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1/164. Acute abdominal pain as a leading symptom for Degos' disease (malignant atrophic papulosis).

    We report a case of a 16-yr-old white female patient with acute abdominal pain due to visceral involvement of Degos' disease that required extensive small bowel resection. skin manifestations of her disease had been present for 2 yr before the correct diagnosis. She died as a result of central nervous system involvement from Degos' disease. ( info)

2/164. Acquired reactive perforating collagenosis associated with diabetes mellitus: eight cases that meet Faver's criteria.

    Reactive perforating collagenosis (RPC) is characterized by umbilicated papules with a central adherent keratotic plug. Histologically, this condition shows transepidermal elimination of altered dermal collagen bundles into a cup-shaped epidermal depression. The present paper describes eight patients with associated diabetes mellitus who meet the diagnostic criteria for the acquired form of RPC (ARPC). Although half of these patients underwent dialysis, the lesions did not tend to develop after dialysis. pruritus and the Koebner phenomenon were common, and histologically a microvasculopathy was noted in the dermis of all patients. We speculate that this disease is triggered by a cutaneous response to superficial trauma. Furthermore, this response acts synergistically with vasculopathy in the dermis, primarily in the case of diabetes mellitus. A secondary sign of ARPC may be degenerated collagen fibres as a result of transepidermal elimination. ( info)

3/164. Striae and acne following cardiac surgery in a child.

    We report a 13-year-old girl with extensive striae and an acneiform eruption following surgery for complex congenital heart disease. These findings were associated with elevated serum and urinary cortisol levels with loss of diurnal rhythm. The resolution of the eruption and the fading of her striae coincided in time with normalization of her blood parameters on day 72 postoperatively. We conclude that the cause of steroid excess in our patient was stress induced by the cardiac surgery and a complicated and protracted postoperative course. To our knowledge, this is the first report in the English language literature of skin changes due to endogenous hypercortisolaemia caused by intense physical and emotional stress. ( info)

4/164. methotrexate-induced papular eruption in patients with rheumatic diseases: a distinctive adverse cutaneous reaction produced by methotrexate in patients with collagen vascular diseases.

    BACKGROUND: In the past few years, low doses of methotrexate have been used for treatment of patients with rheumatoid arthritis and other collagen vascular diseases, mainly as an immunosuppressive and corticosteroid-sparing drug. Several cutaneous adverse reactions have been described in association with methotrexate therapy. OBJECTIVE: We describe the clinical and the histopathologic features of distinctive cutaneous lesions that appeared in 4 patients with acute bouts of collagen vascular diseases who were receiving methotrexate therapy. methods: We clinically and histopathologically evaluated cutaneous lesions caused by methotrexate therapy in 4 patients, 2 with systemic lupus erythematosus, 1 with rheumatoid arthritis, and 1 with Sharp syndrome. RESULTS: Clinically, lesions consisted of erythematous indurated papules most commonly located on proximal areas of the extremities. Histopathologic examination of these papules showed an inflammatory infiltrate mainly composed of histiocytes interstitially arranged between collagen bundles of the dermis, intermingled with few neutrophils. In some foci of deeper reticular dermis, small rosettes composed of clusters of histiocytes surrounding a thick central collagen bundle were seen. Cutaneous lesions showed a direct chronologic relationship with methotrexate therapy, and they disappeared when the drug was tapered or withdrawn and corticosteroids were increased. CONCLUSION: patients receiving low doses of methotrexate for acute bouts of collagen vascular diseases may experience characteristic cutaneous lesions with distinctive clinical and histopathologic findings shortly after methotrexate administration. We discuss the differential diagnosis with other dermatoses showing similar histopathologic findings that have been described in patients with collagen vascular diseases. ( info)

5/164. Ofuji's papuloerythroderma. An association with early gastric cancer.

    A case of Ofuji's papuloerythroderma in an 88-old man, affected by early gastric cancer, is reported. The association noticed in our case with early visceral malignancy is the starting point for the consideration of pathological relationships, nosological and prognostic evaluations in regard to this rare dermatosis which is distinctive of elderly people, and underlines the importance of a long-term follow-up of these patients. ( info)

6/164. panniculitis mimicking lupus erythematosus profundus: a new histopathologic finding in malignant atrophic papulosis (Degos disease).

    We present the case of a 57-year-old woman with a 1-year history of a cutaneous eruption clinically and histopathologically characteristic of Degos disease. In one of the two cutaneous biopsy specimens taken from the trunk lesions, the histopathology consisted of necrosis and sclerosis of the subcutaneous lobules, a finding that to our knowledge has not been previously described in the cutaneous lesions of Degos disease. Two months after cutaneous biopsies were taken, the patient developed ptosis and an episode of acute abdominal pain; intestinal perforation and many characteristic lesions of visceral Degos disease involving the entire small bowel were noted at laparotomy. ( info)

7/164. Late onset focal dermal elastosis: a distinct clinicopathologic entity?

    Two patients, aged 72 and 69 years, had skin lesions clinically resembling pseudoxanthoma elasticum (PXE). On histologic examination, the lesions showed an increased accumulation of normal-appearing elastic fibers lacking the abnormalities that characterize PXE. Similar lesions have been described previously in two elderly patients by Tojima et al. (1995). The clinical and histologic differential diagnosis of these lesions is discussed, and it is concluded that they represent a clinicopathologic entity with undetermined prevalence and etiopathogenesis. ( info)

8/164. purpura as a cutaneous association of sickle cell disease.

    A common chronic feature of sickle cell disease is the presence of painful, punched-out leg ulcers. Other cutaneous findings in patients with homozygous sickle cell disease have not been described in the literature. We present a case of a 50-year-old black woman with sickle cell disease who was admitted for acute onset of arm and hip pain. After admission she deteriorated clinically, with multiorgan failure and mental status changes. Examination of the skin revealed erythematous papules and plaques with scaly centers and purpura on the upper trunk. The clinical differential diagnosis was vasculitis versus sepsis. Skin biopsy of two representative lesions was performed. hematoxylin- and eosin-stained sections showed a superficial perivascular mixed inflammatory infiltrate with numerous eosinophils and extravasated erythrocytes, some of which exhibited bizarre morphology of sickled red blood cells. These findings indicated that the patient's cutaneous lesions, possibly multifactorial in origin, could be a component of her sickle cell crisis. This case is presented as an unusual one in which evaluation of erythrocyte morphology contributed to patient management and to emphasize the importance of examining erythrocyte morphology as a part of the histologic evaluation of stained tissue. ( info)

9/164. Transepidermal elimination of urate-like crystals: a new perforating disorder?

    We report two men who developed a transient perforating disorder characterized by transepidermal elimination of negatively birefringent needle-shaped crystals similar to monosodium urate. This striking clinical presentation has not previously been described and we propose that it be added to the group of diseases known as the primary perforating disorders. ( info)

10/164. Benign familial Degos disease worsening during immunosuppression.

    We describe a 61-year-old woman with skin lesions consistent with those found in Degos disease, both in clinical and in histological appearance. She had had several of these lesions for many years, as had her mother, sister and niece. In 1991, she underwent cadaveric renal transplantation and was treated with immunosuppression: prednisolone, azathioprine and cyclosporin. At that time, she developed many more characteristic skin lesions, and these were slightly larger and more noticeable than those she had had previously. She and the other affected family members appear to fit into the more benign subgroup of Degos disease, and it seems that her immunosuppression aggravated her cutaneous disease. ( info)
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