Amyloidosis-macular=الداء النشواني البقعي |
macular Amyloidosis
Amyloidosis is a generic term that signifies the abnormal extracellular tissue deposition of one of a family of biochemically unrelated proteins that share certain characteristic staining properties, including apple-green birefringence of Congo red–stained preparations viewed under polarizing light. Under electron microscopy (EM), amyloid deposits are composed of linear, nonbranching, aggregated fibrils that are 7.5-10 nm thick of indefinite length arranged in a loose meshwork.1 PathophysiologyAmyloid deposits in macular amyloidosis and lichen amyloidosis bind to antikeratin antibodies. These deposits contain sulfhydryl groups pointing to altered keratin as a source for these deposits. Apaydin et al found no differences in staining characteristics of cytokeratins between macular amyloidosis and lichen amyloidosis.3 Interestingly, in their study, all the cytokeratins detected in amyloid deposits were of basic type (type II). This may be because, in amyloidogenesis, acidic cytokeratins such as cytokeratin 14 are degraded faster than basic types.
HistoryMacular amyloidosis is a pruritic eruption that is variable in severity. Frequently, patients seek medical attention because of the hyperpigmentation. PhysicalMacular amyloidosis is a pruritic eruption consisting of small, dusky-brown or grayish pigmented macules distributed symmetrically over the upper back and, in some patients, the arms. Although a reticulated or rippled pattern of pigmentation has been emphasized as a characteristic and diagnostic feature of macular amyloidosis, in 2 case series, less than 50% of patients had this feature.
CausesConstant friction and rubbing with a nylon brush or towel may cause macular amyloidosis
Histologic FindingsMany stains can demonstrate amyloid deposits in the skin. The best known is the Congo red stain, which under polarizing light gives apple-green birefringence. Other stains include periodic acid-Schiff (PAS); methyl violet; crystal violet; various cotton dyes (eg, pagoda red, Sirius red); and the fluorescent dyes, thioflavin-T and Phorwhite BBU.
Medical Care
Surgical Care
MedicationThe goal of pharmacotherapy is to reduce morbidity. AntihistaminesThese agents act by competitive inhibition of histamine at the H1 receptor. They may control itching by blocking effects of endogenously released histamine. Chlorpheniramine (Chlor-Trimeton)Competes with histamine or H1 receptor sites on effector cells in blood vessels and respiratory tract.
Adult4 mg PO q4-6h; not to exceed 24 mg/d Pediatric<2 years: Not established
CNS toxicity increases with coadministration of other CNS depressants, tricyclic antidepressants, MAOIs, and phenothiazines
Documented hypersensitivity; asthma attacks; narrow-angle glaucoma; symptomatic prostate hypertrophy; bladder neck obstruction; stenosing peptic ulcer
PregnancyB - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals PrecautionsDrowsiness, dizziness, and dryness of mouth are the most common adverse effects; not for administration to premature or full-term neonates Diphenhydramine (Benadryl, Belix)For symptomatic relief of pruritus caused by endogenous release of histamine.
Adult25-50 mg PO tid/qid; not to exceed 400 mg/d Pediatric12.5-25 mg PO tid/qid or 5 mg/kg/d or 150 mg/m2/d PO divided tid/qid; not to exceed 300 mg/d
Potentiates effect of CNS depressants; because of alcohol content, do not give syr dosage form to patient taking medications that can cause disulfiramlike reactions
Documented hypersensitivity; MAOIs
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus PrecautionsDrowsiness, dizziness, and dryness of mouth are the most common adverse effects; may exacerbate angle-closure glaucoma, hyperthyroidism, peptic ulcer, and urinary tract obstruction Topical anti-inflammatory agentsThis industrial solvent has been used with mixed results. Dimethyl sulfoxide (Rimso-50)May help relieve symptoms. DMSO, an oxidation product of dimethyl sulfide, is an exceptional solvent possessing a number of commercial uses. Not an FDA-approved indication.
Adult50% solution in water applied topically PediatricNot established
None reported
Documented hypersensitivity
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus PrecautionsGarliclike breath odor and taste in the mouth due to excretion of small amount of DMSO as dimethyl sulfide (usually lasts only 24-48 h)
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