Nodular vasculitisA18.4

Rare, chronic, gynecotropic, usually symmetrical, "infection-allergic" lobular panniculitis combined with vasculitis of the small vessels of the fat lobules. The dorsal parts of the lower leg are affected, which has been interpreted as a hyperergic reaction against Mycobacterium tuberculosis(see below). The delimitation as an independent entity is still controversial!

A necrotizing vasculitis of medium vessels is discussed as a hyperergic(Id reaction) against Mycobacterium tuberculosis (= tuberculid); also in atypical mycobacterioses.

For some of the cases, the association with tuberculosis can be considered certain (detection of mycobacterial DNA from biopsy material; therapeutic success with tuberculostatics!).

In the absence of this causal relationship, many authors refer to the clinical diagnosis of "erythema induratum" as nodular vasculitis or nodular vasculitis.

Here, other infectious and non-infectious triggers are discussed:

• constitutional type (acrocyanosis, livedo reticularis, lipedema)
• chronic exposure to cold
• Crohn's disease (K50.9) (Misago N et al. 2012)
• Strep throat
• hepatitis C
• HIV

Women are more frequently affected than men. Occurs mainly in women between 30-50 years of age, with a pycnical constitution and increased sensitivity to cold, a tendency to acrocyanosis and functional livedo reticularis.

Lower leg, flexor. extensor/ tibial . Less frequently on thighs, buttocks, upper arms and mammae.

Inflammatory, in interval phases only moderately, in recurrence phases clearly painful, red to brown-red, firm, cutaneous or subcutaneous nodules and plaques. Size: 2.5 cm, rarely up to 10 cm. Frequently, but not always, with mostly deep-reaching central necrotic melting and subsequent roundish, poorly healing ulceration. Extremely chronic or chronically recurrent course over several years possible. Healing leaving brownish pigmented, sunken scars.

Common accompanying symptoms: acrocyanosis, livedo reticularis, erythrocyanosis crurum puellarum, perniosis follicularis.

Phasic course with 4 stages:

• Acute stage: Vasculitis of medium-sized vessels (often not detected histologically). Multiple rosette-like palisade granulomas with histiocytes and neutrophilic granulocytes are seen in the fat lobules. Peripherally followed by lymphocyte sheaths. Few plasma cells. Within the inflammatory zone, lipocytes appear optically enlarged (so-called micropseudofatty cysts). Furthermore, capillaries and postcapillary venules with lymphocytic infiltrate sleeves are found. The adipose tissue septa are edematous altered, with lymphocytes and some giant cells. Diagnosis: Lobular granulomatous panniculitis.
• Intermediate stage: As a result of confluence of granulomas, adipose tissue necrosis increases. Appositional granuloma formation is seen in adjacent (not yet affected) lobules.
• Full stage: Complete involvement of the clinical nodule with homogeneous dense granulomatous inflammation. The septa are widened and fibrotic. Necrosis and thrombosis of septal small and larger vessels are evident, as well as small vessels not yet occluded and enveloped by dense lymphocyte coats. Focally, eosinophilic adipose tissue necrosis is visible. Furthermore, micropseudofatty cysts are formed. In the marginal area of the inflammatory zone, the changes of stage I can still be detected.
• Late stage: Fibrotic-scarring transformation. Wide septa and correspondingly narrowed lobules dominate the picture.

Peripheral circulatory disorders: Warm footwear, avoidance of cold floors and preferred stay in warm rooms. Alternating baths or baths with circulation-promoting agents containing nicotinic acid ester (e.g. rubriment baths), circulation-promoting ointments (e.g. enelbin paste). Compression bandages are particularly important and good because of their circulatory and warming effects.

Otherwise indifferent therapy with blank, caring external agents like Ungt. emulsificans aquosum (DAB) or powder (e.g. talcum, zinc oxide).

With (today rare constellation!) proven tuberculosis a tuberculostatic polychemotherapy must be carried out (therapy duration > 6 months).

In patients without tuberculosis detection, initial therapy with systemic glucocorticoids (Note: Glucocorticoids are effective in this disease in high and medium doses without exception; however, they do not represent a long-term therapy, since a relapse is pre-programmed if the individual dose threshold is not reached!)

Supplementary: Symptomatic pain therapy with analgesics.

Alternative therapeutic approaches (poor data situation!): Mycophenolic acid (1.4g/day), Ciclosporin (3.0-5.0mg/kgkgkgkg/day), Tetracyclines, Dapson (150mg/day).

Alternatively (case reports): Adalimumab (initial 40mg s.c./every 14 days, as maintenance dose 40mg s.c./every 3 weeks)

The synonymously used terms: Tuberculosis cutis indurativa; Tuberculosis indurativa cutanea et subcutanea should not be used because they imply active tuberculosis.

1. Bazin PAE (1861) Leçons théoriques et cliniques sur la scrofule, considérée en ellemême et dans ses rapports avec la syphilis, la dartre et l'arthritis. Adrien Delahaye (París), 2nd edition, pp. 145, 501.
2. Bilan P et al. (2015) Cutaneous tuberculosis and erythema induratum: A retrospective study of 13 cases in France. Ann Dermatol Venereol 142: 237-244
3. Braun-Falco O et al (1995) On the tuberculid term from a contemporary perspective. Dermatologist 46: 383-387
4. Chang MW (1999) Erythema induratum of Bazin in an infant. Pediatrics 103: 498-499
5. Campbell SM et al (2013) Erythema Induratum Caused by Mycobacterium chelonei in an Immunocompetent Patient. J Clin Aesthet Dermatol 6:38-40
6. Degitz K (1993) Successful treatment of erythema induratum of bazin following rapid detection of mycobacterial DNA by polymerase chain reaction. Arch Dermatol 129: 1619-1620
7. Fischer C et al. (2011) Successful therapy of nodular vasculitis with adalimumab (Humira®). Abstract CD 46th DDG Conference: P16/15
8. Heinemann C (2000) Erythema induratum of Bazin and Poncet's disease -- successful treatment with antitubercular drugs. J Eur Acad Dermatol Venereol 17: 334-336
9. Jacinto SS (2003) Erythema induratum of bazin: role of polymerase chain reaction in diagnosis. Int J Dermatol 42: 380-381
10. Marker M et al (2004) Erythema induratum bazin nodular vasculitis. JDDG 3: 206-213

11. Misago N et al (2012) Erythema induratum (nodular vasculitis) associated with Crohn's disease: a rare type of metastatic Crohn's disease. Am J Dermatopathol 34:325-329.

12. Muller CSL et al (2016) Diagnostic and histologic features of cutaneous vasculitides/vasculopathies. Act Dermatol 42: 286-301
13. Ratzinger G et al (2015) The vasculitis wheel-an algorithmic approach to cutaneous vasculitides. JDDG 1092-1118
14. Sharma S et al (2015) Clinicopathologic Spectrum of Cutaneous Tuberculosis: A Retrospective Analysis of 165 Indians. Am J Dermatopathol 37: 444-450
15. Teramura K et al (2014) Disseminated erythema induratum of Bazin. Eur J Dermatol 24:697-698