Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Over the past nine years (from 1981 to 1989), four patients with perianal Paget's disease were treated. All were male with an average age of 58.5 years. Clinically, perianal Paget's disease manifests as a slowly enlarging eczematous, and sharply demarcated perianal skin rash that may be oozing or itching. In the characteristic pathology finding, Paget's cells appear as large, rounded signet-ring cells with abundant mucin stain positive cytoplasm in the basal layer of the acanthotic epidermis. All but one, who suffered from primary sweat gland carcinoma, had underlying rectal adenocarcinoma. The first two cases expired soon after a delayed diagnosis of terminal underlying malignancy. Only in the later two cases was there a preoperative suspicion of perianal Paget's disease. There is often a delay in diagnosis due to clinical ignorance. Patients with persisting perianal skin rash should be biopsied frequently. If perianal Paget's disease is diagnosed, the underlying malignancy should be surveyed and managed thoroughly.
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PMID:Perianal Paget's disease--report of 4 cases. 217 40

37 keloid patients were treated with triamcinolone intralesionally every 15 days, in all 6 inoculations. Histologically after the treatment we observed alterations in dermal vascularization, in morphology, in color characteristics and in the arrangement of the collagen bundles. The disappearance of mucin and the presence of palisade granulomas was observed in some cases probably because of the corticoid deposited. There was regression of pruritus and pain in all the patients. We believe that the intralesionally infiltration of corticoid is even today one of the best methods of treatment, and it may also be effected together with other technics.
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PMID:[Clinico-pathological evaluation of keloids treated with intralesional injections of corticoid]. 330 1

We report the case of a patient who, while on long-term hemodialysis (HD), developed nephrogenic fibrosing dermopathy, a newly described sclerosing skin disorder. This disorder is characterized by thickened, hardened skin with brawny hyperpigmentation and raised plaques. The most common patient complaints are pruritus and dysesthesia. The extremities are predominantly involved with sparing of the torso and face. Dysfunction of internal organs has not been described, distinguishing it from other fibrosing conditions such as scleroderma. The skin biopsy is characterized by haphazardly arranged dermal collagen spindle cells in the reticular dermis. Extensive mucin deposits are interposed between collagen bundles and there are an increased number of fibroblast-type cells. In contrast to scleroderma, inflammatory cells are generally absent. Corticosteroid therapy can be tried, but in our patient was of no benefit.
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PMID:Nephrogenic fibrosing dermopathy: an unusual skin condition associated with kidney disease. 1275 92

A 52-year-old black woman presented with a 5-year history of gradual swelling and slowed hair growth involving the vertex and both parietal regions of the scalp. Gradually, the swelling progressed to involve the entire scalp, only sparing a peripheral crown. She reported no history of trauma or medications. Slight pruritus of the involved area was the only accompanying symptom. There was no family history of a similar condition. Her past medical history included surgery for ovarian cysts, 10 years previously, and cholelithiasis. Physical examination revealed diffuse hair thinning and alopecia, more prominent along the vertex and parietal regions (Fig. 1a). There was no evidence of scalp inflammation, scaling, or increased hair fragility. The scalp was mildly tender on palpation and had a boggy, spongy consistency. The hairs which still remained in the involved areas were thin, short, and soft (Fig. 1b). The involved area was slightly hypopigmented when compared with adjacent noninvolved scalp. The rest of the physical examination was within normal limits. No abnormalities of the hair shaft were observed on microscopic examination of several plucked hairs. Laboratory investigations, including a complete blood cell count, blood chemistry, urinalysis, sedimentation rate, antinuclear antibodies, and serologic tests for syphilis, hepatitis B and C virus, and human immunodeficiency virus, were negative. A computed tomography scan of the skull demonstrated diffuse and regular thickening of subcutaneous fatty tissue of the scalp, disclosing a maximum scalp thickness of 15 mm at the vertex (Fig. 2). The biopsy from the vertex revealed a normal epidermis and dermis, with diffuse loss of hair follicles. The most striking feature consisted of a large increase in thickness of the subcutaneous fatty tissue (Fig. 3a). Pre-existing hair follicles were replaced by vertical fibrous tracts of lamellar fibroplasia with no inflammatory infiltrate (Fig. 3b). Adipocytes showed a normal size and shape, but the connective tissue septa, which are normally present separating the subcutaneous tissue into fat lobules, were lacking, and subcutaneous fatty tissue consisted of a continuous and diffuse sheet of mature adipocytes (Fig. 3c). Orcein stain revealed normal contents of elastic fibers with foci of condensation at the sites of disappeared pre-existing hair follicles (Fig. 3d). Colloidal iron and Alcian blue (pH 2.5) stains revealed no mucin deposits.
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PMID:Lipedematous alopecia: an uncommon clinicopathologic variant of nonscarring but permanent alopecia. 1847 57

Extramammary Paget's disease (EMPD) is considered to be an intraepithelial adenocarcinoma. Typically involved anatomical sites are the vulvar, perianal, perineal, scrotal and penile regions. Clinically, the lesions present as well-defined, moist, erythematous plaques usually accompanied by pruritus. An unusual feature of EMPD is its association with cutaneous, adnexal-structure adenocarcinomas and its association with internal malignancies. Histopathological examination shows epidermal acanthosis and elongated rete ridges. Paget's cells are large intraepidermal cells with a large nucleous and abundant pale cytoplasm. Recent studies of perianal and vulvar EMPD have described distinct immunohistochemical subtypes termed cutaneous and endodermal. Cutaneous EMPD is characteristically positive for cytokeratin (CK)7, negative for CK20, and positive for gross cystic disease fluid protein (GCDFP)15+, whereas endodermal EMPD shows a CK7+ CK20+ GCDFP15- phenotype. Surgery remains the treatment of choice, with either wide surgical excision or Mohs' micrographic surgery. We present a case of EMPD with an underlying carcinoma, which combined immunohistochemical findings suggestive of the cutaneous subtype (positive for CK7, GCDFP15, mucin (MUC)1, human epidermal growth factor receptor (HER)2/neu positive) and the endodermal subtype, frequently associated with internal malignancy (CK20, MUC2, CDX-2 positve); however, our patient had no associated internal malignancy.
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PMID:Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcome. 1848 20

A 62-year-old woman with hypothyroidism presented with a seven-year history of paresthesias, itching, and edema of the skin. Physical examination showed indurated, edematous plaques on the lower extremities. A biopsy specimen showed increased mucin deposition that was consistent with myxedema, and monoclonal IgM was observed on immunofixation. The constellation of findings, which included paresthesias, endocrinopathy, monoclonal gammopathy, and skin changes was consistent with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome, which is a rare multisystemic disease that is associated with an underlying plasma-cell dyscrasia.
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PMID:POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes). 1989 17

Folliculosebaceous cystic hamartoma (FSCH) is a cutaneous hamartoma of pilosebaceous origin that usually occurs on the face. We present what we believe is only the second reported case of FSCH of the external auditory canal, and the first in an adult. The patient was a 59-year-old woman who presented with a 4-year history of a firm nodule on the left external auditory canal that had caused hearing loss, pruritus, and pain. The lesion was excised, and histopathologic examination identified infundibular cysts, hyperplasia of the sebaceous lobules, and a large amount of mucin, features that are consistent with FSCH. One year postoperatively, the patient was asymptomatic, and no evidence of recurrence was noted.
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PMID:Folliculosebaceous cystic hamartoma of the external auditory canal in an adult. 2235 38

A 50-year-old diabetic female presented with highly pruritic vesicles and excoriated lesions over the anterior aspect of both lower legs. The lesions were recurrent over the last two years. She received a lot of medications with partial response. Hb A1c was 10.8% (normal up to 7%). CBC showed microcytic, hypochromic anemia. Serum zinc, folate, IgE, TSH and T4 were all within normal ranges. Biopsy showed epidermal separation secondary to keratinocyte necrosis and minimal monocytic, perivascular infiltrate. Direct immunofluorescence was negative for intraepidermal and subepidremal deposition of immunoglobulin. The dermis was positive for mucin deposition stainable by both PAS and Alcian blue while it was negative for Congo red and APC immunoperoxidase staining for amyloid material. In conclusion, the case was diagnosed as bullosis diabeticorum by distinctive clinical and pathological features and after exclusion of other possible differentials. Pruritus was partially controlled by topical potent steroid and the case was resolved spontaneously after eight months.
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PMID:Pruritic vesicular eruption on the lower legs in a diabetic female. 2419 86

Lichen planopilaris (LPP) is a rare form of cicatricial alopecia that has occasionally been reported in children. Because of the limited number of patients reported, little information is available about demographic characteristics, clinical presentation, or treatment options for these patients. A retrospective chart review of LPP cases in patients under 18 years of age from 1976 to 2013 was performed to further define clinicopathologic features of pediatric LPP. Four pediatric LPP patients ages 13 to 16 years were identified (three male, one female). One patient had scalp pruritus and one had other cutaneous findings of lichen planus (LP). Perifollicular scale and scarring were the most common physical examination findings, although changes mimicking those of alopecia areata were observed. Three patients were treated with topical or intralesional steroids. One patient was treated with minocycline. Histopathologic findings included perifollicular interface and perifollicular fibrosis in all cases. There was focal interfollicular interface in two cases and mild dermal mucin in one case. LPP is exceedingly rare in children. It may be misdiagnosed as alopecia areata in children because of the lack of symptoms and other features of LP. There should be a high index of suspicion for LPP in children with alopecia that is unresponsive to standard treatment or who have findings that are atypical for more common childhood alopecias.
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PMID:Pediatric Lichen Planopilaris: Clinicopathologic Study of Four New Cases and a Review of the Literature. 2605 19

Scleromyxedema is a rare, chronic and persistent idiopathic disorder characterized by a generalized papular eruption due to dermal mucin deposition with an increase in dermal collagen. Patients usually have associated paraproteinemia. We describe the case of a 59-year-old gentleman with features of scleromyxedema, who had severe pruritus, scalp involvement, unrestricted mobility and associated peripheral eosinophilia, but no monoclonal gammopathy.
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PMID:Scleromyxedema: An Atypical Case. 2612 Jan 84


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