Granuloma annulare | |
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Perforating form of granuloma annulare on hand | |
Specialty | Dermatology |
Granuloma annulare (GA) is a common, sometimes chronic skin condition which presents as reddish bumps on the skin arranged in a circle or ring. [1] 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. [2]
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) over the backs of the forearms, hands or feet, often centered on joints or knuckles. The 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 ]
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 ]
Granuloma annulare microscopically consists of dermal epithelioid histiocytes around a central zone of mucin—a so-called palisaded granuloma. [3]
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. [2] [4] [5]
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. [6] [7] Another study found evidence suggesting blood vessel involvement with masses of intercellular fibrin and thickened basal lamina found around capillaries. [6] [8]
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. [9] 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. [10] Further data has been collected supporting this finding. [6] [11] [12]
Granuloma annulare may be divided into the following types: [13] : 703–5
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. If local treatment fails it may be treated with systemic corticosteroids. [14] [15] 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. [16]
The disease was first described in 1895 by Thomas Colcott Fox as a "ringed eruption of the fingers", [2] and it was named granuloma annulare by Henry Radcliffe Crocker in 1902. [17]
Dermatology is the branch of medicine dealing with the skin. It is a speciality with both medical and surgical aspects. A dermatologist is a specialist medical doctor who manages diseases related to skin, hair, nails, and some cosmetic problems.
A granuloma is an aggregation of macrophages that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate. Such substances include infectious organisms including bacteria and fungi, as well as other materials such as foreign objects, keratin, and suture fragments.
Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, and is only named that because it looks like one. It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale, commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. The hue may be gray-brown in people with darker skin. Although there is a broad clinical range of LP manifestations, the skin and oral cavity remain as the major sites of involvement. The cause is unknown, but it is thought to be the result of an autoimmune process with an unknown initial trigger. There is no cure, but many different medications and procedures have been used in efforts to control the symptoms.
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, but improvable with treatment, and 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.
Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes.
A pyogenic granuloma or lobular capillary hemangioma is a vascular tumor that occurs on both mucosa and skin, and appears as an overgrowth of tissue due to irritation, physical trauma, or hormonal factors. It is often found to involve the gums, skin, or nasal septum, and has also been found far from the head, such as in the thigh.
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.
Acne fulminans is a severe form of the skin disease, acne, which can occur after unsuccessful treatment for another form of acne, acne conglobata. The condition is thought to be an immunologically induced disease in which elevated level of testosterone causes a rise in sebum and population of Cutibacterium acnes bacteria. The increase in the amount of C acnes or related antigens may trigger the immunologic reaction in some individuals and lead to an occurrence of acne fulminans. In addition to testosterone, isotretinoin may also precipitate acne fulminans, possibly related to highly increased levels of C acnes antigens in the patient's immune system. Acne fulminans is a rare disease. Over the past several years, fewer cases of this disease have occurred, possibly because of earlier and better treatment of acne. Approximately 100 patients with acne fulminans have been described.
Leishmania major is a species of parasite found in the genus Leishmania, and is associated with the disease zoonotic cutaneous leishmaniasis. L. major is an intracellular pathogen which infects the macrophages and dendritic cells of the immune system. Though Leishmania species are found on every continent aside from Antarctica, Leishmania major is found only in the Eastern Hemisphere, specifically in Northern Africa, the Middle East, Northwestern China, and Northwestern India.
Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy or sometimes as Destombes–Rosai–Dorfman disease, is a rare disorder of unknown cause that is characterized by abundant histiocytes in the lymph nodes or other locations throughout the body.
Subacute cutaneous lupus erythematosus is a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as poly-cyclic annular lesions or plaques similar to those of plaque psoriasis.
Kyrle disease is identified as a form of an acquired perforating disease. Other major perforating diseases are elastosis perforans serpiginosa and reactive perforating collagenosis. Recently, however, there is a controversy on categorizing Kyrle disease with perforating dermatosis or a subtype of acquired perforating collagenosis.
Fibrous papule of the nose is a harmless small bump on or near the nose. It is typically dome-shaped, skin-colored, white or reddish, smooth and firm. Less frequently it can occur elsewhere on the face. Sometimes there are a few. It may be shiny and remains unchanged for life. There may be a central hair.
Solar purpura is a skin condition characterized by large, sharply outlined, 1- to 5-cm, dark purplish-red ecchymoses appearing on the dorsa of the forearms and less often the hands.
Cutaneous small-vessel vasculitis (CSVV), is inflammation of small blood vessels, usually accompanied by small lumps beneath the skin. The condition is also known as hypersensitivity vasculitis, cutaneous leukocytoclastic vasculitis, hypersensitivity angiitis, cutaneous leukocytoclastic angiitis, cutaneous necrotizing vasculitis and cutaneous necrotizing venulitis,
Majocchi's granuloma is a skin condition characterized by deep, pustular plaques, and is a form of tinea corporis. It is a localized form of fungal folliculitis. Lesions often have a pink and scaly central component with pustules or folliculocentric papules at the periphery. The name comes from Domenico Majocchi, who discovered the disorder in 1883. Majocchi was a professor of dermatology at the University of Parma and later the University of Bologna. The most common dermatophyte is called Trichophyton rubrum.
Annular elastolytic giant-cell granuloma is a cutaneous condition characterized histologically by a dermal infiltrate of macrophages.
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.
Cutaneous manifestations of COVID-19 are characteristic signs or symptoms of the Coronavirus disease 2019 that occur in the skin. The American Academy of Dermatology reports that skin lesions such as morbilliform, pernio, urticaria, macular erythema, vesicular purpura, papulosquamous purpura and retiform purpura are seen in people with COVID-19. Pernio-like lesions were more common in mild disease while retiform purpura was seen only in critically ill patients. The major dermatologic patterns identified in individuals with COVID-19 are urticarial rash, confluent erythematous/morbilliform rash, papulovesicular exanthem, chilbain-like acral pattern, livedo reticularis and purpuric "vasculitic" pattern. Chilblains and Multisystem inflammatory syndrome in children are also cutaneous manifestations of COVID-19.
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