1/16. leukemia cutis in a patient with chronic neutrophilic leukemia.Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative disorder. Less than 50 cases have been reported. We report the first case of CNL with an associated leukemia cutis. CNL was diagnosed in a 74-year-old white woman in 1998, based on neutrophilic infiltration of the bone marrow and absence of the philadelphia chromosome. The patient presented to the dermatology service in August 1998 with a 2-week history of a pruritic eruption on the arms, hands, and legs. physical examination revealed red to violaceous plaques on both thighs and knees, in addition to purpuric patches and plaques on the dorsal hands, arms, and legs. leukemia cutis was demonstrated on biopsy specimens of several lesional sites. The eruption progressed, despite treatment with topical and systemic corticosteroids. Treatment with systemic chemotherapy did affect partial resolution of the eruption, with parallel decreases in bone pain and white blood cell count, but the disease progressed and the patient ultimately died 5 months after her initial skin findings. Only one other case of CNL with dermatologic manifestations has been reported, CNL associated with a reactional neutrophilic dermatosis. Comparison to and differentiation from this case is discussed. The importance of distinguishing the specific infiltrates of leukemia from the nonspecific infiltrates of reactional dermatoses, such as Sweet's syndrome, is illustrated.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
2/16. colchicine treatment in a patient with neutrophilic dermatosis associated with rheumatoid arthritis.Neutrophilic dermatoses (ND) are characterized by epidermal and dermal infiltrates of polymorphonuclear leukocytes (PMN), without any infectious or other detectable agents as a cause. We describe a case of ND, which very rapidly improved upon colchicine treatment. The patient was a 67-year-old female with an 8-month history of dusky-red, tender, swollen plaques and nodules with superimposed vesicles and bullae on the buttocks, hands, and ankles associated with rheumatoid arthritis. The diagnosis of ND was made on the basis of the clinical and histopathological features of the skin lesions. She was treated with 0.6 mg of oral colchicine twice daily. Within one week, the erythematous plaques and arthralgia began to recede and disappeared within four weeks. In our case, colchicine seemed to improve the signs and symptoms of ND and appeared to be more effective than the other drugs we had used.- - - - - - - - - - ranking = 4keywords = dermatosis (Clic here for more details about this article) |
3/16. Hyperkeratosis lenticularis perstans (Flegel's disease)--lack of response to treatment with tacalcitol and calcipotriol.Hyperkeratosis lenticularis perstans (HLP) or Flegel's disease is a rare dermatosis characterized by asymptomatic hyperkeratotic papules predominantly located on the lower extremities. Lesional and non-lesional epidermis samples were studied by light- and electron-microscopic examination. The main ultrastructural finding was the presence of structurally altered Odland bodies/membrane-coating granules. Different therapeutic options for HLP have been reported, but none of the treatments was shown to be consistently effective. Here, we report on a patient with Flegel's disease who did respond to topical 5-fluorouracil, whereas topical vitamin d(3) synthetics were ineffective.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
4/16. Bowen disease associated with porokeratosis of Mibelli.A 73-year-old woman developed Bowen precancerous dermatosis in an area of porokeratosis of Mibelli on her leg. This is the fourth reported case of Bowen disease arising from porokeratosis of Mibelli. The recent literature indicates that porokeratosis of Mibelli may be due to an abnormal clone of cells, predisposing affected individuals to development of malignant neoplasms over the involved areas.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
5/16. Elastosis perforans serpiginosa in an adult with Down's syndrome: report of a case with symmetrical localized involvement.Elastosis perforans serpiginosa (EPS) is a rare perforating dermatosis in which elastic fibers extrude from the papillary dermis producing umbilicated papules, characteristically arranged to form arciform or serpiginous patterns on the skin. It can be observed in patients with Down's syndrome, in whom in some cases the disease has been reported to be more widespread and to run a longer course. We present the case of a 20-year-old girl with Down's syndrome, hypothyroidism, acne and hypertrichosis, who had a 2-year history of multiple atrophic lesions with an arciform pattern on the distal extensor portions of both thighs, histologically showing the typical features of EPS.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
6/16. Erosive pustular dermatosis of the leg: report of three cases.Erosive pustular dermatosis of the leg is a distinct form of spongiform amicrobial pustulosis. The disorder typically affects the lower limbs of elderly patients presenting with chronic venous insufficiency and stasis dermatitis, and has a chronic course. Three elderly patients with chronic venous ulcers are described, who developed pustules and moist eroded lesions on the leg. The clinical and histological features were typical for erosive pustular dermatosis. The lesions rapidly responded to topical treatment with either tacrolimus or corticosteroids. Of note, this condition was associated with a diverticular disease in two patients, while in another patient an epidermoid carcinoma of the tongue was present. Erosive pustular dermatosis of the leg is an uncommon but distinct skin disorder typically associated with trophic changes of the lower limbs. Our observations raise the question of the relation of erosive pustular dermatosis of the leg with the group of neutrophilic dermatoses. Topical immunotherapy with tacrolimus may constitute a novel therapeutic option for this frequently recalcitrant condition.- - - - - - - - - - ranking = 8keywords = dermatosis (Clic here for more details about this article) |
7/16. Acute pustulosis of the legs in diverticulitis with sigmoid stenosis: an overlap between bowel-associated dermatosis-arthritis syndrome and pustular pyoderma gangrenosum.BACKGROUND: Bowel-associated dermatosis-arthritis syndrome denotes the occurrence of diarrhoea with arthritis and skin lesions related to bowel disease with or without bowel bypass. In this condition, the histological finding of cutaneous aseptic neutrophilic cell infiltrate is non-specific and common to a wide spectrum of neutrophilic dermatoses, including pyoderma gangrenosum. observation: We describe a 78-year-old woman with fever, abdominal discomfort and arthralgias, who developed grouped pustular lesions on her shins with histologically spongiform pustule formation. Aetiological assessment disclosed diverticular disease with sigmoid stenosis. CONCLUSION: Although clinical and histological features in our case fit the diagnosis of bowel-associated dermatosis-arthritis syndrome, they may also correspond to a pustular variant of pyoderma gangrenosum. Our observation raises the question of the nosological classification of bowel-associated dermatosis-arthritis syndrome within the spectrum of neutrophilic diseases.- - - - - - - - - - ranking = 7keywords = dermatosis (Clic here for more details about this article) |
8/16. Purpuric generalized lichen nitidus: an unusual eruption simulating pigmented purpuric dermatosis.BACKGROUND: Generalized haemorrhagic lichen nitidus is rare. To our knowledge, this form of presentation has only been reported once. OBJECTIVE: To describe a new case of generalized haemorrhagic lichen nitidus simulating a pigmented purpuric dermatosis. methods AND RESULTS: We document a 24-year-old man who presented with an 8-month history of a progressive non-pruritic, red-brown papular eruption on the dorsa of the feet, ankles and distal third of the legs. A diagnosis of Schamberg's progressive pigmentary dermatosis was made, and no treatment was prescribed. Two months later, the lesions had extended to the abdomen, groins, forearms, elbows and wrists. biopsy of the skin of the right foot revealed lesions typical of lichen nitidus with subepidermal extravasation of red cells and capillary wall hyalinization. macrophages and T lymphocytes were abundant in the infiltrate. CONCLUSION: Purpuric generalized lichen nitidus should be included in the differential diagnosis of pigmented purpuric dermatoses.- - - - - - - - - - ranking = 6keywords = dermatosis (Clic here for more details about this article) |
9/16. A report of two cases of pigmented purpuric dermatoses treated with puva therapy.This is a report of 2 patients with pigmented purpuric dermatosis treated successfully with Psoralen photochemotherapy (PUVA). Treatment for pigmented purpuric dermatosis is generally unsatisfactory, and we believe this to be the first report of successful response of this condition to PUVA.- - - - - - - - - - ranking = 2keywords = dermatosis (Clic here for more details about this article) |
10/16. Unusual presentation of porokeratosis palmaris, plantaris et disseminata.porokeratosis plantaris, palmaris et disseminata is an autosomal dominant genodermatosis characterized by multiple lesions on the palms and soles, and later on other areas, both sun-exposed and non-sun-exposed. We report a 66-year-old man with porokeratosis plantaris, palmaris et disseminata whose disease had an unusual evolution. To our knowledge this is the first case of the disease in which the lesions first appeared on the trunk and extremities and later involved the palms and soles.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
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