Granuloma anulare disseminatum L92.0

Rare variant of granuloma anulare with multiple, disseminated, mostly non-symptomatic, rarely slightly itchy, but occasionally also slightly prickly, skin-coloured or red, also brownish, firm, flat nodules or plaques on the trunk and extremities. The face and mucous membranes remain free.

Clinically, a papular form can be distinguished from a large-area, often anular plaque form. Older lesions may also manifest themselves exclusively as brown (sometimes atrophic) patches.

Etiology and pathogenesis remain mostly unrecognized.

Diabetes mellitus is observed in about 10-12% of patients. Disturbances in fat metabolism (hypercholesterolemia, hypertriglyceridemia) are observed in about 45% of patients.

Allopurinol has also been described as a trigger (more recent confirmation of drug induction is missing)

Occurs mainly in adulthood (thus later initial manifestation than in the classic anular granuloma).

Papular exanthema with multiple, disseminated, regionally or over the entire integument distributed, solitary or confluent into larger plaques, 0.1-0.2 cm in size, blue-reddish to brownish, surface-smooth, flat papules.

The skin changes can remain asymptomatic; slight itching is possible, but also an unpleasant stinging pain.

The classic anular structure of granuloma anulare may be absent, so that the diagnosis is only made histologically. Large anular structures can be formed by the arrangement of papules around a healed center (see figure).

However, such formations are rarely seen in granuloma anulare disseminatum.

Small nodular disseminated sarcoidosis: Dense, spotty, small, papular or small-nodular, possibly lichenoid, reddish-brownish or blue-reddish lesions mainly on the face (would be completely atypical for gran.a.d.), on the extensor sides of the extremities, rarely on the trunk. Later hyperpigmentation and development of telangiectasia.

Lichen planus exanthematicus: clinically quite close analogies, the typical "shine" of the lesions is missing in the granoderm, also an infestation of the oral or genital mucosa. The histology of the L.p.d. is conclusive.

Maculo-papular (lichenoid) drug exanthema: Course with a significantly higher acuteity than in granuloma anulare disseminatum. Histology excludes the granuloma anulare disseminatum.

The therapy depends on the extent of the skin changes and ultimately also on the patient's level of suffering. In principle, a local therapeutic approach will be considered the therapy of first choice. If no satisfactory results can be achieved, local therapy should be combined with a systemic therapy.

Local therapeutic approaches:

• The first choice therapy for this form of anular granuloma is PUVA therapy in the form of systemic PUVA or PUVA bath therapy. There are well-documented, positive experience reports about this therapy approach.
• Positive individual experiences have also been made with dithranol in the form of minute therapy.
• In addition, local glucocorticoids such as 0.1% triamcinolone acetonide cream(e.g. Triamgalen, rp. 259 ) or 0.1% betamethasone lotio(e.g. Betagalen, rp. 030 ) can be used.
• An alternative is the application of a 0.1% tacrolimus ointment(e.g. Protopic).

System therapeutic approaches:

• According to well-documented experience reports, therapy with fumaric acid esters(off-label use) is highly effective.
• Positive individual experiences were reported via Etanercept (2 times/week 50 mg).
• Alternative: Hydroxychloroquine
• There are also positive casuistry reports on methotrexate, ciclosporin, retinoids and clofazimine.

The course is generally protracted, often recurring for months; it is not uncommon to observe changing courses over 10 years or longer. Spontaneous healing is significantly less frequent than in the described form of the anular granuloma.

1. Choi JC et al (2003) Generalized perforating granuloma annulare in an infant. Pediatric Dermatol 20: 131-133
2. Jantke ME et al (2011) Therapy of the granuloma anulare disseminatum with anthralin. Dermatologist 62: 935-939
3. Kain S, Stephens CJ (2003) Successfull treatment of disseminated granuloma anulare with topical tacrolimus. Br J Dermatol 149(Suppl64): 24
4. Kohn S et al (2001) Rod-shaped bodies resembling birbeck granule-like structures in endothelial cells of dermal capillaries in generalized granuloma annulare. J Dermatol 28: 5-11
5. Lukács J et al (2015) Treatment of generalized granuloma annulare - a systematic review. J Eur Acad Dermatol Venereol 29:1467-1480
6. Schulze-Dirks A et al (2001) Granuloma annulare disseminatum: successful therapy with fumaric acid ester. dermatologist 52: 228-230
7. Shupack J et al (2006) Resolving granuloma annulare with etanercept. Arch Dermatol 142: 394-395
8. Tursen U et al (2002) Generalized granuloma anulare associated with Plummer's disease. J Eur Acad Dermatol Venereol 16: 419-420
9. Wollina U (2008) Granuloma annulare disseminatum responding to fumaric acid esters. Dermatol Online J 14:12