Giant cell granuloma anular elastolytic L92.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 05.11.2021

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Synonym(s)

Actinic granuloma; AERZG; Annular elastolytic giant cell granuloma; Anular elastolytic giant cell granuloma; Giant elastolytic giant cellular granuloma

History
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O'Brien 1975; Hanke, 1979

Definition
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Rare granulomatous inflammation of unclear etiopathogenesis, with frequent predilection for sun-exposed areas.

Etiopathogenesis
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Phagocytotic granulomatous inflammation induced and maintained by UV-altered elastic (or elastotic) fibrous material of the skin. Relation or even identity with actinic granuloma is discussed. The etiological relationship with the repeatedly reported comorbidities is still unclear.

Manifestation
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Occurs in middle age, usually after the 3rd or 4th decade.

Localization
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Areas exposed to light: Mainly head or neck, rarely also generalized and therefore not exposed to light.

Clinical features
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Either solitary, more rarely multiple, 0.3 cm to 5 cm large, occasionally moderately itchy, mostly anular foci with raised, firm, deep red edges and central atrophy and fading. In addition to anular, confluence can also lead to circine formations.

Laboratory
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Useful to rule out comorbidity. Otherwise, not conclusive.

Histology
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Granulomatous, diffuse, lympho-histiocytic or also lympho-plasmacellular infiltrate in the upper and middle dermis with many giant multinuclear cells of the foreign body type. Loss of elastic fibers due to phagocytosis by the multinuclear giant cells. Detection of fragmented elastic fibres in the cells.

Differential diagnosis
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Clinic: Actinic granuloma: Probably closely related or identical clinical picture. Sarcoidosis: Clinical relation when occurring in the facial region present; no system involvement in AERZG; histology in sarcoidosis clearly different ("naked granulomas"). Necrobiosis lipoidica: localization different, mainly lower leg. No fibrosis, no necrosis zones. Granuloma anulare: localization in the area of the sun-exposed areas atypical for Granuloma anulare; histology is evidence of Granuloma anulare.

Complication(s)
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Comorbities with arteritis temporalis, diabetes mellitus, vitiligo, malignant hematological diseases (B-cell lymphoma, T-cell lymphoma, chronic lymphocytic leukemia, paraproteinemia) have been described several times and should be excluded clinically.

External therapy
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Glucocorticoids should first be applied as a cream, e.g. 0.1% Triamcinolon Cream R259, 0.1% Mometasone (e.g. Ecural Fat Cream), 0.25% Prednicarbate (e.g. Dermatop Cream). If unsuccessful, use intralesional glucocorticoid infiltration with triamcinolone crystal suspension (e.g. Volon A 1:3 diluted with LA, e.g. Scandicain). Only moderate results are described below.

Internal therapy
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  • In cases of severe suffering, try therapy with chloroquine (e.g. Resochin, 125-250 mg/day for about 3 months.
  • Alternatively DADPS (e.g. Dapson Fatol) 100 mg/day for at least 6-8 weeks, later maintenance therapy with approx. 50 mg/day p.o.
  • Alternatively clofazimine (e.g. Lamprene) initially 50-100 mg/day, maintenance therapy with 50 mg/day. With TD of 50 mg practically free of NW.
  • Successful in individual case reports: trial with fumaric acid esters.
  • The treatment of a facultative basic disease (e.g. B-cell lymphoma) does not necessarily lead to an improvement of the symptoms.

Operative therapie
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For circumscribed lesions, excision or cryosurgery (carbonic acid snow or liquid nitrogen).

Progression/forecast
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Benign, prognosis may depend on possible comorbidities. Otherwise, the course may last for years (possibly decades).

Note(s)
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Malignant, in particular hematological diseases (e.g. B-cell lymphomas, chronic lymphatic leukaemia) have repeatedly been reported as comorbidities. In addition, comorbidities have been reported casuistically for necrobiosis lipoidica, arteritis temporalis and diabetes mellitus.

Literature
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  1. Doulaveri G et al (2003) Annular elastolytic giant cell granuloma in a 70-year-old woman. Int J Dermatol 42: 290-291
  2. Dowling GB, Jones EW (1967) Atypical (annular) necrobiosis lipoidica of the face and scalp. A report of the clinical and histological features of 7 cases. Dermatologica 135: 11-26
  3. Fujimura T et al (2003) Disseminated papular interstitial elastolytic giant cell granuloma. Acta Derm Venereol 83: 234-235
  4. Gutzmer R et al (2002) Successful therapy of annular elastolytic giant cell granuloma with fumaric acid esters. Dermatology 205: 421-424
  5. Hanke CW et al (1979) Annular elastolytic giant cell granuloma. A clinicopathologic study of five cases and a review of similar entities. J Am Acad Dermatol 1: 413-421
  6. Hermes B, Haas N, Czarnetzki BM (1995) Annular elastolytic giant cell granuloma with a spontaneous healing tendency. dermatologist 46: 490-493
  7. Hohenleutner S et al (1997) Elastolysis of the mid-dermis and annular elastolytic giant cell granuloma: different stages in the clinical spectrum of dermal elastolysis? Case report and review of the literature: dermatologist 48: 45-50
  8. Kowalzick L et al (2012) Generalized giant cellular elastolytic anular granuloma in high malignant B-cell lymphoma. Act Dermatol 38: 173-176
  9. Mielke V et al (1995) Reticular elastolytic giant cell granuloma. A variant of the annular elastolytic giant cell granuloma. dermatologist 46: 259-262
  10. Vehring KH et al. (1991) The giant cell granuloma of the anular elastolytic system-a differential diagnosis of cutaneous granulomatosis. dermatologist 42: 391-395

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Last updated on: 05.11.2021