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1/5. Palisaded neutrophilic and granulomatous dermatitis: an unusual cutaneous manifestation of immune-mediated disorders.

    OBJECTIVE: Palisaded neutrophilic and granulomatous dermatitis (PNGD) is an uncommon skin eruption most often associated with rheumatoid arthritis and other immune-mediated diseases. We present 4 cases to familiarize rheumatologists with the clinical presentation and histopathology of PNGD. methods: We report 4 cases to illustrate the clinical and histologic spectrum of this rare skin disease found in rheumatologic patients. The disease pathogenesis and treatment options are discussed. RESULTS: All 4 patients presented with symmetric erythematous-to-violaceous papules and plaques that, upon biopsy, revealed a dermatitis composed of variable numbers of histiocytes and neutrophils. Some cases responded to topical corticosteroid treatment or to dapsone, whereas others resolved spontaneously. CONCLUSIONS: PNGD is a rare cutaneous finding in patients with a variety of immune-mediated systemic diseases, most often rheumatoid arthritis. It is a benign condition that may spontaneously remit or may respond favorably to topical corticosteroids or dapsone.
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2/5. Familial occurrence of axillary papular mucinosis.

    Primary cutaneous mucinosis encompasses a wide range of different skin conditions with circumscribed, follicular or diffuse manifestation. For the first time, to our knowledge, we report on the occurrence of so-called "atypical" localized papular mucinosis in the axillary pits of a mother and her (identical) twin daughters presenting as asymptomatic soft minuscule papules. This unique observation allows us to define an autosomal dominant inheritance pattern with a high level of penetrance for this rare skin disease.
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keywords = skin disease
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3/5. Valdecoxib-associated acute generalized exanthematous pustulosis.

    AGEP (acute generalized exanthematous pustulosis) is a relatively rare exfoliative skin syndrome consisting of generalized eruption of pustules in response to medication or infection. Because AGEP may have other systemic manifestations, such as renal failure, hyperthermia, lab abnormalities, and/or hemodynamic instability, it is important to make the distinction between AGEP and other life-threatening generalized skin diseases, such as toxic epidermal necrolysis (TEN). Here we present a case of AGEP in response to valdecoxib, which has not previously been described in the literature. The patient presented with profound hypotension requiring fluid and vasopressor support and was referred to the burn service for treatment of TEN, but her skin lesions were inconsistent with this diagnosis. A dermatology consult was obtained and suggested a diagnosis of AGEP, which a biopsy confirmed. TEN and AGEP present with similar history, types of associated drugs, and immunology. Both can be associated with antibiotics, non-steroidals, and anticonvulsants, but AGEP is more frequent with aminopenicillins, while TEN is associated more often with sulfonamide antibiotics. Both disorders have a proposed T cell-mediated immune response, but they differ in the mechanism. A description of valdecoxib and its role as a sulfonamide in producing cutaneous reactions is also provided.
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keywords = skin disease
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4/5. Clear cell papulosis: case report and literature review.

    Clear cell papulosis is a newly described skin disease characterized by multiple white papules. Histopathologically, diagnostic clear cells were seen among the basal cells of the epidermis. We report clear cell papulosis on the lumbar area and buttocks of a 1-year-old girl.
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keywords = skin disease
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5/5. Terrien's marginal degeneration associated with erythema elevatum diutinum.

    PURPOSE: To describe a patient with Terrien's marginal degeneration associated with a chronic skin disease, erythema elevatum diutinum. methods: A 27-year-old woman with peripheral thinning of her left cornea associated with adjacent conjunctival and episcleral inflammation was clinically examined. RESULTS: Episodes of worsening skin lesions were associated with development of inflammation in her left eye. Administration of sulfones was effective for reducing both skin and ocular inflammation. CONCLUSION: erythema elevatum diutinum should be considered an underlying systemic disease of Terrien's marginal degeneration.
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keywords = skin disease
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