 30 cm long and 0.6 cm wide, reddish, coarse, painless plaque in the posterior axillary line with overlapping on the proximal upper arm. In the area of the left posterior axillary line a slightly shorter and less prominent similar strand appeared.
  • 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.

Literature
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  1. Dykmann CJ et al (1965) Linear sucutaneous bands in rheumatoid arthritis. Ann Internal Med 63: 134-140
  2. 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
  3. Harpster EF et al (1989) Linear granuloma annulare. J Am Acad Dermatol 29:1138-1141
  4. Jorizzo JL et al (1983) Dermatological conditions reported in patients with rheumatoid arthritis. J Am Acad Dermatol 8: 439-457
  5. Ratzinger G et al. (2015) The Vasculitis Wheel-an algorithmic approach to cutaneous vasculitis. J Dtsch Dermatol Ges 13: 1092-1118
  6. Rose C et al(2017) Granulomatous reaction pattern of the skin: Interstitial granulomatous dermatitis - lymphoma - vasculitis. Dermatologist 68:553-559.
  7. Sanchez JL et al (1990) Rheumatoid neutrophilic dermatitis. J Am Acad Dermatol 22: 922-925
  8. Tomasini C et al (2002) Interstitial granulomatous dermatitis with plaques. J Am Acad Dermatol 46: 892-899

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Last updated on: 01.03.2022