Granuloma anulare (overview) L92.-

Authors: Prof. Dr. med. Peter Altmeyer, Dr. med. Gesa Hansen-Prenz

All authors of this article

Last updated on: 18.12.2020

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Synonym(s)

annular granuloma; Pseudorheumatoid nodules

History
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Calcott Fox, 1895

Definition
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Asymptomatic, chronic, necrobiotic, granulomatous inflammation of the skin (also subcutaneous), with formation of skin-coloured or slightly reddened papules and plaques, which develop into characteristic anular outline formations of varying size. Clinically, different clinical pictures, with different clinical morphology and different prognosis are presented, which only the common histopathological substrate (necrobiotic collagen degeneration with radiating histiocytic inflammatory reaction: palisade granulomas) connects.

Classification
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Clinical variants of the anular granuloma:

Occurrence/Epidemiology
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Rare disease. Exact incidence figures are not available. In large dermatological centres 0.1-0.5% of all patients are diagnosed with anular granuloma. Individual case reports of familial occurrence are known.

Etiopathogenesis
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Etiology and pathomechanism are unknown. Hypothetically were considered:

  • delayed type IV immune response to focal connective tissue aging
  • trauma-induced necrobiosis
  • malignant neoplasias
  • diabetes mellitus, dyslipoproteinemia, autoimmune thyroiditis
  • Infection triggering through various Pathogens (especially viruses)
  • Drugs

For further details see below Granuloma anulare classic type

Manifestation
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Preferred among children and adolescents and young adults. In 2/3 of all patients the disease begins before the 30th year of life f>m=2:1. casuistic reports of a family clustering (Weidenthaler-Barth B 2017).

Clinical features
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In the classic and most common granuloma anulare, there is a solitary, or a few, circular or ring-shaped, completely asymptomatic ring structures that can reach a diameter of 1.0 to 3.0 cm. These rings are composed of smaller, aggregated, firm, surface-smooth nodules and plaques. Their growth is slow and can last for many months. When 2 ring structures meet, arcuate ornaments result.

Typically, the single efflorescence of classic granuloma anulare does not exceed a maximum size of 5.0 cm.

In rare cases, however, an extreme extension up to 10.0 cm may occur (granuloma anulare giganteum), without any explanation in individual cases.

Number of lesions: 85% of patients have less than 10 foci. Rarer is the exanthematous granuloma anulare disseminatum, which occurs in episodes and is also asymptomatic, with multiple smaller and often only suggestive anular plaques.

Perforating granuloma anulare (granuloma anulare perforans) shows central umbilication with crust formation.

The extent to which granulomatosis disciformis chronica et progressiva (Miescher's granuloma) has a special clinical status is still under discussion.

Histology
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Circumscribed nodular sites of inflammation in the upper and middle dermis (rarely located in subcutaneous fatty tissue) with central necrobiosis and throughput with nuclear fragments and mucin. Characteristic is an enclosing palisade granuloma consisting of lymphocytes, macrophages (histiocytes), fibroblasts and more rarely of multinuclear giant cells. Occasionally also admixtures of eosinophilic leukocytes and plasma cells. With the granuloma anulare of the interstitial type, less nodular but rather diffuse interstitial infiltrates of CD68-positive histiocytes and lymphocytes around alcian blue-positive mucin deposits in the reticular dermis are observed. Here the necrobiosis is missing. In the rare form of perforating anular granuloma, degenerated connective tissue is ejected.

Therapy
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See below the individual clinical pictures.

Progression/forecast
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Wide range of variation in the duration of stocks. Spontaneous healing within the first two years is expected in about 60% of patients. In children the spontaneous remission rate is 80-90%. High local recurrence rate (about 40%).

Literature
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  1. Butsch F et al (2015) Granuloma anulare. Dermatologist 66: 867-87
  2. Boyoung Lee S et al (2016) Vemurafenib-induced granuloma anulare. JDDG 14: 305-308
  3. Kowalski L et al (20169 Vesicular granuloma anulare under etanercept or abatacept therapy. Nude Dermatol 42:38-40
  4. Weidenthaler-Barth B (2017) Clinical and histological spectrum of palisaded granulomatous dermatitides:Granuloma annulare, necrobiosis lipoidica, rheumatoid nodules, and necrobiotic xanthogranuloma. Dermatologist 68:536-541.

See below the individual clinical pictures

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.