Nodular vasculitis A18.4

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.

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