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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cutaneous lesions are present in up to 40% of patients with primary and myeloma-associated systemic amyloidosis and occur as a result of tissue deposition of immunoglobulin light chain material derived from a circulating paraprotein. The occurrence of waxy, purpuric mucocutaneous lesions provides a crucial early pointer to underlying occult plasma cell dyscrasia; the combination of the symptoms of the carpal tunnel syndrome, macroglossia, and specific mucocutaneous lesions is highly characteristic. Although secondary systemic (reactive) amyloidosis rarely gives rise to clinically evident cutaneous lesions, it may be etiologically related to a number of chronic dermatoses. Lesions of nodular primary localized cutaneous amyloidosis are indistinguishable from those of primary and myeloma-associated systemic amyloidosis, and they result from local plasma cell infiltration. Macular and papular (lichen amyloidosus) variants of primary localized cutaneous amyloidosis may have a familial or racial basis and are characterized by a tendency for keratinocytes to undergo filamentous degeneration and apoptosis. The prognosis of patients with plasma cell dyscrasia-related systemic amyloidosis remains poor, since there is little response to therapy with cytotoxic agents, colchicine, or dimethylsulfoxide. Colchicine is the drug of choice in the prevention and treatment of the renal amyloidosis associated with familial Mediterranean fever, and dimethylsulfoxide may be useful in the management of patients with secondary systemic amyloidosis. Macular amyloid and lichen amyloidosus generally follow a chronic course with intractable pruritus; there have been isolated reports of the beneficial effect of dermabrasion, topical dimethylsulfoxide, and therapy with the aromatic retinoid, etretinate.
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PMID:Amyloid and amyloidosis. 327 77

The purpose of this presentation is to offer a classification of psychocutaneous disease and to examinine factors which result in itching and scratching becoming chronic. Pruritus is a very common sensation in dermatoses. All forms of itching, whatever the cause, may be intensified by emotional stress. The patient sample of my own study consisted of seven diagnostic groups, giving a total of 99 patients. The dermatoses were: dermatitis herpetiformis, lichen ruber planus, chronic eczema, atopic eczema, neurodermatitis circumscripta, lichen corneus obtusus and pruritus psychogenes. The principal findings were as follows: 1. Many of the different mechanisms by which skin diseases became chronic had a psychological background. 2. Psychiatric disturbances were clearly more common than in the average population. Possibilities of psychiatric treatment were evaluated. The results confirm the hypothesis that in those skin diseases in which the disease mechanism is not yet sufficiently understood, psychosomatics is of considerable importance or offers an easier way to explain the mechanism.
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PMID:Psychosomatic aspects in dermatoses. 366 7

This study is based on clinical findings in 160 patients with skin-limited amyloidosis: 151 macular, 8 biphasic and 1 lichen amyloidosis. 82.5 p. 100 of the patients were women and 17.5 p. 100 were men. 95 p. 100 of the patches were located in the back and 64 p. 100 in the suprascapular region. No less than 88 p. 100 of the patients had pruritus on the patches or on other sites. 75 p. 100 had allergic diseases or a history of allergy, and 45 p. 100 had non-allergic pruriginous diseases. Signs of scratching were noted in most of the patients. The principal causes of pruritus were dermographism, photodermatitis and dermatitis. Macular amyloidosis is now considered as secondary to scratching motivated by pruriginous diseases and not as a primary condition as hitherto described. The term "scratch amyloidosis" is proposed for macular and lichenoid amyloidosis, and the existence of a "preamyloidotic" histological stage is described.
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PMID:[Origin of macular amyloidosis. Apropos of 160 cases]. 367 63

Twenty patients with vulvar dystrophy (19 Lichen sclerosus, 1 Lichen ruber planus) were treated for 3 months with etretinate (Tigason) with an initial dose 0.54 mg/kg/day, maintenance dosage 0.26 mg/kg/day. All the patients had been unsuccessfully treated previously with topical oestrogen and corticosteroids. The therapeutic effect of etretinate on the subjective and objective symptoms of the disease was excellent. In most of the patients the pruritus and burning symptoms diminished within 2 weeks of treatment, and after 3 months the grade of symptoms was lower in 95% of cases. Clinically, a decrease in severity was achieved in 93% of cases among the group with severe vulvar dystrophy. The therapeutic effect of etretinate is strongly anti-inflammatory and it has a powerful effect on the epidermal tissues. According to the latest studies, etretinate also has a strong immuno-modifying effect on the epidermal cells. The secondary inflammatory changes, such as excoriatia, fissures and superinfections disappeared. In the histopathological follow-up hyperkeratosis in the stroma diminished and the inflamed cells and connective tissue normalised after 3 months of treatment. Side effects included cheilitis, dryness of mucous membranes and slight loss of hair. It must be taken into account that etretinate may cause liquid metabolism disturbances, particularly among risk factor patients (diabetes, obesity etc.). In our experience the best results to date in the treatment of vulvar dystrophy can be achieved with etretinate. Due to the teratogenic effect of etretinate, a reliable method of contraception must be used by fertile women of childbearing age.
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PMID:[Oral treatment of vulvar dystrophy with an aromatic retinoid, etretinate]. 371 Jan 21

Male HLA-identical twins with numerous congenital abnormalities were discovered to have lichen amyloidosus in similar distribution on their chests and abdomens. Pruritus was absent in both. The finding of identical distribution of nonpruritic lesions was previously reported in familial lichen amyloidosus. This suggests that a subset of these patients have a nevoid condition in which a circumscribed patch of keratinocytes undergoes filamentous degeneration and may appear in identical distribution in family members in the absence of pruritus.
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PMID:Primary cutaneous amyloidosis: identically distributed lesions in identical twins. 372 2

Macular amyloidosis, a variant of lichen amyloidosus, is an uncommon cutaneous eruption that presents with moderate to severe pruritus associated with areas of hyperpigmentation. Effective modalities for the treatment of this condition have been lacking. A case of macular amyloidosis is reported in which successful management of pruritus was obtained using ultraviolet B (UVB) therapy. Mechanisms of UVB action in this condition are discussed.
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PMID:Macular amyloidosis: treatment with ultraviolet B. 373 72

When a decision is being made on the therapy of atopic eczema, the complex pathogenetic interactions involved in this disease have to be taken into consideration. The acute inflammatory changes respond to short-term glucocorticoid steroid treatment (topical); long-term steroid therapy should be avoided. In chronic lichen-type lesions, non-steroid topical applications can be helpful. Frequent acute infections of the skin require the application of antimicrobials. Intense pruritus is treated by antihistamines (possibly also H2-antagonists). Due to the well-known psychosomatic influence in this disease, careful counseling of the patient (and if a child, of the family) is necessary. Pathogenetically relevant allergic reactions of the immediate type can be treated successfully in some cases by hyposensitization. Prophylactic measures include allergen avoidance (climate therapy, no pets, well-established food allergens etc.). Possible new perspectives may be seen in the development of agents acting at the disturbed T-cell regulation, as well as by the introduction of mast-cell-blocking substances. The basis of every therapeutic approach in atopic eczema is, however, intensive skin care, using emollients and oil baths, especially during the remission phase.
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PMID:[Therapy of atopic eczema]. 643 91

25 cases of primary cutaneous amyloidosis are studied. 16 patients had macular amyloidosis (MPA) and 9 lichen amyloidosus (LPA). gamma-Globulins were increased in 50% of the patients. IgG and IgA were increased in the serum of 5 and 3 patients with MPA and 4 and 2 patients with LPA, respectively. Volume of amyloid deposits was similar in both forms. By direct immunofluorescence we demonstrated IgG in the amyloid deposits of 21 of the 25 cases and C3 in 13; IgM was present in 9 cases of MPA and in 3 cases of LPA. MPA was more frequent than LPA; histologically, it was impossible to distinguish MPA from LPA; correlation between serum levels of gamma-globulins and their presence in amyloid deposits was weak; MPA and LPA seem to be distinct clinical manifestations of the same disease and itching does not cause transformation of MPA in LPA.
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PMID:Primary cutaneous amyloidosis: clinical, laboratorial and histopathological study of 25 cases. Identification of gammaglobulins and C3 in the lesions by immunofluorescence. 698 9

Seven patients with lichen amyloidosus were treated with dermabrasion during the last seven years. All patients have had follow-up examinations for at least five years (one patient for seven years). The main symptom, itching, disappeared in all cases, and the clinical results were satisfactory, except for mild hyperpigmentary and depigmentary changes in the treated areas.
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PMID:Dermabrasion for lichen amyloidosus. Report of a long-term study. 708 19

In current textbooks of dermatology, lichen amyloidosus is said to be a papular, intensely pruritic type of amyloidosis of unknown aetiology. When this concept was developed, there was no way of discriminating between the different types of amyloid. It is now known that amyloid in lichen amyloidosus is not derived from immunoglobulins or serum proteins, as it is in systemic amyloidoses, but from keratin peptides of necrotic keratinocytes. Even several years ago, chronic scratching was invoked as a possible cause of damage to keratinocytes in lichen amyloidosus. In support of this hypothesis, four cases are presented. Apart from amyloid in the papillary layer, all biopsy specimens revealed histopathological signs of chronic scratching (epithelial hyperplasia with hypergranulosis and compact orthokeratosis, coarse collagen in vertical streaks in the stratum papillare), such as are also found in prurigo nodularis and lichen simplex chronicus. Lichen amyloidosus is considered to be a variant of these conditions. Just as in prurigo nodularis and lichen simplex chronicus, pruritus seems to be the cause and not a symptom of the papular skin lesions. Consequently, treatment of lichen amyloidosus should not be directed at removing amyloid, but at improving the pruritus.
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PMID:[Lichen amyloidosus--disease entity or the effect of scratching]. 775 42


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