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Necrotizing granulomas can develop in patients with rheumatoid arthritis, typically manifesting as bumps in the soft tissues around the joints (so-called rheumatoid nodules) or in the lungs.
Sarcoidosis involves the skin in between 9 and 37% of persons and is more common in African Americans than in European Americans. The skin is the second most commonly affected organ after the lungs. The most common lesions are erythema nodosum, plaques, maculopapular eruptions, subcutaneous nodules, and lupus pernio. Treatment is not required, since the lesions usually resolve spontaneously in two to four weeks. Although it may be disfiguring, cutaneous sarcoidosis rarely causes major problems. Sarcoidosis of the scalp presents with diffuse or patchy hair loss.
The frequency of cardiac involvement varies and is significantly influenced by race; in Japan more than 25% of persons with sarcoidosis have symptomatic cardiac involvement, whereas in the US and Europe only about 5% of cases present with cardiac involvement. Autopsy studies in the US have revealed a frequency of cardiac involvement of about 20–30%, whereas autopsy studies in Japan have shown a frequency of 60%. The presentation of cardiac sarcoidosis can range from asymptomatic conduction abnormalities to fatal ventricular arrhythmia. Conduction abnormalities are the most common cardiac manifestations of sarcoidosis in humans and can include complete heart block. Second to conduction abnormalities, in frequency, are ventricular arrhythmias, which occurs in about 23% of persons with cardiac involvement. Sudden cardiac death, either due to ventricular arrhythmias or complete heart block is a rare complication of cardiac sarcoidosis. Cardiac sarcoidosis can cause fibrosis, granuloma formation, or the accumulation of fluid in the interstitium of the heart, or a combination of the former two.
Ulcerative sarcoidosis is a cutaneous condition affecting roughly 5% of people with sarcoidosis.
Annular sarcoidosis is a cutaneous condition characterized by papular skin lesions arranged in annular
patterns, usually with a red-brown hue.
A foreign-body granuloma occurs when a foreign body (such as a wood splinter, piece of metal, glass etc.) penetrates the body's soft tissue followed by acute inflammation and formation of a granuloma. In some cases the foreign body can be found and removed even years after the precipitating event.
Morpheaform sarcoidosis is a very rare cutaneous condition characterized by specific cutaneous skin lesions of sarcoidosis accompanied by substantial fibrosis, simulating morphea.
Erythrodermic sarcoidosis is a cutaneous condition and very rare form of sarcoidosis.
Hypopigmented sarcoidosis is a cutaneous condition characterized by areas of hypopigmented skin. It is usually diagnosed in darkly pigmented races and may be the earliest sign of sarcoidosis.
Papular sarcoid is a cutaneous condition characterized by papules, which are the most common morphology of cutaneous sarcoidosis.
Ichthyosiform sarcoidosis is a cutaneous condition resembling ichthyosis vulgaris or acquired ichthyosis, with fine scaling usually on the distal extremities, by caused by sarcoidosis.
Acne conglobata is a highly inflammatory disease presenting with comedones, nodules, abscesses, and draining sinus tracts.
This condition generally begins between the ages of 18 and 30. It usually persists for a very long time, and often until the patient is around 40 years old. Although it often occurs where there is already an active acne problem, it can also happen to people whose acne has subsided. Although the cause of this type of acne is unknown, it is associated with testosterone and thus appears mainly in men. It can be caused by anabolic steroid abuse and sometimes appears in men after stopping testosterone therapy. It can also happen to someone who has a tumor that is releasing large amounts of androgens, or to people in remission from diseases, such as leukemia. In certain persons, the condition may be triggered by exposure to aromatic hydrocarbons or ingestion of halogens.
Acne conglobata presents with blackheads appearing around the face, neck, chest, upper arms and buttocks in groups of two or three. Pimples form around the blackheads; they are large and engorged with fluid and may be sensitive to touch. They remain for a while and continue to grow and fill with pus until they eventually rupture. After the lesion has drained, it fills up again. After they rupture, several nodules can fuse together to form larger shapes. The lesions remain for a long time. They form a scab in the center but they continue to spread outwards. When the lesions do eventually heal, they leave scars that can be the usual type of acne scar (atrophic) or a raised bump like those normally left behind by a burn or a cut (keloidal).
The first signs of erythema nodosum are often flu-like symptoms such as a fever, cough, malaise, and aching joints. Some people also experience stiffness or swelling in the joints and weight loss.
Erythema nodosum is characterised by nodules (rounded lumps) below the skin surface, usually on the shins. These subcutaneous nodules can appear anywhere on the body, but the most common sites are the shins, arms, thighs, and torso. Each nodule typically disappears after around two weeks, though new one may continue to form for up to six or eight weeks. A new nodule usually appears red and is hot and firm to touch. The redness starts to fade and it gradually becomes softer and smaller until it disappears. Each nodule usually heals completely without scarring over the course of about two weeks. Joint pain and inflammation sometimes continues for several weeks or months after the nodules appear.
Less common variants of erythema nodosum include:
- Ulcerating forms, seen in Crohn's disease
- Erythema contusiforme, when a subcutaneous hemorrhage (bleeding under the skin) occurs with a erythema nodosum lesion, causing the lesion to look like a contusion (bruise)
- Erythema nodosum migrans (also known as subacute nodular migratory panniculitis), a rare form of chronic erythema nodosum characterized by asymmetrical nodules that are mildly tender and migrate over time.
Signs and symptoms may include:
- Persistent or recurrent enlargement of the lips, causing them to protrude. If recurrent, the interval during which the lips are enlarged may be weeks or months. The enlargement can cause midline fissuring of the lip ("median cheilitis") or angular cheilitis (sores at the corner of the mouth). The swelling is non-pitting (c.f. pitting edema) and feels soft or rubbery on palpation. The mucous membrane of the lip may be erythemaous (red) and granular. One or both lips may be affected.
- Oral ulceration (mouth ulcers) which may be aphthous like, or be more chronic and deep with raised margins. Alternatively, lesions similar to pyostomatitis vegetans may occur in OFG, but this is uncommon.
- "Full width" gingivitis (compare with marginal gingivitis).
- Gingival enlargement (swelling of the gums).
- Fissured tongue (grooves in the tongue).
- Enlargement of the mucous membrane of the mouth, which may be associated with cobblestoning and mucosal tags (similar lesions often occur on the intestinal mucosa in Crohn's disease).
- Enlargement of the perioral and periorbital soft tissues (the tissues of the face around the mouth and the eyes). The facial skin may be dry, exfoliative (flaking) or erythematous.
- Cervical lymphadenopathy (enlarged lymph nodes in the neck).
- Facial palsy (weakness and altered sensation of the face).
The enlargement of the tissues of the mouth, lips and face seen in OFG is painless. Melkersson-Rosenthal syndrome is where OFG occurs with fissured tongue and paralysis of the facial nerve. The cause of the facial paralysis is thought to be caused by the formation of granulomas in the facial nerve, which supplies the muscles of facial expression.
This condition is characterized by:
- a diffuse infiltration of all the skin which never transforms into nodule
- a complete alopecia of eyebrows and eyelashes and body hair
- an anhydrotic and dysesthesic zones of the skin
- a peculiar type of lepra reaction named Lucio's phenomenon or necrotic erythema
Lucio's phenomenon consists of well-shaped erythematous spots which later become necrotic with scabs, ulcerations and scars. These lesion usually on the lower extremities and may be extensive They are frequently painful. Rarely it may be fatal.
OFG could be classified as a type of cheilitis (lip inflammation), hence the alternative names for the condition using the word cheilitis, and a granulomatous condition.
The disease mimics many other dermatoses and can be confused with conditions, such as sarcoidosis, discoid lupus erythematosus, mycosis fungoides, and fixed drug eruption.
Sarcoidosis is a systemic disease of unknown cause that results in the formation of non-caseating granulomas in multiple organs. The prevalence is higher among blacks than whites by a ratio of 20:1. Usually the disease is localized to the chest, but urogenital involvement is found in 0.2% of clinically diagnosed cases and 5% of those diagnosed at necropsy. The kidney is the most frequently affected urogenital organ, followed in men by the epididymis. Testicular sarcoidosis can present as a diffuse painless scrotal mass or can mimic acute epididymo-orchitis. Usually it appears with systemic manifestations of the disease. Since it causes occlusion and fibrosis of the ductus epididymis, fertility may be affected. On ultrasound, the hypoechogenicity and ‘infiltrative’ pattern seen in the present case are recognized features. Opinions differ on the need for histological proof, with reports of limited biopsy and frozen section, radical orchiectomy in unilateral disease and unilateral orchiectomy in bilateral disease. The peak incidence of sarcoidosis and testicular neoplasia coincide at 20–40 years and this is why most patients end up having an orchiectomy. However, testicular tumours are much more common in white men, less than 3.5% of all testicular tumours being found in black men. These racial variations justify a more conservative approach in patients of descent with proven sarcoidosis elsewhere. Careful follow-up and ultrasonic surveillance may be preferable in certain clinical settings to biopsy and surgery, especially in patients with bilateral testicular disease.
Two main approaches to genitourinary sarcoidosis have been proposed. Based on the marked relationship between testicular cancer and sarcoidosis, orchiectomy is recommended, even if evidence of sarcoidosis in other organs is present. By contrast, others consider immediate orchiectomy as being quite aggressive because of several factors associated with a benign diagnosis, as well as the involvement of the epididymis or vas deferens and bilateral testicular involvement. If the malignant diagnosis is established by exploration and intraoperative ultrasound-guided biopsy, orchiectomy is performed in cases of diffuse involvement of a testis. Spontaneous resolution has been reported in 50% to 70% of patients with active sarcoidosis. If the diagnosis is not established unequivocally, immunosuppressive agents (frequently steroids) will resolve the inflammation in patients who wish to salvage their fertility; and in those with severely advanced disease, after careful consideration.
A new approach has been proposed recently, based on the absence of evidence for malignant transformation in pathologically confirmed benign diagnosed testicular sarcoidosis, and it involves the open exploration of both testes, with resection of the largest lesion (on the right tunica). In this technique, patient was not given steroids after the operation. Nevertheless, careful follow-up may be preferred to medication or surgery in certain clinical settings.
Pain may be the first noticed symptom. People with lipodermatosclerosis have tapering of their legs above the ankles, forming a constricting band resembling an inverted champagne bottle. In addition, there may be brownish-red pigmentation and induration.
The diffuse leprosy of Lucio and Latapí, also known as diffuse lepromatous leprosy or "pretty leprosy" is a clinical variety of lepromatous leprosy. It was first described by Lucio and Alvarado in 1852 and re-identified by Latapí in 1936. It is common in Mexico (23% leprosy cases) and in Costa Rica and very rare in other countries.
Granuloma faciale (GF) is an uncommon benign chronic skin disease of unknown origin characterized by single or multiple cutaneous nodules, usually occurring over the face. Occasionally, extrafacial involvement is noted, most often on sun-exposed areas.
Pterygium unguis (also known as "Dorsal pterygium") forms as a result of scarring between the proximal nailfold and matrix, with the classic example being lichen planus, though it has been reported to occur as a result of sarcoidosis and Hansen's disease.
Lipodermatosclerosis (also known as "chronic panniculitis with lipomembranous changes", "hypodermitis sclerodermiformis", "sclerosing panniculitis", and "stasis panniculitis") is a skin and connective tissue disease. It is a form of lower extremity panniculitis, an inflammation of the layer of fat under the epidermis.
It is characterized by enlargement of the lymph nodes near the inner border of the lungs (called "hilar lymphadenopathy") as seen on x-ray, and tender red nodules (erythema nodosum) are classically present on the shins, predominantly in women. It may also be accompanied by arthritis (more prominent in men) and fever. The arthritis is often acute and involves the lower extremities.
Löfgren syndrome consists of the triad of erythema nodosum, bilateral hilar lymphadenopathy on chest radiograph, and arthralgia.
Bilateral hilar lymphadenopathy is a bilateral enlargement of the lymph nodes of pulmonary hila. It is a radiographic term that describes the enlargement of mediastinal lymph nodes and is most commonly identified by a chest x-ray.
Retroperitoneal fibrosis or Ormond's disease is a disease featuring the proliferation of fibrous tissue in the retroperitoneum, the compartment of the body containing the kidneys, aorta, renal tract, and various other structures. It may present with lower back pain, kidney failure, hypertension, deep vein thrombosis, and other obstructive symptoms. It is named after John Kelso Ormond, who rediscovered the condition in 1948.
Uveoparotitis is a symptom of sarcoidosis. It describes a chronic inflammation of the parotid gland and uvea. There is also a phenomenon called Waldenström's uveoparotitis. In this case, the symptom is related to Heerfordt's syndrome.
The following are causes of BHL:
- Sarcoidosis
- Infection
- Tuberculosis
- Fungal infection
- Mycoplasma
- Intestinal Lipodystrophy (Whipple's disease)
- Malignancy
- Lymphoma
- Carcinoma
- Mediastinal tumors
- Inorganic dust disease
- Silicosis
- Berylliosis
- Extrinsic allergic alveolitis
- Such as bird fancier's lung
- Less common causes also exist:
- Eosinophilic granulomatosis with polyangiitis
- Human immunodeficiency virus
- Extrinsic allergic alveolitis
- Adult-onset Still's disease