HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Rare, gynaecotropic, granulomatous systemic disease of unknown cause, which manifests itself on the integument and here preferably on the lower legs in the form of large, brown-red plaques which, if persisting for a long time, may also be ulcerated and then painful. An association with diabetes mellitus is present in about 65% of patients.
Granulomatosis disciformis chronica et progressiva is seen by some authors as a special form, by others as a differential diagnosis.
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Occurrence/EpidemiologyThis section has been translated automatically.
More common in diabetes mellitus patients. Frequency in diabetics: 3:1,000 patients.
In larger collectives the following comorbidities are listed, although pathogenetic relationships cannot be postulated from them:
- Diabetes mellitus type I (E10) 20.6%
- Diabetes mellitus type II (E11) 13.7%
- Essential hypertension (I10) 9.2%
- Crural ulcer (L97): 7.3%
- Other venous diseases, varices of the lower limbs (I87/3): 5.7%
- Obesity (E66): 4,6%
- Heart failure (I50): 4.1%.
- disorders of lipoprotein metabolism (E78): 2.3%.
- psoriasis (L40): 2,3%.
- Erysipelas (A46): 2,3%
- Multiple sclerosis (G35): 1,9%
EtiopathogenesisThis section has been translated automatically.
Systemic granulomatosis of unexplained etiology.
Discussed are vascular changes ( microangiopathy, fibronectin and factor 8 associated antigen elevation, disturbances in prostaglandin synthesis), changes in sweat glands, collagen disorders, immune mechanisms, disturbed leukocyte functions. Occasionally the occurrence in older scars has been reported (see fig.).
ManifestationThis section has been translated automatically.
Mainly occurring in middle age (50-55 years). Approximately 10% of those affected are in the age groups 75-80 and 15-20 years. Women are affected 2-3 times more frequently than men (recent figures show a ratio of 6:4).
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
Frequently occurring after a (often not remembered) minor trauma, irregularly configured, sharply demarcated, atrophic, indurated plaque with a yellow to brownish-yellow, speckly shiny center, interspersed with telangiectasias. The initial focus is barely the size of a coin, clinically unremarkable, and often misrecognized as a traumatic hemosiderin deposit.
However, a reddish-purple to brown-red rim indicates ongoing inflammatory activity and peripheral growth.
The focus(s) show gradual centrifugal growth over months and years. The subcutis may also be involved in granulomatous inflammation. This deep component results in atrophy of the subcutaneous adipose tissue with depression of the atrophic surface. This results in increased vulnerability of the foci. Confluence of multiple foci is possible, resulting in map-like plaques (see Fig.).
In about 30% of cases, poorly healing, painful ulcers with a yellowish speckled, necrotic base develop in the lesions, following trivial trauma; depending on the localization (e.g., tibial edge), (painful) concomitant periostitis may also occur.
Healing with atrophy of skin and subcutaneous fat with loss of skin appendages is possible.
HistologyThis section has been translated automatically.
Epidermis thinned, otherwise without specific changes. In the middle and lower dermis, sometimes also in the subcutaneous adipose tissue, there are nodular or striated granulomatous inflammatory zones consisting of histiocytes, epithelioid cells and multinucleated giant cells of varying composition, as well as focal nests of lymphocytes and plasma cells.
Sporadic formation of lymphoid follicles. In some cases plasma cells may dominate the picture.
In addition to this inflammatory component, the cutis is characterized by extensive degenerative changes of the collagen (collagen necrobiosis). The zones of necrobiosis may occur in the midst of a granulomatous nodule, but may also alternate with granuloma zones in a sandwich-like fashion.
Wall-thickened or occluded vessels may be consistently detectable. Granulomatous inflammation may involve the subcutaneous fat tissue.
The extent to which granulomatosis disciformis chronica et progressiva represents an independent entity remains to be discussed.
DiagnosisThis section has been translated automatically.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
A plethora of different therapeutic proposals exist, which on the other hand means that there is no such thing as "the promising form of therapy". The treatment has to be adapted to the local situation (age of the lesion, large or small foci, ulcerated or non-ulcerated) and to a possible underlying systemic disease (e.g. diabetes mellitus). Important: Any treatment approach requires patience from both physician and patient. It is to be applied for years.
General therapyThis section has been translated automatically.
Treatment of the underlying disease, e.g. diabetes mellitus (adjustment of blood sugar!), hypertension, Crohn's disease (see Enteritis regionalis), ulcerative colitis. As a rule, however, treatment of the underlying disease does not improve the cutaneous symptoms.
In the case of localization on the lower legs, therapy should always be accompanied by consistent compression therapy, since this relatively often prevents progression and promotes healing (always perform arterial Doppler sonography before compression therapy).
External therapyThis section has been translated automatically.
- Non-ulcerated skin lesions:
- Therapy of choice are topical glucocorticoids, e.g. Mometasone (Ecural®) under occlusion. Permanent wearing, change once/day for 14 days up to 4 weeks.
- In a case report healing of the NL was reported under a local therapy with 0,1% tacrolimus (Protopic®)
- Ulcerated lesions:
- Ulcer-adapted wound therapy. .
Internal therapyThis section has been translated automatically.
- In the case of ulcerations or highly inflammatory components, systemic therapy with glucocorticoids in medium dosages such as prednisolone 60-80 mg/day (e.g. Decortin H) for one week, then 30 mg/day for one month. Cave! Monitoring the blood sugar level! After healing of the ulcers, transition to a non-steroidal alternative therapy (e.g. fumaric acid ester or TNF-alpha-blocker).
- Alternatively: fumaric acid ester in the therapy modality indicated for psoriasis (see below psoriasis vulgaris).
- Alternative: Ciclosporin A systemic: initial 2.5-7.5 mg/kg bw/day p.o., after one month gradual reduction to a maintenance dose of 1-2.5 mg/kg bw/day. By determining the blood plasma level, an optimal effective level of 100-200 ng/ml can be set (blood sample in the morning, before taking the preparation!)
- Alternative: Therapy trial with the TNF-alpha-blocker Adalimumab (initially 80mg s.c., 1 week later 40mg s.c.; maintenance therapy 40mg s.c. every 14 days).
- Alternative: Mycophenolatmofetil systemic: Initial 2,5-7,5 mg/kg bw/day p.o. As long-term therapy Mycophenolatmofetil monotherapy with 1,0 g/2mal/day p.o.
- Alternative or additive: Pentoxifyllin (e.g. Trental) 3 times/day 400 mg p.o. or Nicotinamide (e.g. Nicobion 3 times/day 500 mg p.o. can be tried supportively. In persistent cases acetylsalicylic acid 1.5-4.5 g/day p.o., dipyridamole (Curantyl) 225 mg/day p.o. individually or in combination (Aggrenox)..,
- Experimental: Assuming that tissue hypoxia is a major cause of the disease, experimental approaches try to achieve improvements by oxygenation of the blood.
Progression/forecastThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Aslan E et al (2007) Successful therapy of an excruciated necrobiosis lipoidica non diabeticorum with Ciclosporin. Dermatologist 58: 684-685
- Aye M et al (2002) Dermatological care of the diabetic foot. Am J Clin Dermatol 3: 463-474
- Barth D et al. (2011) Topical application of Tacrolimus for Necrobiosis lipoidica. Dermatologist 62: 459-462
- Beattie PE et al (2006) UVA1 phototherapy for treatment of necrobiosis lipoidica. Clin Exp Dermatol 31: 235-238
- De Rie MA et al (2002) Treatment of necrobiosis lipoidica with topical psoralen plus ultraviolet A. Br J Dermatol 147: 743-747
- Dwyer CM et al (1993) Ulceration in necrobiosis lipoidica - a case report and study. Clin Exp Dermatol 18: 366-369
- Gambichler T et al (2003) Clearance of Necrobiosis lipoidica with Fumaric Acid Esters. Dermatology 207: 422-424
- Jockenhöfer F et al (2016) Cofactors and comorbidities in necrobiosis lipoidica - Analysis of the detuscehn DRG data from 2012 JDDG 13: 277-285
- Kreuter A et al (2005) Fumaric acid esters in necrobiosis lipoidica: results of a prospective noncontrolled study. Br J Dermatol 153::802-807
- Leister L et al (2013) Successful treatment of a patient with refractory, exulcerated necrobiosis lipoidica non diabeticorum. Dermatologist 64: 509-511
- Marcoval J et al (2015) Necrobiosis lipoidica: a descriptive study of 35 cases. Actas Dermosifiliogr 106: 402-407
- Oppenheim M (1929) Peculiar disseminated degeneration of connective tissue in a diabetic. Zentralbl Haut- und Geschlechtskrankh (Berlin) 32: 179
- Oppenheim M (1932) A skin disease not yet described in Diabetess mellitus (Dermatitis atrophicans lipoides diabetica) Wiener klin Weekly news 45: 314-315
- Pătraşcu V et al. (2014) Ulcerated necrobiosis lipoidica to a teenager with diabetes mellitus and obesity. Rome J Morphol Embryol 55:171-176
- Tokura Y et al (2003) Necrobiosis lipoidica of the glans penis. J Am Acad Dermatol 49: 921-924
- Urbach E (1932) Contributions to a physiological and pathological chemistry of the skin. X. A new diabetic metabolic dermatosis. Necrobiosis lipoidica diabeticorum. Arch Dermatol Syphil (Berlin) 166: 273
- Thomas M et al (2013) Necrobiosis lipoidica: A clinicopathological study in the Indian scenario. Indian Dermatol Online J 4:288-291
- 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.
Incoming links (28)Adiposity skin changes; Atrophie blanche; Collagenosis reactive perforating; CVID; Dermadrome; Dermatitis atrophicans lipoides diabetica; Dermatoliposclerosis; Diabetic necrosis lipoidica; Dyslipoidosis cutanea; Giant cell granuloma anular elastolytic; ... Show all
Outgoing links (28)Acetylsalicylic acid; Adalimumab; Atrophy of the skin (overview); Chronic venous insufficiency (overview); Ciclosporin a; Circumscripts of scleroderma (overview); Compression therapy; Crohn disease, skin alterations; Glucocorticosteroids; Glucocorticosteroids systemic; ... Show all
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