Granuloma annulare

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Granuloma annulare
Granuloma annulare.jpg
Perforating form of granuloma annulare on hand
Specialty Dermatology   OOjs UI icon edit-ltr-progressive.svg

Granuloma annulare (GA) is a rare, [1] sometimes chronic skin condition which presents as reddish bumps on the skin arranged in a circle or ring. [2] It can initially occur at any age, though two-thirds of patients are under 30 years old, and it is seen most often in children and young adults. Females are two times as likely to have it as males. [3]

Contents

Signs and symptoms

Granuloma annulare Granuloma annulare svg hariadhi.svg
Granuloma annulare

Aside from the visible rash, granuloma annulare is usually asymptomatic. Sometimes the rash may burn or itch. People with GA usually notice a ring of small, firm bumps (papules) more commonly over the backs of the forearms, hands or feet, often centered on joints or knuckles. GA, however, can be anywhere on the body and has also been seen on necks, backs, ankles and wrists. Bumps are caused by the clustering of T cells below the skin. These papules start as very small, pimple looking bumps, which spread over time from that size to dime, quarter, half-dollar size and beyond. Occasionally, multiple rings may join into one. Rarely, GA may appear as a firm nodule under the skin of the arms or legs. It also occurs on the sides and circumferential at the waist and without therapy can continue to be present for many years. Outbreaks continue to develop at the edges of the aging rings.[ citation needed ]

Causes

The condition is usually seen in otherwise healthy people. Occasionally, it may be associated with diabetes or thyroid disease. It has also been associated with autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, Lyme disease and Addison's disease. At this time, no conclusive connection has been made between patients.[ citation needed ]

Pathology

Micrograph showing a palisaded granuloma in a case of granuloma annulare. H&E stain. Granuloma annulare - add - high mag.jpg
Micrograph showing a palisaded granuloma in a case of granuloma annulare. H&E stain.

Granuloma annulare microscopically consists of dermal epithelioid histiocytes around a central zone of mucin—a so-called palisaded granuloma. [4]

Pathogenesis

Granuloma annulare is an idiopathic condition, though many catalysts have been proposed. Among these is skin trauma, UV exposure, vaccinations, tuberculin skin testing, and Borrelia and viral infections. [3] [5] [6]

The mechanisms proposed at a molecular level vary even more. In 1977, Dahl et al. proposed that since the lesions of GA often display a thickening of, occlusion of, or other trauma to blood vessels, blood vessels may be responsible for GA. From their study of 58 patients, they found that immunoglobin M (IgM), complement, and fibrinogen were in the blood vessels of GA areas, suggesting that GA may share similarities with an immune-mediated, type 3 reaction or that chronic immune vasculitis may be involved in the pathogenesis. [7] [8] Another study found evidence suggesting blood vessel involvement with masses of intercellular fibrin and thickened basal lamina found around capillaries. [7] [9]

Umbert et al. (1976), proposed an alternative pathogenesis: cell-mediated immunity. Their data suggests that lymphokines, such as macrophage-inhibiting factor (MIF), leads to sequestration of macrophages and histiocytes in the dermis. Then, upon lysosomal enzyme release by these sequestered cells, connective tissue damage ensues, which results in GA. [10] Later, these authors found data suggesting that activation of macrophages and fibroblasts are involved in the pathogenesis of GA and that fibrin and the rare IgM and C3 deposition around vessels were more likely a delayed-type hypersensitivity with resulting tissue and vessel changes rather than an immune-complex mediated disease. [11] Further data has been collected supporting this finding. [7] [12] [13]

Diagnosis

Types

Granuloma annulare may be divided into the following types: [14] :703–5

Treatment

Because granuloma annulare is usually asymptomatic and self-limiting with a course of about two years, initial treatment is generally topical steroids or calcineurin inhibitors; if unimproved with topical treatments, it may be treated with intradermal injections of steroids or phototherapy. If local treatment fails it may be treated with systemic corticosteroids. [15] [16] Treatment success varies widely, with most patients finding only brief success with the above-mentioned treatments. Most lesions of granuloma annulare disappear in pre-pubertal patients with no treatment within two years while older patients (50+) have rings for upwards of 20 years. The appearance of new rings years later is not uncommon. [17]

History

The disease was first described in 1895 by Thomas Colcott Fox as a "ringed eruption of the fingers", [3] and it was named granuloma annulare by Henry Radcliffe Crocker in 1902. [18]

See also

Related Research Articles

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<span class="mw-page-title-main">Lichen sclerosus</span> Itchy skin rash usually affecting the genitals

Lichen sclerosus (LS) is a chronic, inflammatory skin disease, of unknown cause, which can affect any body part of any person, but has a strong preference for the genitals, and is also known as balanitis xerotica obliterans (BXO) when it affects the penis. Lichen sclerosus is not contagious. There is a well-documented increase of skin cancer risk in LS, potentially improvable with treatment. LS in adult age women is normally incurable, although treatment can lessen its effects, and it often gets progressively worse if not treated properly. Most males with mild or intermediate disease, restricted to foreskin or glans, can be cured by either medical or surgical treatment.

<span class="mw-page-title-main">Erythema annulare centrifugum</span> Red, circular lesion on the skin

Erythema annulare centrifugum (EAC), is a descriptive term for a class of skin lesion presenting redness (erythema) in a ring form (anulare) that spreads from a center (centrifugum). It was first described by Darier in 1916. Many different terms have been used to classify these types of lesions and it is still controversial on what exactly defines EAC. Some of the types include annular erythema, erythema perstans, erythema gyratum perstans, erythema gyratum repens, darier erythema and erythema figuratum perstans.

Eosinophilic folliculitis is an itchy rash with an unknown cause that is most common among individuals with HIV, though it can occur in HIV-negative individuals where it is known by the eponym Ofuji disease. EF consists of itchy red bumps (papules) centered on hair follicles and typically found on the upper body, sparing the abdomen and legs. The name eosinophilic folliculitis refers to the predominant immune cells associated with the disease (eosinophils) and the involvement of the hair follicles.

Tumid lupus erythematosus is a rare, but distinctive entity in which patients present with edematous erythematous plaque.

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Anetoderma is a benign but uncommon disorder that causes localized areas of flaccid or herniated sac-like skin due to a focal reduction of dermal elastic tissue. Anetoderma is subclassified as primary anetoderma, secondary anetoderma, iatrogenic anetoderma of prematurity, congenital anetoderma, familial anetoderma, and drug-induced anetoderma.

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<span class="mw-page-title-main">Angiolymphoid hyperplasia with eosinophilia</span> Medical condition

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Reticular erythematous mucinosis (REM) is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides. It is a disease that tends to affect women in the third and fourth decades of life.

<span class="mw-page-title-main">Solar purpura</span> Medical condition

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<span class="mw-page-title-main">Generalized granuloma annulare</span> Medical condition

Generalized granuloma annulare is a skin condition of unknown cause, tending to affect women in the fifth and sixth decades, presenting as a diffuse but symmetrical, papular or annular eruption of more than ten skin lesions, and often hundreds.

Perforating granuloma annulare (PGA) is a skin condition of unknown cause, usually appearing on the dorsal hands, presenting as papules with a central keratotic core.

Actinic granuloma (AG) was first described by O'Brien in 1975 as a rare granulomatous disease. Lesions appear on sun-exposed areas, usually on the face, neck, and scalp, with a slight preference for middle-aged women. They are typically asymptomatic, single or multiple, annular or polycyclic lesions measuring up to 6 cm in diameter, with slow centrifugal expansion, an erythematous elevated edge, and a hypopigmented, atrophic center.

Granuloma multiforme is a cutaneous condition most commonly seen in central Africa, and rarely elsewhere, characterized by skin lesions that are on the upper trunk and arms in sun-exposed areas. It may be confused with tuberculoid leprosy, with which it has clinical similarities. The condition was first noted by Gosset in the 1940s, but it was not until 1964 that Leiker coined the term to describe "a disease resembling leprosy" in his study in Nigeria.

Progressive nodular histiocytosis is a cutaneous condition clinically characterized by the development of two types of skin lesions: superficial papules and deeper larger subcutaneous nodules. Progressive nodular histiocytosis was first reported in 1978 by Taunton et al. It is a subclass of non-Langerhans cell histiocytosis and a subgroup of xanthogranuloma.

Acanthoma fissuratum, also known as granuloma fissuratum is a cutaneous condition characterized by local thickening of the skin in response to pressure caused by an eyeglass frame. Acanthoma fissuratum is a hard, folded, flesh-colored lesion or plaque with a central groove. It affects the ear and is common in people wearing poorly fitting spectacle frames. It can also affect other locations like the penis, outer auditory canal, and posterior forchette of the vulva.

<span class="mw-page-title-main">Livedoid vasculopathy</span> Blood vessel disorder causing ulcers in the lower limbs

Livedoid vasculopathy (LV) is an uncommon thrombotic dermal vasculopathy that is characterized by excruciating, recurrent ulcers on the lower limbs. Livedo racemosa, a painful ulceration in the distal regions of the lower extremities, is the characteristic clinical appearance. It heals to form porcelain-white, atrophic scars, also known as Atrophie blanche.

Granuloma gluteale infantum is a cutaneous condition that appears in the anogenital region of infants as a complication of diaper dermatitis. According to some, no granulomas are found.

Rheumatoid neutrophilic dermatitis, also known as rheumatoid neutrophilic dermatosis, is a cutaneous condition associated with rheumatoid arthritis.

References

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  2. Dennis, Mark; Bowen, William Talbot; Cho, Lucy (2012). "Granuloma annulare". Mechanisms of Clinical Signs. Elsevier. p. 525. ISBN   978-0729540759; pbk{{cite book}}: CS1 maint: postscript (link)
  3. 1 2 3 Dermatology. Jean Bolognia, Julie V. Schaffer, Lorenzo Cerroni (Fourth ed.). [Philadelphia, Pa.] 2018. pp. 1650–1653. ISBN   978-0-7020-6342-8. OCLC   1011508489.{{cite book}}: CS1 maint: location missing publisher (link) CS1 maint: others (link)
  4. Günaştı, Suhan; Aksungur, Varol L. (January 2014). "Granulomatous disorders". Clinics in Dermatology. 32 (1): 47–65. doi:10.1016/j.clindermatol.2013.05.025. PMID   24314377.
  5. Tekin, Tamer (2010). "A case of unusual cranial fracture due to land mine explosion". Turkish Neurosurgery. doi:10.5137/1019-5149.jtn.3529-10.2. ISSN   1019-5149.
  6. Marcoval, Joaquim; Moreno, Abelardo; Maña, Juan (August 2004). "Foreign bodies in cutaneous sarcoidosis: Letter to the Editor". Journal of Cutaneous Pathology. 31 (7): 516. doi:10.1111/j.0303-6987.2004.00225.x. PMID   15239685. S2CID   42375301.
  7. 1 2 3 Piette, Evan W.; Rosenbach, Misha (September 2016). "Granuloma annulare: Pathogenesis, disease associations and triggers, and therapeutic options". Journal of the American Academy of Dermatology. 75 (3): 467–479. doi:10.1016/j.jaad.2015.03.055. PMID   27543210.
  8. Dahl, Mark V. (1977-04-01). "Vasculitis in Granuloma Annulare: Histopathology and Direct Immunofluorescence". Archives of Dermatology. 113 (4): 463–467. doi:10.1001/archderm.1977.01640040071010. ISSN   0003-987X. PMID   322621.
  9. Friedman-Birnbaum, Rachel; Ludatscher, Ruth M. (August 1986). "Comparative Ultrastructural Study of Generalized and Localized Granuloma Annulare". The American Journal of Dermatopathology. 8 (4): 302–308. doi:10.1097/00000372-198608000-00005. ISSN   0193-1091. PMID   3766919.
  10. Umbert, Pablo; Belcher, Richard W.; Winkelmann, R. K. (1976). "Lymphokines (MIF) in the serum of patients with sarcoidosis and cutaneous granuloma annulare". British Journal of Dermatology. 95 (5): 481–485. doi:10.1111/j.1365-2133.1976.tb00857.x. ISSN   1365-2133. PMID   791335. S2CID   7026704.
  11. Umbert, Pablo (1977-12-01). "Histologic, Ultrastructural, and Histochemical Studies of Granuloma Annulare". Archives of Dermatology. 113 (12): 1681–1686. doi:10.1001/archderm.1977.01640120049007. ISSN   0003-987X. PMID   596898.
  12. Bergman, Reuven; Lichtig, Chaim; Friedman-Birnbaum, Rachel (December 1993). "Histopathological and Direct Immunofluorescence Study of Early Lesions, and the Adjacent Normal-Looking Skin of Actively Spreading Lesions". The American Journal of Dermatopathology. 15 (6): 544–548. doi:10.1097/00000372-199312000-00005. ISSN   0193-1091. PMID   8311184.
  13. Buechner, S. A. (1983-02-01). "Identification of T-cell Subpopulations in Granuloma Annulare". Archives of Dermatology. 119 (2): 125–128. doi:10.1001/archderm.1983.01650260033012. ISSN   0003-987X. PMID   6218788.
  14. James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: Clinical Dermatology. Saunders Elsevier. ISBN   0-7216-2921-0.
  15. "Granuloma Annulare Treatment". www.buzzle.com. Archived from the original on 2010-01-25.{{cite web}}: CS1 maint: unfit URL (link)
  16. "Granuloma annulare | DermNet NZ".
  17. Ghadially, Ruby (15 October 2020). "Granuloma Annulare: Treatment & Management". Medscape. Retrieved 19 August 2023.
  18. Shanmuga1, Sekar C.; Rai1, Reena; Laila1, A.; Shanthakumari, S.; Sandhya, V. (2010), "Generalized granuloma annulare with tuberculoid granulomas: A rare histopathological variant", Indian Journal of Dermatology, Venereology and Leprology , 76 (1): 73–75, doi: 10.4103/0378-6323.58691 , PMID   20061743 , retrieved 23 May 2010{{citation}}: CS1 maint: numeric names: authors list (link)