1.4.2 Granuloma annulare
ICD-11
EE80.0
Synonyms
None.
Epidemiology
Granuloma annulare is a common skin disorder . Prevalence is 0.1% to 0.4%, annual incidences 0.1% to 0.4%, women more affected than men, and 2/3 of the patients are less than 30 years old.
Definition
Granuloma annulare is a granulomatous dermal or subcutaneous disorder characterised by infiltrating mononuclear cells that presents with skin-coloured or reddish, annular or papular lesions related to different aetiologic background.
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Granuloma annulare is a granulomatous dermal and/or subcutaneous disorder characterised by infiltrating mononuclear cells that presents with skin-coloured or reddish, annular or papular lesions related to different aetiologic background.
It is more common in children, young adults and people with diabetes mellitus.
Aetiology & Pathogenesis
The cause of granuloma annulare is unclear but it can be associated with autoimmune diseases, diabetes mellitus, HIV infection or malignancies.
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The cause of granuloma annulare is unclear. It can be associated with autoimmune diseases or diabetes mellitus. Granuloma annulare can be caused by a large number of certain drugs. Disseminated forms have been described in association with HIV infection or cancer (solid cancers or haematological malignancies). Other comorbidities are significant associations with hyperlipidemia, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE).
Signs & Symptoms
Granuloma annulare consists of localized or disseminated firm, small, skin-coloured or reddish papules arranged in rings or arcs, or hard, mobile, dermal and subcutaneous nodules.
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Granuloma annulare consists of localized or disseminated firm, small, skin-coloured or reddish papules arranged in rings or arcs, or hard, mobile, dermal and subcutaneous nodules.
Generally, there are no associated symptoms and the size of the lesions is only a few centimeters. It may be localized or generalized (disseminated).
Localisation
Granuloma annulare typically appears on the extensor surfaces of the extremities, in a disseminated form also on the trunk.
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Granuloma annulare typically appears on the extensor surfaces of the extremities (especially fingers and dorsum of hands). In its disseminated form, the trunk is also often involved.
Classification
Localized, disseminated, subcutaneous and perforating types.
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The lesions can present as localized, disseminated, subcutaneous, perforating types or patch-type.
Laboratory & other workups
In isolated granuloma annulare, no laboratory testing is needed. In disseminated granuloma annulare, HIV serology, complete blood count, glycemia, lipid profile may be tested. Unusual courses of the disease and clinical pictures need histological confirmation.
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In isolated granuloma annulare no laboratory testing is needed. In disseminated granuloma annulare, HIV serology, complete blood count, hyperglycemia and lipid profile may be tested. Autoimmune diseases and cancers can be looked for, depending on the clinical context. Unusual courses and clinical aspects of the disease need histological confirmation.
Dermatopathology
Granuloma annulare is characterized by loose, degenerative collagen bundles (necrobiosis) with a peripheral cuff of lymphocytes and histiocytes and palisading of the granuloma.
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Granuloma annulare is characterized by bundles, degenerative collagen bundles (necrobiosis) with a peripheral cuff of lymphocytes and histiocytes and a palisadic organisation of the granuloma (palisading pattern). The interstitial pattern is characterized by histiocytes between collagen bundles with mucin deposition in the superficial and mid dermis.
Course
Spontaneous remission, recurrences or a chronic evolution are possible. The treatment of disseminated chronic forms is difficult.
Complications
None, except for granuloma annulare associated with an underlying disease.
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There are no complications, except for granuloma annulare associated with an underlying disease. In rare subsets, a perforating course of the lesions can be seen.
Diagnosis
The diagnosis is obtained by clinico-pathological correlation.
Differential Diagnosis
Sarcoidosis, necrobiosis lipoidica, foreign body granuloma, rheumatoid nodules, interstitial granulomatous dermatitis, tuberculoid leprosy and syphilis.
Prevention & Therapy
Therapeutic abstention except detection of underlying disease or psychoreactive reactions, topical corticosteroids or cryotherapy are possible in limited forms. Phototherapy, dapsone, retinoids, hydroxychloroquine and TNF alpha blockers can be considered in disseminated forms. Strong regulation of diabetic metabolism. Appropriate medication in rheumatic diseases.
Preventive measures are not possible.
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Therapeutic abstention except detection of underlying disease or psychoreactive reactions, topical corticosteroids, topical calcineurin inhibitor or cryotherapy are possible in limited forms.
In disseminated forms, phototherapy, dapsone, retinoids, hydroxychloroquine and TNF alpha blockers can be considered. Recently, TNF alpha antagonist (adalimumab) have been reported to be very successful. In the disseminated and polyarthritis-associated types, treatment with biologics. The treatment of these disseminated forms is generally difficult. Strong regulation of diabetic metabolism is mandatory. Rheumatic diseases must be treated with appropriate medications.
Preventive measures are not possible.
Special
None.
Differential Diagnosis
Podcasts
Tests
- This 10-year-old child presents with a asymptomatic lesion on the back of this left foot. What systemic disease is associated with the disseminated form of this skin disease?
- Which is the primary lesion in granuloma annulare?
- Statement 1 A chest x-ray is always required if granuloma annulare is the diagnosis
- Which of these diseases must be considered in the differential diagnosis of granuloma annulare?
- 25-year-old lady with hay fever complains of new lesion on back of hand for weeks, spreading slowly. No recent travels. What is the diagnosis?
- True or false?
Further images / DOIA
Review Articles
- E.W. Piette, M. Rosenbach: Granuloma annulare: Pathogenesis, disease associations and triggers, and therapeutic options (2016)
- E.W. Piette, M. Rosenbach: Granuloma annulare: Clinical and histologic variants, epidemiology, and genetics (2016)
- S.J. Schmieder, C.D. Harper, G.J. Schmieder: Granuloma Annulare (2020)
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