Granulomatosis disciformis chronica et progressiva L92.1

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 14.06.2022

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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Controversial and apparently rare, chronic, granulomatous (non-necrobiotic) disease whose entity is controversially discussed. An affiliation to necrobiosis lipoidica is often assumed (minus variant of necrobiosis lipoidica).

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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.

Clinical features
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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) Granulomatosis disciformis Miescher. dermatologist 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) Granulomatosis (tuberculoides) pseudosclerodermiformis symmetrica chronica. Arch Dermatol Syph 172: 142
Miescher G, Lederer M (1948) Granulomatosis disciformis chronica et progressiva (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. On Bras Dermatol 92:438-439.