Granulomatosis disciformis chronica et progressiva - Altmeyers Encyclopedia

Synonym(s)

facial necrobiosis Miserable; Granulomatosis pseudosclerodermiformis symmetrica chronica (Gottron); Granulomatosis tuberculoides pseudosclerodermiformis; macular necrobiosis; Miescher Granulomatosis; Miescher`s granulomatosis; necrobiosis lipoidica granulomatosa

History
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Gottron, 1935; Miescher and Lederer, 1948

Definition
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A controversial and apparently rare, chronic, non-necrobiotic granulomatosis whose clinical entity is the subject of debate. It is widely assumed to be a form of necrobiosis lipoidica (a milder variant of necrobiosis lipoidica).

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Dermatology

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Etiopathogenesis
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Unknown, trauma, a diabetic metabolic state is not always present in this sepcific granulomatosis (Oliveira ARFM et al. 2017).

Manifestation
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Middle adulthood; predominantly in women (note: the clinical data available in the literature are insufficient in this respect).

Localization
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Mainly lower leg extension sides, back of the foot, but also described in the face and neck.

Clinic
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Slowly centrifugally growing, 1.0-10.0 cm in size, asymptomatic, flatly raised or barely palpable, bizarrely configured, reddish-brown or light brown plaques (or spots) with partly yellowish parts; telangiectasia, atrophic surface; tendency to confluence.

Histology
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Perivascular epithelial cell granulomas with few giant cells. In contrast to Necrobiosis lipoidica, there are hardly any necrobiosis foci.

Differential diagnosis
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Necrobiosis lipoidica; granuloma anulare; sarcoidosis; circumscribed scleroderma.

External therapy
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Glucocorticoid-containing external preparations (e.g. 0.1% triamcinolone cream R259 or 0.1% betamethasone ointment) and foil dressings, injection of glucocorticoid crystal suspension intrafocally (e.g. Volon A 10 mg, 1:3-1:5 diluted with the local anaesthetic scandicain). For small foci excision.

Progression/forecast
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Mostly chronic progressive course; very rarely spontaneous healing.

Literature
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Almond-Roesler B et al. (1998) Miescher's granulomatosis disciformis. Hautarzt 49: 228–230
Bergler-Czop B et al. (2009) Miescher’s granulomatosis (granulomatosis disciformis chronica et progressiva) in a non-diabetic patient—case report. Diagn Pathol 4:28
Gottron HA (1935) Symmetrical chronic pseudosclerodermiform granulomatosis (tuberculoides). Arch Dermatol Syph 172: 142
Miescher G, Lederer M (1948) Chronic and progressive disciform granulomatosis (atypical tuberculosis). Dermatologica (Basel) 97: 25–34
Miescher G (1949) Necrobiosis maculosa. Dermatologica (Basel) 98: 199-204
Oliveira ARFM et al. (2017) Granulomatosis disciformis in a non-diabetic patient. An Bras Dermatol 92:438-439.