Giant cell granuloma anular elastolytic L92.1

O'Brien 1975; Hanke, 1979

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

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.

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

Light-exposed areas: Mainly head or neck, rarely generalized and thus non-light-exposed.

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.

Useful to rule out comorbidity. Otherwise, not conclusive.

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.

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.

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.

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.

In case of pronounced suffering, therapy trial 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) 50-100 mg/day initially, maintenance therapy at 50 mg/day. At TD of 50 mg virtually free of NW.

Successful in single case reports: trial with fumaric acid esters.

Treatment of a facultative underlying disease (e.g., B-cell lymphoma) does not necessarily improve symptoms.

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

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

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.

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