As it is a rare disease, a clear set of symptoms is difficult to define. Usually, patients show severe pain and swelling is reported but clinical presentations vary. It can have an 'orange peel' like appearance. Less common features are joint pain and carpal tunnel syndrome.

Eosinophilic fasciitis (), also known as "Shulman's syndrome", is a form of fasciitis, the inflammatory diseases that affect the fascia, the connective tissues surrounding muscles, blood vessels and nerves. Unlike other diseases in that category, it is limited to the arms and legs, and usually resolves itself, although some cases require corticosteroids, and some cases are associated with aplastic anemia.

The presentation of eosinophilic fasciitis is similar to scleroderma or systemic sclerosis. However, unlike scleroderma, it affects the fascia, not the skin (dermis). The characteristic and severe effects of scleroderma and systemic sclerosis, such as Raynaud's syndrome, involvement of the extremities, prominent small blood vessels (telangiectasia), and visceral changes such as swallowing problems, are absent.

It was first characterized in 1974, and it is not yet known whether it is actually a distinct condition or just a different presentation. However, it remains used for diagnostic purposes.

Several cases have been reported after strenuous exercise.

Affected individuals typically present with sudden painful proptosis, redness, and edema. Proptosis will vary according to the degree of inflammation, fibrosis, and mass effect. Occasionally, ptosis, chemosis, motility dysfunction (ophthalmoplegia), and optic neuropathy are seen. In the setting of extensive sclerosis there may be restriction, compression, and destruction of orbital tissue. Symptoms usually develop acutely (hours to days), but have also been seen to develop over several weeks or even months.Malaise, headaches, and nausea may accompany these symptoms. Other unusual presentations described include cystoid macular edema, temporal arteritis, and cluster headaches.

Pediatric IOI accounts for about 17% of cases idiopathic orbital inflammation. The most common sign is proptosis, but redness and pain are also experienced. Presentation varies slightly compared to adults with bilateral involvement, uveitis, disc edema and tissue eosinophilia being more common in this population. The presence of uveitis generally implies a poor outcome for pediatric IOI. Bilateral presentation may have a higher incidence of systemic disease.

In medicine, fasciitis is an inflammation of the fascia, which is the connective tissue surrounding muscles, blood vessels and nerves.

In particular, it often involves one of the following diseases:

- Necrotizing fasciitis

- Plantar fasciitis

- Eosinophilic fasciitis

- Paraneoplastic fasciitis

Idiopathic orbital inflammatory (IOI) disease, or orbital pseudotumor, refers to a marginated mass-like enhancing soft tissue involving any area of the orbit. It is the most common painful orbital mass in the adult population, and is associated with proptosis, cranial nerve (Tolosa–Hunt syndrome), uveitis, and retinal detachment. Idiopathic orbital inflammatory syndrome, also known as orbital pseudotumor, was first described by Gleason in 1903 and by Busse and Hochhmein. It was then characterized as a distinct entity in 1905 by Birch-Hirschfeld. It is a benign, nongranulomatous orbital inflammatory process characterized by extraocular orbital and adnexal inflammation with no known local or systemic cause. Its diagnosis is of exclusion once neoplasm, primary infection and systemic disorders have been ruled-out. Once diagnosed, it is characterized by its chronicity, anatomic location or histologic subtype.

Idiopathic orbital inflammation has a varied clinical presentation depending on the involved tissue. It can range from a diffuse inflammatory process to a more localized inflammation of muscle, lacrimal gland or orbital fat. Its former name, orbital pseudotumor, is derived due to resemblance to a neoplasm. However, histologically it is characterized by inflammation. Although a benign condition, it may present with an aggressive clinical course with severe vision loss and oculomotor dysfunction.

People usually complain of intense pain that may seem excessive given the external appearance of the skin. People initially have signs of inflammation, fever and a fast heart rate. With progression of the disease, often within hours, tissue becomes progressively swollen, the skin becomes discolored and develops blisters. Crepitus may be present and there may be a discharge of fluid, said to resemble "dish-water". Diarrhea and vomiting are also common symptoms.

In the early stages, signs of inflammation may not be apparent if the bacteria are deep within the tissue. If they are "not" deep, signs of inflammation, such as redness and swollen or hot skin, develop very quickly. Skin color may progress to violet, and blisters may form, with subsequent necrosis (death) of the subcutaneous tissues.

Furthermore, people with necrotizing fasciitis typically have a fever and appear sick. Mortality rates are as high as 73% if left untreated. Without surgery and medical assistance, such as antibiotics, the infection will rapidly progress and will eventually lead to death.

When it affects the groin it is known as Fournier gangrene.

Texier's disease is a pseudosclerodermatous reaction that occurs after injection with vitamin K, a subcutaneous sclerosis with or without fasciitis that lasts several years.

These conditions are sometimes considered together with the small vessel vasculitides.

Polyarteritis nodosa (PAN). Systemic necrotizing vasculitis and aneurysm formation affecting both medium and small arteries. If only small vessels are affected, it is called microscopic polyangiitis, although it is more associated with granulomatosis with polyangiitis than to classic PAN. At least 3 out of 10 criteria yields sensitivity and specificity of 82 and 87%:

- unexplained weight loss > 4 kg

- livedo reticularis

- testicular pain

- myalgias, weakness

- Abdominal pain, diarrhea, and GI bleeding

- mononeuropathy or polyneuropathy

- new onset diastolic blood pressure > 90 mmHg

- elevated serum BUN (> 40 mg/dL) or serum creatinine (> 1.5 mg/dL)

- hepatitis B infection

- arteriographic abnormalities

- arterial biopsy showing polymorphonuclear cells

Kawasaki disease. Usually in children(age<4), it affects large, medium, and small vessels, prominently the coronary arteries. Associated with a mucocutaneous lymph node syndrome. Diagnosis requires fever lasting five days or more with at least 4 out of 5 criteria:

- bilateral conjunctival injection

- injected or fissured lips, injected pharynx, or strawberry tongue

- erythema of palms/soles, edema of hands/feet, periungual desquamation

- polymorphous rash

- cervical lymphadenopathy (at least one node > 1.5 cm)

Isolated cerebral vasculitis. Affects medium and small arteries over a diffuse CNS area, without symptomatic extracranial vessel involvement. Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy.

IgG4-related disease has been described as an indolent condition. Although possibly based on opinion rather than on objective assessments, symptoms, if any, are commonly described as mild in the medical literature. This can be in spite of considerable underlying organ destruction. People are often described as being generally well at the time of diagnosis, although some may give a history of weight loss.

Pain is generally not a feature of the inflammation. However it may occur as a secondary effect, for example due to either obstruction or compression.

Often diagnosis is made due to the presence of painless swellings or mass lesions, or due to complications of masses, e.g. jaundice due to involvement of the pancreas, biliary tree or liver. Symptoms are commonly attributed to other conditions and other diagnoses may have been made years before diagnosis, e.g. urinary symptoms in men attributed to common prostate conditions. Lesions may also be detected incidentally on radiological images, but can be easily misdiagnosed as malignancies.

Reported cases do include some significant symptoms or findings however:

Giant-cell arteritis and Takayasu's arteritis have much in common, but usually affect patients of different ages, with Takayasu's arteritis affecting younger people, and giant-cell arteritis having a later age of onset.

Aortitis can also be considered a large-vessel disease.

Takayasu arteritis. Primarily affects the aorta and its main branches. At least 3 out of 6 criteria yields sensitivity and specificity of 90.5 and 97.8%:

- onset < 40 years affects young and middle -aged women (ages 15–45)

- claudication of extremities

- decreased pulsation of one or both brachial arteries

- at least 10 mmHg systolic difference in both arms

- bruit over one or both carotid arteries or abdominal aorta

- arteriographic narrowing of aorta, its primary branches, or large arteries in upper or lower extremities

- Ocular manifestation

- visual loss or field defects

- Retinal hemorrhages

- Neurological abnormalitis

- Treatment: steroids

Giant cell (temporal) arteritis. Chronic vasculitis of both large and medium vessels, primarily affecting cranial branches of the arteries arising from the aortic arch. At least 3 out of 5 criteria yields sensitivity and specificity of 95 and 91%:

- Age at onset ≥ 50 years

- New onset headache with localized tenderness

- Temporal artery tenderness or decreased pulsation

- Elevated ESR ≥ 50 mm/hour Westergren

- Temporal artery biopsy showing vasculitis with mononuclear cell infiltrate or granulomatous inflammation, usually with multinucleated giant cells

Necrotizing fasciitis (NF), commonly known as flesh-eating disease, is an infection that results in the death of the body's soft tissue. It is a severe disease of sudden onset that spreads rapidly. Symptoms include red or purple skin in the affected area, severe pain, fever, and vomiting. The most commonly affected areas are the limbs and perineum.

Typically the infection enters the body through a break in the skin such as a cut or burn. Risk factors include poor immune function such as from diabetes or cancer, obesity, alcoholism, intravenous drug use, and peripheral vascular disease. It is not typically spread between people. The disease is classified into four types, depending on the infecting organism. Between 55% and 80% of cases involve more than one type of bacteria. Methicillin-resistant "Staphylococcus aureus" (MRSA) is involved in up to a third of cases. Medical imaging is helpful to confirm the diagnosis.

Prevention is by good wound care and handwashing. It is usually treated with surgery to remove the infected tissue and intravenous antibiotics. Often a combination of antibiotics are used such as penicillin G, clindamycin, vancomycin, and gentamicin. Delays in surgery are associated with a higher risk of death. Despite high quality treatment the risk of death is between 25% and 35%.

Necrotizing fasciitis affects 0.4 to 1 person per 100,000 per year. Both sexes are affected equally. It becomes more common among older people and is very rare in children. Necrotizing fasciitis has been described at least since the time of Hippocrates. The term "necrotising fasciitis" first came into use in 1952.

IgG4-related disease (IgG4-RD), formerly known as IgG4-related systemic disease, is a chronic inflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, various degrees of fibrosis (scarring) and a usually prompt response to oral steroids. In approximately 51–70% of people with this disease, "serum" IgG4 concentrations are elevated during an acute phase.

It is a relapsing–remitting disease associated with a tendency to mass forming, tissue-destructive lesions in multiple sites, with a characteristic histopathological appearance in whichever site is involved. Inflammation and the deposition of connective tissue in affected anatomical sites can lead to organ dysfunction, or even organ failure, if not treated.

Early detection is important to avoid organ damage and potentially serious complications. Treatment is recommended in all symptomatic cases of IgG4-RD and also in asymptomatic IgG4-RD involving certain anatomical sites.

Although a clear understanding of the various skin lesions in IgG4-related disease is a work in progress, skin lesions have been classified into subtypes based on documented cases:

- Angiolymphoid hyperplasia with eosinophilia (or lesions that mimic it) and cutaneous pseudolymphoma

- Cutaneous plasmacytosis

- Eyelid swelling (as part of Mikulicz's disease)

- Psoriasis-like eruptions

- Unspecified maculopapular or erythematous eruptions

- Hypergammaglobulinemic purpura and urticarial vasculitis

- Impaired blood supply to fingers or toes, leading to Raynaud's phenomenon or gangrene

Note:

In addition, Wells syndrome has also been reported in a case of IgG4-related disease.

Vitamin K reactions occur after injection with vitamin K, and there are two patterns of presentation, (1) a reaction may occur several days to 2 weeks after inection with skin lesions that are pruritic, red patches and plaques that can deep-seated, involving the dermis and subcutaneous tissue, or (2) with subcutaneous sclerosis with or without fasciitis, that appears at the site of injection many months after treatment. The latter pseudosclerodermatous reaction has been termed Texier's disease and lasts several years.

Corneal involvement in VKC may be primary or secondary due to extension of limbal lesions. Vernal keratopathy includes 5 types of lesions.

1. Punctuate epithelial keratitis.

2. Ulcerative vernal keratitis.

3. Vernal corneal plaques.

4. Subepithelial scarring.

5. Pseudogerontoxon.

Based on severity, authors have classified VKC into clinical grades:

Grade 0 - Absence of symptoms

Grade 1 MILD - Symptoms but no corneal involvement

Grade 2 MODERATE - Symptoms with photophobia but no corneal involvement

Grade 3 SEVERE - Symptoms, photophobia, milfd to moderate SPK's OR with Diffuse SPK or corneal ulcer

EG typically presents with a combination of chronic nonspecific GI symptoms which include abdominal pain, diarrhea, occasional nausea and vomiting, weight loss and abdominal distension. Approximately 80% have symptoms for several years; a high degree of clinical suspicion is often required to establish the diagnosis, as the disease is extremely rare. It doesn't come all of a sudden but takes about 3–4 years to develop depending upon the age of the patient. Occasionally, the disease may manifest itself as an acute abdomen or bowel obstruction.

- Mucosal EG (25–100%) is the most common variety, which presents with features of malabsorption and protein losing enteropathy. Failure to thrive and anaemia may also be present. Lower gastrointestinal bleeding may imply colonic involvement.

- Muscular EG (13–70%) present with obstruction of gastric outlet or small intestine; sometimes as an obstructing caecal mass or intussusception.

- Subserosal EG (4.5% to 9% in Japan and 13% in the US) presents with ascites which is usually exudative in nature, abundant peripheral eosinophilia, and has favourable responses to corticosteroids.

- Other documented features are cholangitis, pancreatitis, eosinophilic splenitis, acute appendicitis and giant refractory duodenal ulcer.

Eosinophilic gastroenteritis (EG) is a rare and heterogeneous condition characterized by patchy or diffuse eosinophilic infiltration of gastrointestinal (GI) tissue, first described by Kaijser in 1937. Presentation may vary depending on location as well as depth and extent of bowel wall involvement and usually runs a chronic relapsing course. It can be classified into mucosal, muscular and serosal types based on the depth of involvement. Any part of the GI tract can be affected, and isolated biliary tract involvement has also been reported.

The stomach is the organ most commonly affected, followed by the small intestine and the colon.

Initial symptoms of Fournier gangrene include swelling or sudden pain in the scrotum, fever, pallor and generalized weakness. More marked cases are characterized by a foul odor and purulent discharge from the infected tissue. Crepitus has been reported. It begins as a subcutaneous infection. However, soon necrotic patches appear in the overlying skin which later develop into necrosis.

Plantar calcaneal bursitis is a medical condition in which there is inflammation of the plantar calcaneal bursa, a spongy fluid filled sac that cushions the fascia of the heel and the calcaneus (heel bone). It is characterized by swelling and tenderness of the central plantar heel area. It is sometimes called 'Policeman's heel'. It sometimes was, and should not be, confused with plantar fasciitis, which is inflammation of the plantar fascia and can affect any part of the foot.

The symptoms of DRESS syndrome usually begin several weeks after exposure to the offending drug. No gold standard exists for diagnosis, and at least two diagnostic criteria have been proposed. The RegiSCAR criteria and the Japanese consensus group criteria are detailed in the table below.

Symptoms may be severe and involve many different organs. In a retrospective Taiwanese cohort study of 60 patients, these incidences were observed.

When plantar fasciitis occurs, the pain is typically sharp and usually unilateral (70% of cases). Heel pain is worsened by bearing weight on the heel after long periods of rest. Individuals with plantar fasciitis often report their symptoms are most intense during their first steps after getting out of bed or after prolonged periods of sitting. Improvement of symptoms is usually seen with continued walking. Rare, but reported symptoms include numbness, tingling, swelling, or radiating pain. Typically there are no fevers or night sweats.

If the plantar fascia continues to be overused in the setting of plantar fasciitis, the plantar fascia can rupture. Typical signs and symptoms of plantar fascia rupture include a clicking or snapping sound, significant local swelling, and acute pain in the sole of the foot.

Papuloerythroderma of Ofuji is a rare disorder most commonly found in Japan, characterized by pruritic papules that spare the skinfolds, producing bands of uninvolved cutis, creating the so-called deck-chair sign. Frequently there is associated blood eosinophilia. Skin biopsies reveal a dense lymphohistiocytic infiltrate, eosinophils in the papillary dermis, and increased Langerhans cells (S-100 positive). Systemic steroids are the treatment of choice and may result in long-term remissions.

It was characterized in 1984.

Use of PUVA in treatment has been described.

DRESS syndrome is one of several terms that have been used to describe a severe idiosyncratic reaction to a drug that is characterized by a long latency of onset after exposure to the offending medication, a rash, involvement of internal organs, hematologic abnormalities, and systemic illness. Other synonymous names and acronyms include hypersensitivity syndrome (DIHS), anticonvulsant hypersensitivity syndrome, drug-induced hypersensitivity syndrome, drug-induced delayed multiorgan hypersensitivity syndrome, and drug-induced pseudolymphoma.

Until recently, nodular fasciitis have been considered a reactive process of uncertain cause. However, recent findings indicate that nodular fasciitis is a self-limited clonal neoplastic process (see below). Clinically, nodular fasciitis presents as a subcutaneous "growth" over a period of 3–6 weeks that eventually regresses. The lesion usually reaches a size of 2–3 cm. Larger lesions are unusual. Local recurrence has been described after simple surgical excision but it is rare.