Cases reported "Skin Diseases"

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1/3572. Dystrophic calcinosis cutis in subacute lupus.

    Dystrophic calcinosis cutis is known to be associated with various connective tissue disorders but to the best of our knowledge has never been reported in subacute cutaneous lupus erythematosus (SCLE), a distinctive cutaneous subset in the spectrum of lupus erythematosus. It occurs without calcium and phosphorus metabolic abnormalities and may be localized or generalized. We report a patient with SCLE who developed calcinosis cutis and had normal serum calcium and phosphorus levels and, interestingly, a normal concentration of blood ionized calcium. This latter, which represents the active form in the total amount of blood calcium, is a parameter only rarely assessed in patients with dystrophic calcinosis cutis. Thus, other pathogenic factors should be investigated to clarify the pathophysiology of the dystrophic type of calcification. ( info)

2/3572. Hypergranulotic dyscornification: a distinctive histologic pattern of maturation of epidermal epithelium present in solitary keratoses.

    Hypergranulotic dyscornification is an appellation proposed to designate a newly recognized distinctive pattern of epidermal maturation that is analogous to other epithelial reaction patterns such as epidermolytic hyperkeratosis, focal acantholytic dyskeratosis, cornoid lamellation, pale-cell acanthosis, and follicular mucinosis. The name "benign hypergranulotic keratosis with dyscornification" is proposed to specify solitary keratoses with digitated epidermal hyperplasia that exhibit this exceptional pattern of cornification. This abnormal type of cornification is characterized by hypergranulosis. A pale-staining basophilic substance is present intercellularly within the upper spinous layer and the hyperplastic granular layer. Overlying the thickened granular layer in foci at tips of epidermal papillations are orthokeratotic mounds of large, dull, eosinophilic staining corneocytes that are sharply demarcated from the thickened granular layer. Basophilic keratohyalin granules are focally retained within these corneocytes. There is overlying compact orthokeratosis that extends across the entire lesion. The compact orthokeratosis is slightly basophilic, and lies below a laminated and basket-weave orthokeratotic stratum corneum. There is a predominantly lymphocytic infiltrate at the base of these neoplasms with some spongiosis. There is parakeratosis focally present in the stratum corneum overlying these individual areas of abnormal cornification. The histopathologic and clinical findings in eight lesions that exhibit hypergranulotic dyscornification, a heretofore undescribed unique pattern of epidermal cornification, are presented. ( info)

3/3572. Clear cell acanthoma presenting as polypoid papule combined with melanocytic nevus.

    We report on a case of clear cell acanthoma that developed above a preexisting melanocytic nevus. Although melanocytic nevi can be complicated by the presence of several different types of epithelial or appendageal tumors, there have been no reported case of clear cell acanthoma combined with a nevus. Interestingly, in this case the clear cell acanthoma had a polypoid configuration, an unusual finding. ( info)

4/3572. Solitary keratoacanthoma of the nipple in a male. Case report.

    A heretofore undescribed localization for solitary keratoacanthoma, namely the skin of the nipple, is reported. The tumor developed in a 69-year-old man who had suffered a trauma in the thorax near the left nipple 5 months before. A possible origin in mammary ducts, considering the breast as a modified sweat gland, is discussed. ( info)

5/3572. Nodular amyloidosis treated with a pulsed dye laser.

    BACKGROUND: Nodular amyloidosis is a rare form of primary localized cutaneous amyloidosis which is characterized by single or multiple nodules located on the extremities, trunk, genitalia, or face. OBJECTIVE: To determine the clinical and histologic response of nodular amyloidosis to pulsed dye laser treatment. methods: biopsy-proven amyloid nodules were treated with a 585-nm pulsed dye laser (average fluence 5.25 J/cm2; 10 mm spot) at 6- to 8-week time intervals. Clinical and histologic examination of laser-irradiated nodules were performed before and 6 weeks after the final laser treatment. RESULTS: Clinical improvement in the color, size, and pliability of nodules was noted and maintained for 6 months. Histologic examination revealed decreased inflammation and improvement in dermal collagen after laser irradiation. CONCLUSIONS: Since amyloid fibrils may be formed in association with dermatan sulfate-an essential matrix component in collagen fiber formation, it is postulated that the improvement seen in amyloid nodules after pulsed dye laser treatment may be attributed to a mechanism similar to that seen with hypertrophic scars. ( info)

6/3572. skin eruption with gabapentin in a patient with repeated AED-induced Stevens-Johnson's syndrome.

    skin eruptions have been reported with the use of all antiepileptic drugs and there is a significant risk of cross-reactivity between these agents in causing serious eruptions such as Stevens-Johnson's syndrome. Gabepentin is usually considered a safe agent for patients with a previous history of drug allergies and there have been no cases of skin eruption reported to the gabapentin post marketing surveillance. We report a patient who had severe Stevens-Johnson's syndrome induced by phenytoin and later by carbamazepine. Subsequent use of gabapentin also resulted in a skin eruption which was limited to the lower extremities but without systemic or mucosal involvement. This case suggests that patients with a strong history of drug-induced idiosyncratic reactions may experience such reactions to gabapentin as well. ( info)

7/3572. Severe Lhermitte-Duclos disease with unique germline mutation of PTEN.

    Germline mutations in the PTEN gene have recently been identified in some individuals with Cowden disease (CD), Lhermitte-Duclos disease (LDD), and Bannayan-Zonana syndrome. We report on a patient with CD and LDD in whom a unique de novo germline missense mutation is present in the PTEN gene. Direct sequence analysis detected a transitional change (T-->C) at nucleotide 335, resulting in substitution of the amino acid proline for leucine. The mutation is in exon 5, which has been proposed as a "hot-spot" for germline mutations. Comparison of this patient's clinical course with the previously reported cases of CD and LDD shows more extensive and more severe clinical findings than reported previously. Findings in this patient contribute to the current understanding of germline PTEN mutations and clinical outcome. ( info)

8/3572. Juvenile chronic myelogenous leukemia, neurofibromatosis 1, and xanthoma.

    The triple association of leukemia, xanthomatous skin lesions, and neurofibromatosis 1 (NF) was first described by Royer et al. in 1958. Most of the leukemias were of the juvenile chronic myelogenous type (JCML). We describe a 7-year-old male child with xanthoma, neurofibromatosis 1, and juvenile chronic myelogenous leukemia. His mother also had NF1. We suggest that the presence of xanthomas and NF1 in a young child should raise awareness of the possible development of JCML, especially in patients with a family history of NF1. ( info)

9/3572. Anticoagulant-induced skin necrosis in a patient with hereditary deficiency of protein s.

    skin necrosis is a rare but debilitating complication of treatment with vitamin k antagonist anticoagulants such as warfarin. A clinically similar syndrome has been reported less frequently with heparin therapy. We recently managed a thirty-year-old female patient who developed skin necrosis on her left lower extremity while on warfarin for postpartum DVT. The lesions started to develop 48 hr after stopping heparin therapy. Discontinuation of warfarin and reinstitution of heparin was complicated by a rapid decrease in platelet count consistent with heparin-induced thrombocytopenia (HIT) and its associated risk of platelet activation and thrombosis. The diagnosis was supported by the identification of antibodies against heparin/platelet factor 4 complexes in the patient's serum. The platelet count recovered and the patient improved after switching to therapy with the heparinoid danaparoid. Evaluation for a hypercoagulable state revealed a partial deficiency of protein s, a condition that previously was identified in two of her family members. It is not clear if this patient suffered from warfarin-induced skin necrosis, a manifestation of heparin-mediated platelet activation, or a complex condition in which both drugs contributed. HIT may affect 1-3% of patients who receive unfractionated heparin, and this case raises the possibility that heparin may contribute to, or cause, some episodes of skin necrosis attributed to warfarin. Because many patients who develop warfarin-induced skin necrosis have been treated initially with heparin, it would seem prudent to consider HIT in these situations. ( info)

10/3572. Cutaneous protothecosis. Successful treatment with amphotericin b.

    A patient had cutaneous protothecosis because of the alga-like organism, prototheca wickerhamii. in vitro sensitivity tests showed that the organism was sensitive to amphotericin b, and was treated successfully with this polyene antibiotic. As with treatment of some fungal infections, a clinical response was achieved when therapy with low doses of amphotericin b was given during a short period of time. The basis of the amphotericin b response may have been due to a combination of its immunostimulatory and antibiotic properties. ( info)
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