| Granuloma annulare | |
|---|---|
 | |
| Perforating form of granuloma annulare on hand | |
| Specialty | Dermatology  |
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]
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 ]
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. [4]
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]
Granuloma annulare may be divided into the following types: [14] : 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 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]
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]
Sebaceous hyperplasia is a disorder of the sebaceous glands in which they become enlarged, producing flesh-colored or yellowish, shiny, often umbilicated bumps. Sebaceous hyperplasia, primarily affecting older patients in high-concentration areas like the face, head, and neck, typically has a 2-4 mm diameter and causes no symptoms. The lesions are often surrounded by telangiectatic blood vessels, also known as "crown vessels," and a central dell, which is in line with the origin of the lesions.
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.
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.
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.
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.
Nevus oligemicus presents as a patch of livid skin that is cooler than the normal skin, as a result of decreased blood flow, in which vasoconstriction of deep vessels is thought to be the underlying defect.
Angiolymphoid hyperplasia with eosinophilia usually presents with pink to red-brown, dome-shaped, dermal papules or nodules of the head or neck, especially about the ears and on the scalp.
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.
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.
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.
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.
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