Necrobiosis lipoidica is a rare skin condition seen more often in diabetic patients. Histopathology shows horizontal layers of granulomatous inflammation surrounding degenerated collagen. While the cause is unknown, it may involve vascular changes or collagen disease. Lesions typically appear on the lower legs as yellow plaques with red borders. First line treatments include topical steroids and tacrolimus, while second line includes phototherapy. Prognosis is generally poor with lesions progressing over time.

1. DR. SANJAY BOSAMIYA
ASSOCIATE PROFESSOR DEPARTMENT OF DVL
SMIMER SURAT
INDIA
NECROBIOSIS LIPOIDICA

2. History
 The term necrobiosis refers to alteration of dermal connective
tissue with loss of definition, pale staining, and absence of
nuclei
 1929- “Dermatitis atrophicans diabetica” by Oppenheim
 1932- Necrobiosis lipoidica diabeticorum by Urbach
 1935- Necrobiosis lipoidica (NL) by Goldsmith

3. Epidemiology
 Young to middle-aged adults
 For diabetic patients, the average age -25 years while in
non-diabetic / type 2 DM - 46 years
 Female : male ratio of ~3 : 1
 DM, usually type 1, ~65%;
 20% cases - at risk for the development of diabetes
(who have glucose intolerance or a family history of
diabetes)
 Control of the diabetes does not influence the course of

4. Pathogenesis
 Cause of necrobiosis lipoidica is unknown
 Vascular changes
 Microangiopathic vessel changes
 Larger caliber vessels
OR
 Disease of collagen
 Abnormal glucose transport by fibroblasts
 Fibroblasts cultured from NL lesions synthesize less
collagen

5. Clinical Features
 Asymptomatic
 Development of lesions
secondary to Koebner
phenomenon
 Small, sharply bordered,
elevated firm, dull red
papule
 Later, - firm irregularly
round or oval plaque with
well defined borders &
smooth, glistening
(glazed) surface

6. Clinical Features
 Center becomes
depressed and sulfur
yellow, surrounded by a
violet-red or pink border
 In yellow portion,
numerous
telangiectases and
ectatic veins are evident.

7. NL –case
 F/20 years, presented with
well defined sharply
borderd, nontender firm
indurated plaque about 5X5
cm in size with overlying
dusky erythematous skin, &
central yellowish depressed
area - on dorsa of left foot.
 There was H/O prior trauma
 She was known case of type
1 DM- on insulin injection

8. Clinical Features
 Ulceration occurs in one
third
 Hypohidrosis ,
hypoaesthesia, partial
alopecia
 In an unusual case, the
plaques –studded with
exophytic nodules
resembling tuberous
xanthomas
 Marked hyperlipidemia
 Rarely, SCC

9. Clinical Features
 Typical lesions- pretibial
skin
 Ankles, calves, thighs
popliteal areas & feet
 Upper extremities, face
and scalp
 Lesions - more annular
or serpiginous
 Often less atrophic
 Trunk, palms & soles
 Most cases –bilateral
 Koebnerization

10. HPE
 Entire thickness of the
dermis or its lower two-
thirds
 Top-to-bottom and
side-to-side
involvement often-
subcutis is involved
 Rectangular punch due
to sclerosis
 “Layered” tiers of
granulomatous
inflammation like

11. HPE
 Epidermis may be
normal, atrophic, or
hyperkeratotic
 Granuloma - histiocytes
surrounding horizontal
tiers of degenerated
collagen
 Histiocytes may or may
not be arranged in a
palisade
 Giant cells - more likely

12. HPE
 Giant cells - Langhans-
or foreign-body type;
occasionally, Touton
cells or asteroid bodies
 Horizontal, degenerated
collagen between layers
of granuloma
 Acellular, pale,
fragmented and
haphazardly arranged
 No mucin/ subtle; in

13. HPE
 Perivascular lymphocytic
infiltrate, & plasma cells -
common in the deep
dermis
 Blood vessels, particularly
in the middle and lower
dermis, often thickened
walls
 May see cholesterol clefts
or lymphoid nodules
 Often the subcutis is

14. HPE

15. HPE –case

16. HPE –case

17. HPE – case

18. HPE- case

19. HPE
Differential Diagnosis

20. Rectangular punch
 Due to thickened or sclerotic dermis
 Necrobiosis lipodica
 Morphea/scleroderma
 Chronic GvHD
 Chronic radiodermatitis
 Scar
 Normal skin of the back
 Connective tissue nevi
 Scleredema

21. Palisaded granuloma
 GA
 NL
 RN
 PNGD
 IGD
 IGDR
 NXG

22. D/D of NL
 Granuloma annulare
 Annular elastolytic giant cell granuloma
 Rheumatoid nodule
 Necrobiotic xanthogranuloma (NXG)
 Palisaded Neutrophilic & Granulomatous
Dermatitis -PGND

23. Features of GA & NL
Feature Granuloma
annulare
Necrobiosis
lipoidica
Distribution Focal and patchy Diffuse and full
thickness
Granuloma Palisaded or
interstitial
Horizontal tiers
(layers)
Mucin Yes No
Shape of punch
biopsy
Tapered Rectangular
Plasma cells Rare Common
Cholesterol clefts No Occasional

24. Features of GA & NL
GA NL

25. GA- Palisading pattern

26. GA

27. GA

28. GA

29. GA- Interstitial pattern

30. Annular elastolytic granuloma
 Central loss of elastic tissue
(primary basis)
 Ttransitional zone - raised
border - granulomatous
infiltrate
 Zone peripheral to the
annulus - increased amount
of thick elastotic material
 Larger and more numerous
giant cells
 Elastic fibers are engulfed by
palisading giant cells and
histiocytes
(elastophagocytosis)

31. Rheumatoid nodule
 Deep dermis and
subcutis
 Large palisading
granuloma surrounding
deeply staining
eosinophilic fibrinoid
degeneration of collagen
 No mucin
 Foreign-body giant cells

32. Necrobiotic xanthogranuloma
(NXG)
 X-shaped red zones of
necrosis within
granulomatous nodule
 Lipidized histiocytes &
multinucleate wreath giant
cells, & Touton giant cells
 Dermis and subcutis involved
 Neutrophilic debris within
necrotic areas (“pepper
bacon”)
 Cholesterol clefts
 Plasma cells and lymphoid

33. Palisaded Neutrophilic &
Granulomatous Dermatitis -PGND
 Neutrophilic
inflammation
 Karyhorrectic debris
 Frank leukocytoclastic
vasculitis
 More neutrophils,
nuclear dust, and
fibrinoid change than in
pure vasculitis

34. Red & Blue Granuloma

35. Treatment

36. Treatment for NL
 First line
 No treatment
 Topical steroids
 Intralesional steroids
 Topical tacrolimus 0.1%
 Second line
 PUVA
 UVA1
 Third line
 Multiple therapies of uncertain value

37. Multiple therapies of uncertain
value
 Tretinoin (0.05%)
 Chloroquine
 Photodynamic Therapy
 Fibrinolytic Agents
 High‐dose Nicotinamide
 Clofazimine
 Pentoxifylline
 Prostaglandin E1
 Aspirin
 Aspirin/Dipyridamole
Comb.
 Doxycycline
 Colchicine
 Thalidomide
 Mycophenolate mofetil
 Methotrexate
 TNF-α inhibitors
(recalcitrant ulcerated
lesions)
 Cyclosporine (severe
ulcerations)
 5-week courses of
systemic corticosteroid