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It is very rare and estimated to affect 1 in 100,000 per year. Because of its rarity the documentation, cases and information are sparse and not a huge amount is known for certain, meaning that EAC could actually be a set of many un-classified skin lesions. It is known to occur at all ages and all genders equally. Some articles state that women are more likely to be affected than men.
EN is associated with a wide variety of conditions, including:
- Idiopathic
In about 30–50% of cases, the cause of EN is unknown.
- Infection
- Streptococcal infection which, in children, is by far the most common precipitant,
- Primary infection of Tuberculosis
- "Mycoplasma pneumoniae"
- "Histoplasma capsulatum"
- "Yersinia"
- Epstein-Barr virus
- "Coccidioides immitis" (Valley fever)
- Cat scratch disease
- Autoimmune disorders, including
- Inflammatory bowel disease (IBD)
- Behçet's disease
- Sarcoidosis
- Pregnancy
- Medications, including
- Sulfonamides
- Penicillins
- Oral contraceptives
- Bromides
- Hepatitis B vaccination
- Cancer, including
- Non-Hodgkins lymphoma (NHL)
- Carcinoid tumours
- Pancreatic cancer
EN may also be due to excessive antibody production in lepromatous leprosy leading to deposition of immune complexes.
There is an association with the HLA-B27 histocompatibility antigen, which is present in 65% of patients with erythema nodosum.
A useful mnemonic for causes is SORE SHINS (Streptococci, OCP, Rickettsia, Eponymous (Behçet), Sulfonamides, Hansen's Disease (Leprosy), IBD, NHL, Sarcoidosis.
Often no specific cause for the eruptions is found. However, it is sometimes linked to underlying diseases and conditions such as:
- Food (including blue cheese or tomatoes).
- Contact Dermatitis (i.e. cleaning agents, fabric softeners, etc.)
- Fungal, Bacterial and Viral infections such as sinusitis, tuberculosis, candidiasis or tinea.
- Drugs including finasteride, etizolam (and benzodiazepines), chloroquine, hydroxychloroquine, oestrogen, penicillin and amitriptyline.
- Cancer (especially the type known as erythema gyratum perstans, in which there are concentric and whirling rings).
- Primary biliary cirrhosis.
- Graves disease.
- Appendicitis.
- Lupus
- Pregnancy (EAC usually disappears/stops soon after delivery of baby).
- Hormone (Contraceptive Pill, Stress, Hormone Drugs)
- Lyme Disease
Erythema nodosum is the most common form of panniculitis. It is most common in the ages of 20–30, and affects women 3–6 times more than men.
About 15 percent of patients with inflammatory bowel disease develop erythema nodosum.
It is associated with various physiological as well as pathological changes, or may be a normal finding:
- Portal hypertension
- Chronic liver disease (including chronic hepatitis)
- Pregnancy
- Polycythemia
- Thyrotoxicosis
- Rheumatoid arthritis (especially in patients with polycythaemia)
- Eczema and psoriasis
- Deep telangiectasias
- Coxsackievirus A infection (Hand, foot and mouth disease)
- Rocky Mountain spotted fever
- Secondary syphilis
- Kawasaki disease
- Adverse drug reaction: palmoplantar erythrodysesthesia (acral erythema)
Because circulating levels of estrogen increase in both cirrhosis and pregnancy, estrogen was thought to be the cause for the increased vascularity. More recently, nitric oxide has been implicated in the pathogenesis of palmar erythema.
Many suspected aetiologic factors have been reported to cause EM.
- Infections: Bacterial (including Bacillus Calmette-Guérin (BCG) vaccination, haemolytic "Streptococci", legionellosis, leprosy, "Neisseria meningitidis, Mycobacterium, "Pneumococcus, "Salmonella" species, "Staphylococcus" species, "Mycoplasma pneumoniae), "Chlamydial.
- Fungal (Coccidioides immitis)
- Parasitic ("Trichomonas" species, "Toxoplasma gondii), "
- Viral (especially Herpes simplex)
- Drug reactions, most commonly to: antibiotics (including, sulphonamides, penicillin), anticonvulsants (phenytoin, barbiturates), aspirin, antituberculoids, and allopurinol and many others.
- Physical factors: radiotherapy, cold, sunlight
- Others: collagen diseases, vasculitides, non-Hodgkin lymphoma, leukaemia, multiple myeloma, myeloid metaplasia, polycythemia
EM minor is regarded as being triggered by HSV in almost all cases. A herpetic aetiology also accounts for 55% of cases of EM major. Among the other infections, "Mycoplasma" infection appears to be a common cause.
Herpes simplex virus suppression and even prophylaxis (with acyclovir) has been shown to prevent recurrent erythema multiforme eruption.
It can be caused by infection, massage, electrical treatment, acne medication, allergies, exercise, solar radiation (sunburn), cutaneous radiation syndrome, mercury toxicity, blister agents, niacin administration, or waxing and tweezing of the hairs—any of which can cause the capillaries to dilate, resulting in redness. Erythema is a common side effect of radiotherapy treatment due to patient exposure to ionizing radiation.
As PNP is ultimately caused by the presence of a tumor, it is not contagious. There is no known way to predict who will become afflicted with it. Patients with cancer are therefore a group at risk. Although PNP has been known to affect all age groups, it is more likely to afflict middle-aged to older patients.
Erythema disappears on finger pressure (blanching), while purpura or bleeding in the skin and pigmentation do not. There is no temperature elevation, unless it is associated with the dilation of arteries in the deeper layer of the skin.
Palmar erythema is reddening of the palms at the thenar and hypothenar eminences.
Discoid lupus erythematosus (DLE) is a chronic skin condition of sores with inflammation and scarring favouring the face, ears, and scalp and at times on other body areas. These lesions develop as a red, inflamed patch with a scaling and crusty appearance. The centre areas may appear lighter in colour with a rim darker than the normal skin.
Discoid lupus erythematosus can be divided into localised, generalised, and childhood discoid lupus erythematosus.
Erythema induratum is a panniculitis on the calves. It occurs mainly in women, but it is very rare now. Historically, when it has occurred, it has often been concomitant with cutaneous tuberculosis, and it was formerly thought to be always a reaction to the tuberculum bacillus. It is now considered a panniculitis that is not associated with just a single defined pathogen. The medical eponym Bazin disease was historically synonymous, but it applies only to the tuberculous form and is dated.
Erythema ab igne was once commonly seen in the elderly who stood or sat closely to open fires or electric heaters; however, erythema ab igne has been reported in both young and elderly individuals. Women have a higher incidence of erythema ab igne than men. Although wide use of central heating has reduced the overall incidence of erythema ab igne, it is still sometimes found in people exposed to heat from other sources such as heating pads, space heaters, hot water bottles, and electronic devices.
Alpha-1 antitrypsin deficiency panniculitis is a panniculitis associated with a deficiency of the α-antitrypsin enzyme.
Some sources distinguish between the following:
- "Erythema marginatum rheumaticum"
- "Erythema marginatum perstans"
The overwhelming majority of neutrophilic eccrine hidradenitis (NEH) is seen in people with cancer, especially leukaemia, who receive chemotherapy with a cytotoxic drug. These include: Bleomycin, chlorambucil, cyclophosphamide, cytarabine, doxorubicin, lomustine, mitoxantrone, topotecan, and vincristine.
NEH was first described in 1982 in a patient with acute myeloid leukaemia (AML) who had received cytarabine as chemotherapy.
Cancer itself, infections, and other medicinal drugs also can lead to NEH. NEH has been reported in patients with cancer who have not received any form of chemotherapy (i.e., as a paraneoplastic syndrome), in patients with HIV and/or AIDS, and after the use of paracetamol (acetaminophen). Also the use of targeted agents can lead to NEH, e.g. imatinib, a tyrosine kinase inhibitor.
NEH has also been described without any known reason (idiopathic cases), including idiopathic cases in children.
The exact cause of NEH is unknown. In patients receiving chemotherapy, it has been postulated that a high concentration of the cytotoxic drug in sweat has a direct toxic effect on the eccrine glands.
Erythema marginatum is a type of erythema (redness of the skin or mucous membranes) involving pink rings on the torso and inner surfaces of the limbs which come and go for as long as several months. It is found primarily on extensor surfaces.
An association with bradykinin has been proposed in the case of hereditary angioedema.
Erythema multiforme is frequently self-limiting and requires no treatment. The appropriateness of glucocorticoid therapy can be uncertain, because it is difficult to determine if the course will be a resolving one.
The cause is unknown. Geographic tongue does not usually cause any symptoms, and in those cases where there are symptoms, an oral parafunctional habit may be a contributory factor. Persons with parafunctional habits related to the tongue may show scalloping on the sides of the tongue (crenated tongue). Some suggest that hormonal factors may be involved, because one reported case in a female appeared to vary in severity in correlation with oral contraceptive use. People with geographic tongue frequently claim that their condition worsens during periods of psychologic stress. Geographic tongue is inversely associated with smoking and tobacco use. Sometimes geographic tongue is said to run in families, and it is reported to be associated with several different genes, though studies show family association may also be caused by similar diets. Some have reported links with various human leukocyte antigens, such as increased incidence of HLA-DR5, HLA-DRW6 and HLA-Cw6 and decreased incidence in HLA-B51. Vitamin B2 deficiency (ariboflavinosis) can cause several signs in the mouth, possibly including geographic tongue, although other sources state that geographic tongue is not related to nutritional deficiency. Fissured tongue often occurs simultaneously with geographic tongue, and some consider fissured tongue to be an end stage of geographic tongue.
In the past, some research suggested that geographic tongue was associated with diabetes, seborrheic dermatitis and atopy, however newer research does not corroborate these findings. Others suggest allergy as a major factor, e.g. to nickel sulphate. Some studies have reported a link between geographic tongue and psoriasis, although 90% of children who are diagnosed with geographic tongue do not develop psoriasis. Again however, modern research studies do not support any link between psoriasis and geographic tongue. Lesions that are histologically indistinguishable from geographic tongue may also be diagnosed in reactive arthritis (arthritis, uveitis/conjunctivitis and urethritis).
Erythema multiforme major (also known as "erythema multiforme majus") is a form of rash with skin loss or epidermal detachment.
The term "erythema multiforme majus" is sometimes used to imply a bullous (blistering) presentation.
According to some sources, there are two conditions included on a spectrum of this same disease process:
- Stevens–Johnson syndrome (SJS)
- Toxic epidermal necrolysis (TEN) which described by Alan Lyell and previously called Lyell syndrome[5].
In this view, EM major, SJS and TEN are considered a single condition, distinguished by degree of epidermal detachment.
However, a consensus classification separates erythema multiforme minor, erythema multiforme major, and SJS/TEN as three separate entities.
If the lesions are mild, the patient will be subject to a good deal of pain. If the lesions are severe, the overall quality of life is devastating. The impaired skin barrier function commonly leads to localized infection, which sepsis and death may follow. The pain from the oral and pharyngeal ulcers interfere with eating, which can compromise nutritional health.
The general prognosis for PNP is poor. It is more hopeful if the tumor is benign, but in the case of malignant tumors, the mortality rate is roughly 90%. The two most commonly associated types of tumors are non-Hodgkin lymphoma and chronic lymphocytic lymphoma; nearly all of these patients die within two years of diagnosis. This is attributed to the effects of the tumor combined with the negative side effects of the medication administered to treat PNP.
Roughly 1/3 of the deaths from PNP stem from pulmonary insufficiency which is brought about by the action of PNP on the respiratory mucosa. It manifests as dyspnea and progresses to bronchiolitis obliterans (non-reversible obstructive lung disease) via an unknown mechanism.
Predisposing factors include abnormal amount of subcutaneous fat, thick ankles and abnormally poor arterial supply. Abnormal arterial supply causes low-grade ischemia of ankle region. The ankle skin becomes sensitive to temperature changes. When weather is cold, ankle is cold, blue and often tender. In hot weather, ankle becomes hot, edematous, swollen and painful. Chilblains may be present. On palpation, small superficial and painful nodules are felt. They break down to form small and multiple ulcers. Fresh crops of nodules appear in periphery of ulcer and ultimately break down. In nodular stage, pain is present; while it subsides in ulcerative stage.
Because the eruption is transient and self-limiting, no treatment is indicated.
Different types of heat sources can cause this condition such as:
- Repeated application of hot water bottles, heating blankets or heat pads to treat chronic pain—e.g., chronic backache.
- Repeated exposure to heated car seats, space heaters, or fireplaces. Repeated or prolonged exposure to a heater is a common cause of this condition in elderly individuals.
- Occupational hazards of silversmiths and jewellers (face exposed to heat), bakers and chefs (arms)
- Resting a laptop computer on the thigh (laptop computer-induced erythema ab igne). In a 2012 review, Riahi and Cohen describe the characteristics of laptop computer-induced erythema ab igne. Temperatures between 43-47 °C can cause this skin condition; modern laptops can generate temperatures in this range. Indeed, laptops with powerful processors can reach temperatures of 50 °C and be associated with burns. Positioning of the laptop on the thighs can allow for direct exposure to the heating elements of the laptop, which include the central processing unit (CPU) and the graphics processing unit (GPU). At least 15 cases have been reported by 2012 with the condition usually affecting the left anterior thigh. In these reports, 9 of the 15 patients were women (60%) with an average age of 25 years at diagnosis.
- In Kashmir, due to the use of a kanger which also causes kangri cancer
- It is a classic finding in chronic pancreatitis and may also be seen in people with hypothyroidism or lymphoedema
Lipodermatosclerosis is a form of panniculitis associated with chronic venous insufficiency that presents with brown indurations on the front of the shins. It may be associated with pain and other signs of chronic venous insufficiency. The exact cause is unknown.
Other forms include:
- Subcutaneous fat necrosis of the newborn, a form of panniculitis occurring in newborns that is usually self-resolving, that may be a result of hypoxic injury to relatively high levels of brown fat.
- Sclerema neonatorum, affecting premature births.
- Weber–Christian disease, a symmetrical form of the disease of unknown origin occurring in middle-aged women.
- Lupus erythematosus panniculitis, panniculitis associated with lupus erythematosus.
- Forms associated with use of high doses of systemic corticosteroids during rapid corticosteroid withdrawal, and from the injection of silicone or mineral oils.