Sarcoidosis Remission and Aden Protocol
INTRODUCTION Sarcoidosis is a noncaseating granulomatous multi-system disease of unknown etiology that most commonly affects young adults. It affects males and females equally, but females are more likely to show ocular involvement. There is a greater prevalence of sarcoidosis in the southeastern United States, and it is believed to occur more commonly among blacks. A bimodal incidence has been reported. A sub-acute presentation in patients less than 30 years of age is more likely to be self-limited, subsiding within two years. This transient variety is seldom associated with skin lesions. A more chronic form of the disease occurs in older age groups and skin lesions occur in up to 30 of patients. This form is believed to be due to a defect in T-lymphocyte suppressor function, and it frequently presents with hilar adenopathy, lung infiltrations, and more rarely with skin or eye lesions. The incidence of ocular involvement in patients with sarcoidosis is estimated to be 22 and eyelid...
All lesions that involve the eyelids or any other region of the body can be thought of as deriving from two basic sources. Those that arrive in the lids from other more remote sources are exogenous lesions. These include metastatic tumors from sites such as the breast or lung. Also included here are infiltrations in the dermis and epidermis of cellular or other materials that secondarily involve eyelid structures. Included here are diseases such as amyloidosis, sarcoidosis, infectious inflammations such as herpes and cellulitis, xanthelamas, acute atopic dermatitis, erythema multiforme, granuloma annulare, and lymphoid and myeloid infiltrates. All exogenous lesions disturb the normal eyelid architechture to some extent, and may be generalized or confined to specific eyelid tissue types.
A granuloma is a microscopic aggregate containing varying proportions of activated macrophages (epithelioid cells), multinucleated giant cells resulting from fusion of epithelioid cells, and other mononuclear leukocytes (lymphocytes, plasma cells, monocytes, and macrophages). Foreign body granulomas are reactions to relatively inert particles and typically have multinucleated giant cells, macrophages, and usually only small numbers of epithelioid cells. Immune or allergic granulomas are a response to insoluble particles that can induce a cell-mediated immune response they may result from foreign substances such as zirconium, beryllium, or dyes used for tattoos, or microbes such as Mycobacterium tuberculosis and fungi. Immune allergic granulomas typically contain abundant epithelioid cells and variable numbers of multinucleated giant cells. Other descriptors used for granulomas are sarcoidal, tuberculoid, and palisading. Sarcoidal granulomas, also termed naked granulomas, have...
Peripheral neuropathies Spinal mononeuropathies that can be confused with radiculopathies (e.g., diabetic neuropathy, sarcoid spinal mononeuropathy, paraneoplastic sensory neuropathy, combined system disease-vitamin B12 deficiency, pharmaceutical and industrial toxin neuropathy, ischemic neuropathy)
The categories for granulomatous dermatoses are not absolute and are only useful as a skeletal framework from which to start one's interpretation of a histologic section. For example (i) rarely cutaneous sarcoidosis may demonstrate tuberculoid granulomas or foreign body granulomas (ii) cutaneous infections as tuberculosis or leprosy most commonly present as tuberculoid granulomas but can be sarcoidal or palisading in type (iii) some infectious granuloma can be palisaded (as some fungal, mycobacterial infections and so on) (iv) foreign body granulomata can be nodular (sarcoidal, palisaded, or foreign body) or diffuse and mixed. Therefore, although pattern analysis is a helpful way to begin the evaluation of a slide, often evaluation of a granulomatous dermatitis must exceed routine light microscopy with hematoxylin-eosin staining. 2. If the infiltrate is nodular, is the pattern tuberculoid, sarcoidal, palisaded, or foreign body in type
I find FNAB efficacious when lymphadenopathy is suspected, (e.g., inflammation, benign lymphoepithelial disease, sarcoid, or lupus erythematosus). It has merit if lymphoma is a consideration and also for the elderly in whom confirmation of a benign neoplasm might affect surgical decision making. FNAB is beneficial in identifying recurrent neoplasia, benign or malignant.
They are multiple, they may be clustered or form a linear array. Solitary lesions usually occur on the extremities, and multiple lesions may occur on either the trunk or extremities. Pain is commonly reported, and may be precipitated by cold or pressure. The discomfort is described as sharp, burning, or throbbing (7). The differential diagnosis is wide for solitary lesions, and for multiple lesions may include such disparate entities as segmental neurofibromatosis, foreign body granuloma, dermatofibrosar-coma protuberans, and sarcoidosis.
Sarcoidosis is an unusual cause of a scrotal mass, and may rarely be the presenting site of disease in some patients. The pattern of multiple hypoechoic intratesticular masses associated with epididymal enlargement and heterogeneity is characteristic, although it may also be seen in lymphoma.
The efficacy of systemic retinoid therapy in a variety of dermatologic diseases, such as acne, psoriasis (pustular and erythrodermic types), and disorders of keratiniza-tion (ichthyoses, symmetric progressive erythrokeratoderma, Darier disease, pityriasis rubra pilaris, and palmoplantar hyperkeratosis), is well known. There are also reports of successful treatment of other dermatologic conditions, including disorders of epidermal differentiation (epidermodysplasia verruciformis, confluent and reticulated papillomatosis, and axillar granular parakeratosis) and inflammatory and immunodermatoses (atrophoderma vermiculatum, lichen planus, sarcoidosis, and granuloma annulare). Various synthetic retinoids have also been tried in the treatment of patients with different forms of cutaneous LE (CLE), and there are numerous reports of good responses to etretinate, acitretin, and isotretinoin (Duna and Cash 1995, Furner 1990b). Etretinate has been shown to be effective in the treatment of DLE,...
Because CHLE lesions are highly reminiscent of simple chilblains or pernio lesions (Viguier et al. 2001), one could question whether such patients have simple pernio that in the predisposed individual produces a Koebner's phenomenon resulting in DLE. The terms chilblain lupus and perniotic lupus have been used to describe such lesions. Unfortunately, the term lupus pernio has also been used for such lesions, although this term is more properly used to designate a form of cutaneous sarcoidosis (James 1992). For a positive diagnosis of CHLE, it has been proposed to establish two groups of major and minor criteria (Su et al. 1994). Major criteria include (a) cold-induced or cold-aggravated lesions in acral locations and (b) evidence of LE on histopathology or direct immunofluorescence. Minor criteria include (a) the coexistence of SLE or other manifestations of CLE, (b) positive response to LE therapy, and (c) negative results of cryoglobulin and cold agglutinin studies. The diagnosis of...
The synthesis of calcitriol is usually induced through low serum calcium or inorganic phosphate levels. However, changes in active vitamin D levels occur slower than those in PTH as the 'storage form' of vitamin D, 25-hydroxyvitamin D (calcidiol), needs to be converted into the biologically active form of the hormone, i.e., 1, 25-dihydroxyvitamin D. This conversion activation is achieved through the hydroxylation of 25-hydroxyvitamin D in its C1 position by CYP27B (a-hydroxylase). The activity of the latter enzyme, which is predominantly found in kidney parenchymal cells, is upregulated by PTH, and downregulated by plasma HPO4 levels. Conversely, calcitriol inhibits the secretion of PTH from the parathyroid glands.9 Measurement of serum 25 and 1,25 vitamin D levels may be useful in patients with osteoporosis, vitamin D deficiency, renal disease, hypercalcemia, and sarcoidosis.
Anti-La SSB occurs in approximately 15 of patients, and most have SS and may be diagnosed as having SS-SLE overlap syndromes. Anti-Ro SSA (without anti-La SSB) occurs in approximately 30 of patients with SLE without dry eyes or mouth. SS can precede LE by years, but LE can precede the appearance of SS also. Anti-Ro SSA antibodies etc found in polymyositis, scleroderma, and primary biliary cirrhosis, and association with SS is invariably (Venables 1988). The skin changes associated with SS include dry eyes, dry mouth, and dry genitals as major clinical symptoms. Other clinical symptoms include annular erythema, Sweet's syndrome-like lesions, vitiligo-like changes, sarcoidosis, and LP-like and amyloidosis nodularis-like symptoms (Ueki 1994, personal communication). Annular erythema is one of the prominent clinical features of both SS and SLE. Katayama et al. (Katayama et al. 1991) subdivided annular erythema into three types. Type I is an isolated annular erythema with an elevated and...
Sarcoidosis is more likely to affect the epididymis than the testis. More than one third of patients will have bilateral disease. Although discrete nodules will occasionally be seen, the appearance is more commonly one of heterogeneous enlargement. A diagnostic pattern that may be of use in a previously undiagnosed patient with hilar adenopathy (which could be either lymphoma or sarcoid), is to compare the testicular and the epididymal involvement in sarcoidosis, the degree of epididymal disease typically exceeds testis involvement, whereas in lymphoma the converse is expected.
Lash loss is also associated with infiltrative lesions such as sarcoidosis, lymphoma, and cutaneous neoplasms. Inflammatory processes including severe blepharitis can cause lashes to fall out, and chronic infections with the mite Demodex folliculorum, found in 10 to 15 of normal individuals, can also be associated with madarosis. Loss of lashes and facial hair has been reported as a complication of botulinum toxin for oromandibular dystonia, but this is exceedingly uncommon. Iodine plaque brachytherapy and external beam irradiation for choroidal tumors is a known cause of madarosis. Loss of lashes is a common finding in leprosy and ichthyosis. In some cases the loss of lashes can be factitious or idiopathic.
Or sutured together to increase bulk. Conchal cartilage is appropriate for contour improvement of the nasal tip or as an onlay graft. However, septum or rib cartilage is stronger, and provides more support. Previous extensive auricular cartilage harvesting may preclude harvesting of further conchal cartilage. Other contraindications include systemic diseases, such as collagen vascular disease, rheumatic disease, or immunologic disorders involving the auricle, such as lupus, polychondritis, sarcoidosis, and Wegener's granulomatosis. Careful preopera-tive analysis and questioning will guide the surgeon as to which ear should be used to harvest cartilage. Complete conchal cartilage removal may result in slight medialization of the pinna. Therefore, if asymmetric, the more prominent ear should be harvested. Furthermore, for the patient who has a history of sleeping on only one side of the head, the contralateral conchal cartilage should be harvested.
Sarcoid is a multisystem systemic granulomatous disease. Approximately 25 to 33 of the patients demonstrate cutaneous manifestations. The exact etiology of sarcoidosis remains unknown but there is a known association with increased cell-mediated immunity activity and an increase in CD4+ T-helper cells of the Th1 subtype after antigen presentation. Th1 cytokines are increased (including inter-leukin 2 and interferon), which ultimately leads to B-cell stimulation. Granuloma forming T lymphocytes and mono-cytes are in peripheral tissues leading to decreased delayed cellular immunity and lymphopenia with resultant anergy. The antigen responsible for initiating these events is unknown. Sarcoid is considered a diagnosis of exclusion both histologically and clinically. Infections and other systemic diseases must be excluded as the cause of non-caseating granulomas in the skin or other organ systems. However, the identification of a foreign body or infectious agent within the granuloma does...
The etiology and management of Bell's palsy is controversial, although the cause is almost certainly viral. Edema of the facial nerve near the stylomastoid foramen has been demonstrated. Most Bell's palsies recover completely and spontaneously within 6 weeks. If seen in the early stages, however, antiviral treatment and prednisolone orally should be given. Providing there is no general medical contraindication to steroids, a suggested dose of prednisolone is *20 mg q.d.s. five days 20 mg t.d.s. one day 20 mg b.d. one day 20 mg o.d. one day 10 mg o.d. one day. Physiotherapy maintains tone in the muscles during recovery and also has a place in the management of Bell's palsy. Bilateral facial palsy is very rare. These cases, however, require investigation to exclude underlying disease, e.g., Lyme disease, sarcoidosis.
Figure 2 Granulomas. (A) Tuberculoid granuloma. (B) Sarcoidal granuloma. (C) Palisaded granuloma. (D) Caseation necrosis within a granuloma. Figure 2 Granulomas. (A) Tuberculoid granuloma. (B) Sarcoidal granuloma. (C) Palisaded granuloma. (D) Caseation necrosis within a granuloma. Focal swelling within a tattoo. (B) Allergic Figure 7 Sarcoid. (A) Lupus pernio, red nodule on nose. (B-D) Round sarcoidal granulomas. sarcoidal granulomas containing tattoo (E) Fibrin within a tubercle. granules that appear histologically black. Focal swelling within a tattoo. (B) Allergic Figure 7 Sarcoid. (A) Lupus pernio, red nodule on nose. (B-D) Round sarcoidal granulomas. sarcoidal granulomas containing tattoo (E) Fibrin within a tubercle. granules that appear histologically black. Figure 9 Cheilitis granulomatosa. (A) Enlargement of lower lip. (B) Sarcoidal granulomas within mucosal skin. Figure 9 Cheilitis granulomatosa. (A) Enlargement of lower lip. (B) Sarcoidal granulomas within mucosal skin.
Other skin diseases with reduced LC numbers include chronic graft-vs-host disease, sarcoidosis, and acquired immunodeficiency syndrome (Aractingi et al. 1997, Belsito et al. 1984, Fox et al. 1983). In graft-vs-host disease, the reduced number of LCs may imply an attack by donor T cells (Suitters and Lampert 1983). The increase in CD1a-expressing epidermal DCs in inflammatory skin diseases such as atopic dermatitis and psoriasis, which has frequently been attributed to LC, is due to IDECs, a non-LC DC population (Wollenberg et al. 1999). Immunosuppressive treatment such as glucocorticosteroids or psoralen-UVA therapy may reduce LC numbers in the epidermis (Ashworth et al. 1989, Berman et al. 1983).
Glomus jugulare tumors are the most common tumors of the jugular foramen. Jugular schwannomas and meningiomas, although uncommon, are the other most common tumors affecting the jugular foramen, some series reporting the former and other series reporting the latter as the second most common.2'14'16 Chordoma,17'18 chondrosarcoma,16'19 chondroblastoma,16 cholesterol granuloma,20 epidermoid cyst,20 parotid neoplasm,16 adenocarcinoma,16 osteoblastoma,16 metastasis,21 plasmacytoma,22 sarcoidosis,23 fibrous dysplasia,24 chondromyxoid fibroma,25 non-Hodgkin's lymphoma,26 and amy-loidoma27 are among the other types of lesions reported in the jugular foramen.
The cardiomyopathies constitute a diverse group of diseases of both known and unknown etiology characterized by myocardial dysfunction, that is, diseases that are not the result of arteriosclerotic, hypertensive, congenital, or valvular disease.28 Cardiomyopathies can be grouped into three general categories dilated or congestive, hypertrophic, and restrictive-obliterative. The last category is usually rarely encountered by the forensic pathologist, since it deals with entities such as amyloidosis, hemochromatosis, sarcoidosis, glycogen storage disease, and hypereosinophilic syndrome, conditions not usually associated with sudden death and mostly of an infiltrative nature to the myocardium. The exception is sarcoidosis, which, while uncommon, is
Probably is delayed hypersensitivity reaction to a variety of antigens most common associations with streptococcal infections in children and sarcoidosis in adults other associations include tuberculosis, myco-plasma pneumonia, leprosy, coccidioid-omycosis, North American blastomycosis, histoplasmosis, inflammatory bowel disease, pregnancy, and Behcet's disease associated medications include oral contraceptives and sulfonamides
|Sarcoidosis Remission and Aden Protocol|
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