Necrobiosis lipoidica L92.1

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Dr. med. Eva Kämmerer

All authors of this article

Last updated on: 23.02.2021

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Synonym(s)

dermatitis atrophicans lipoides diabetica; diabetic necrosis lipoidica; Dyslipoidosis cutanea (Oppenheim-Urbach); Granulomatosis disciformis chronica et progressiva; necrobiosis lipoidica; necrobiosis lipoidica diabeticorum; Necrobiosis lipoidica non diabeticorum; Oppenheim-Urbach Syndrome; Urbach Syndrome

History
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Oppenheim, 1929; Urbach, 1932

Definition
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Rare, gynecotropic, granulomatous systemic disease of unknown cause, which manifests itself on the integument and here preferably on the lower legs in the form of large-area painful plaques, which may also be ulcerated if persisting for a long time. 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.

Occurrence/Epidemiology
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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:

  1. Diabetes mellitus type I (E10) 20.6%
  2. Diabetes mellitus type II (E11) 13.7%
  3. Essential hypertension (I10) 9.2%
  4. Crural ulcer (L97): 7.3%
  5. Other venous diseases, varices of the lower limbs (I87/3): 5.7%
  6. Obesity (E66): 4,6%
  7. Heart failure (I50): 4.1%.
  8. disorders of lipoprotein metabolism (E78): 2.3%.
  9. psoriasis (L40): 2,3%.
  10. Erysipelas (A46): 2,3%
  11. Multiple sclerosis (G35): 1,9%

Etiopathogenesis
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Systemic granulomatosis of unexplained etiology.

Associations with diabetes mellitus (65%), hypertension, Crohn's disease, ulcerative colitis, granuloma anulare or cutaneous sarcoidosis have been described.

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.).

Manifestation
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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).

Localization
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Mainly lower leg extension sides, back of the foot, ankle region, in 15% other body regions. First unilateral, then often symmetrical manifestation.

Clinical features
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Irregularly configured, sharply defined, plate-like, atrophic, indurated plaque with a yellow to brownish yellow, bacon-like shiny center, often occurring after a minor trauma (usually not remembered) . A reddish-violet to brownish-red rim indicates persistent inflammatory activity and peripheral growth. The foci show gradual centrifugal growth over months and years. The subcutis may also be involved in granulomatous inflammation. This deep component leads to atrophy of the subcutaneous fatty tissue with surface subsidence. Confluence of several foci possible.

In about 30% of cases, lesions, after banal trauma, lead to the formation of poorly healing ulcers with a yellowish, speckled, necrotic base; depending on the location, (painful) accompanying periostitis may also occur.

Healing with atrophy of the skin and loss of skin appendages is possible.

Special forms: Necrobiosis lipoidica maculosa disseminata, necrobiosis lipoidica in the forehead and head area. In the area of the hairy head (extremely rare localization) formation of a pseudopelace.

Histology
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Epidermis without specific changes. In the middle and lower dermis, partly also in the subcutaneous fatty tissue, nodular or striated granulomatous inflammatory zones consisting of histiocytes, epithelioid cells and multinucleated giant cells of different composition as well as focal nests of lymphocytes and plasma cells are found. Occasionally also formation of lymph follicles. In some cases plasma cells may dominate the picture. In addition to this inflammatory component, extensive degenerative changes in collagen can occur (collagen necrobiosis). The necrobiosis zones can occur in the middle of a granulomatous nodule, but can also alternate sandwich-like with granuloma zones. Wall thickened or blocked vessels can always be detected. The granulomatous inflammation can also affect the subcutaneous fatty tissue.

The extent to which granulomatosis disciformis chronica et progressiva represents an independent entity remains to be discussed.

Diagnosis
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Clinic, histology, serum glucose and glucose stress test.

Differential diagnosis
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Therapy
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  • There is a wealth of different therapy proposals, which on the other hand means that "the promising form of therapy" does not exist. The treatment must be adapted to the local situation (age of the lesion, large or small, ulcerated or not ulcerated) and to a possible underlying systemic disease (e.g. diabetes mellitus). Important: Every treatment approach requires patience from both doctor and patient. It should be applied for years.

General therapy
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  • 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 show any improvement in necrobiosis.
  • In the case of localisation on the lower legs, a consistent compression therapy should always be carried out in addition to therapy, as this relatively often prevents progression and promotes healing (always carry out arterial Doppler sonography before compression therapy).

External therapy
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  • 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®)
    • Alternative:
      • Injectglucocorticoids intrafocally, once a month for 2-3 months, e.g. triamcinolone acetonide (e.g. Volon A 10 1:1 with scandicain). Caution: Painfulness; Compliance!
      • Good results were reported from PUVA therapy.
  • Ulcerated lesions:

Internal therapy
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  • 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/forecast
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Chronic course. Possible complications from ulceration. Spontaneous healing in 20% of cases with scarred healing.

Literature
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  1. Aslan E et al (2007) Successful therapy of an excruciated necrobiosis lipoidica non diabeticorum with Ciclosporin. Dermatologist 58: 684-685
  2. Aye M et al (2002) Dermatological care of the diabetic foot. Am J Clin Dermatol 3: 463-474
  3. Barth D et al. (2011) Topical application of Tacrolimus for Necrobiosis lipoidica. Dermatologist 62: 459-462
  4. Beattie PE et al (2006) UVA1 phototherapy for treatment of necrobiosis lipoidica. Clin Exp Dermatol 31: 235-238
  5. De Rie MA et al (2002) Treatment of necrobiosis lipoidica with topical psoralen plus ultraviolet A. Br J Dermatol 147: 743-747
  6. Dwyer CM et al (1993) Ulceration in necrobiosis lipoidica - a case report and study. Clin Exp Dermatol 18: 366-369
  7. Gambichler T et al (2003) Clearance of Necrobiosis lipoidica with Fumaric Acid Esters. Dermatology 207: 422-424
  8. Jockenhöfer F et al (2016) Cofactors and comorbidities in necrobiosis lipoidica - Analysis of the detuscehn DRG data from 2012 JDDG 13: 277-285
  9. Kreuter A et al (2005) Fumaric acid esters in necrobiosis lipoidica: results of a prospective noncontrolled study. Br J Dermatol 153::802-807
  10. Leister L et al (2013) Successful treatment of a patient with refractory, exulcerated necrobiosis lipoidica non diabeticorum. Dermatologist 64: 509-511
  11. Marcoval J et al (2015) Necrobiosis lipoidica: a descriptive study of 35 cases. Actas Dermosifiliogr 106: 402-407
  12. Oppenheim M (1929) Peculiar disseminated degeneration of connective tissue in a diabetic. Zentralbl Haut- und Geschlechtskrankh (Berlin) 32: 179
  13. Oppenheim M (1932) A skin disease not yet described in Diabetess mellitus (Dermatitis atrophicans lipoides diabetica) Wiener klin Weekly news 45: 314-315
  14. Pătraşcu V et al. (2014) Ulcerated necrobiosis lipoidica to a teenager with diabetes mellitus and obesity. Rome J Morphol Embryol 55:171-176
  15. Tokura Y et al (2003) Necrobiosis lipoidica of the glans penis. J Am Acad Dermatol 49: 921-924
  16. 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
  17. Thomas M et al (2013) Necrobiosis lipoidica: A clinicopathological study in the Indian scenario. Indian Dermatol Online J 4:288-291
  18. 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.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 23.02.2021