1/9. A case of pre-sezary syndrome preceded by hand lesions.Pre-sezary syndrome is an erythroderma with a chronic course, clinical findings of sezary syndrome, lymphocytic subepidermal band infiltration at times, and repeated cycles of circulating Sezary cells of less than 1,000 cells/mm3. Duration of the pre-existing skin diseases preceding pre-Sezary erythroderma varies from a few weeks to 20 years. Before the erythroderma develops, these patients are diagnosed with contact dermatitis, neurodermatitis, chronic dermatitis, atopic dermatitis, or asteatotic eczema. hand lesion also precedes the pre-Sezary erythroderma. This condition has been controlled by three cycles of chemotherapy consisting of vincristine, cytoxan, doxorubicin, and prednisolone. We describe a case of pre-sezary syndrome preceded by hand lesion and treated with chemotherapy.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
2/9. A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens.We report a new type of paraneoplastic mixed bullous skin disease in a patient with a B-cell lymphoma associated with monoclonal IgM kappa paraproteinaemia. The patient's clinical and histological features were reminiscent of bullous pemphigoid. Characterization of antiepidermal antibodies by immunoblotting and indirect immunoelectron microscopy demonstrated a novel pattern of reactivity: IgG antibodies were directed against desmoplakins I-II and BPAG2, and were associated with antidesmoglein 3 polyclonal IgM antibodies. Such an autoreactive pattern involving antidesmoplakins, anti-BPAG2 and antidesmoglein 3 antibodies has not been previously reported. It reflects the breakdown of tolerance to multiple epidermal antigens observed in some patients with malignancy.- - - - - - - - - - ranking = 5keywords = skin disease (Clic here for more details about this article) |
3/9. Hereditary non-polyposis colorectal cancer associated with disseminated superficial porokeratosis. microsatellite instability in skin tumours.A 73-year-old man presented with typical lesions of disseminated superficial porokeratosis (DSP) and multiple seborrhoeic keratoses on his face, trunk and extremities, and later developed a keratoacanthoma on his lip. He belonged to a cancer-prone pedigree susceptible to colonic, uterine and other internal cancers, and had a personal history of early gastric cancer and advanced adenocarcinoma of the descending colon without adenomatous polyps at age 59 years. polymerase chain reaction amplification of skin samples for seven separate microsatellite polymorphisms revealed microsatellite instability (MSI) at multiple loci in five of six seborrhoeic keratoses and the keratoacanthoma, strongly suggesting underlying defects in dna mismatch repair. Although no germline mutations in two mismatch repair genes hMSH2 and hMLH1 were found, our patient was recognized as having hereditary non-polyposis colorectal cancer (HNPCC) based on the family history and the findings of the microsatellite analysis of skin tumours. This confirmed the usefulness of detection of MSI in prevalent and readily accessible skin lesions, including non-sebaceous non-dysplastic tumours such as seborrhoeic keratosis in the screening of HNPCC families. Although DSP may also be inherited as an autosomal dominant condition, this particular skin disease appeared to be sporadic in our patient and, to our knowledge, no association of DSP or other forms of porokeratosis with HNPCC has previously been reported. In contrast to the seborrhoeic keratoses and keratoacanthoma, no MSI was observed in two samples from DSP lesional epidermis examined.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
4/9. rothmund-thomson syndrome in three siblings and development of cutaneous squamous cell carcinoma.rothmund-thomson syndrome (RTS) is a rare autosomal recessive genodermatosis. It is characterized by early onset of progressive poikiloderma and several other cutaneous and extracutaneous findings including alopecia, dystrophic teeth and nails, juvenile cataracts, short stature, hypogonadism, and bone defects. There are several reported cases of skin malignancies in RTS patients, indicating a possibly higher incidence of cutaneous and noncutaneous malignancies. We report three siblings with RTS who developed cutaneous squamous cell carcinoma (SCC).- - - - - - - - - - ranking = 0.55137577466369keywords = dermatosis (Clic here for more details about this article) |
5/9. Neutrophilic dermatosis of the hands: four new cases and review of the literature.BACKGROUND: Isolated or predominantly hand involvement in Sweet's syndrome, pyoderma gangrenosum, or pustular vasculitis is a rare presentation in the spectrum of neutrophilic dermatoses and is often associated with an occult malignancy or other systemic inflammatory disorder. When these disorders occur on the hands, they are often clinically indistinguishable, but they can sometimes be separated histologically by the presence of papillary dermal edema (Sweet's syndrome), ulceration and necrosis (pyoderma gangrenosum), or vasculitis (pustular vasculitis). These distinctions may be arbitrary, however, and reflect differences in the temporal course of the disease and in the degree of inflammation at the time of biopsy. methods: We report four cases of neutrophilic dermatosis affecting the hands and a review of the literature for similar cases. RESULTS: Of the four patients presented, two had associated carcinomas and one had myelodysplasia in transition to leukemia. The cutaneous symptoms preceded the finding of an occult malignancy. Thirty-two reported cases of neutrophilic dermatoses presenting on the hands showed clinicopathologic features similar to those in our series. Taken together, the mean age at diagnosis was 60.5 years, and 58% of the patients were female. Twenty-five per cent (nine patients) also had myelodysplasia or leukemia, 14% (five patients) ulcerative colitis, 6% (two patients) carcinoma, 6% (two patients) Crohn's disease, and 6% (two patients) seropositive arthritis. CONCLUSIONS: These cases illustrate the importance of recognizing that neutrophilic dermatoses may present uniquely or predominantly on the hands. This presentation is distinctive, and prompt diagnosis may prevent unnecessary medical or surgical therapy and may lead to the earlier diagnosis and treatment of an associated malignancy or other systemic disorder.- - - - - - - - - - ranking = 2.7568788733185keywords = dermatosis (Clic here for more details about this article) |
6/9. Atypical manifestations of pityriasis lichenoides chronica: development into paraneoplasia and non-Hodgkin lymphomas of the skin.Three patients with atypical courses and manifestations of pityriasis lichenoides chronica (PLC) are presented. The first patient is a 21-year-old white woman who showed a good response of her PLC lesions as well as her reactive oligoarthritis to repeated PUVA treatments combined with oral prednisone during 1 year. The effect of the treatment then decreased. The patient developed a low-grade malignant lymphoma of the lung. When the lymphoma of the lung improved after chemotherapy, the PLC eruptions improved, too. The second patient is a 41-year-old man, whose Hodgkin's disease stage IVa was successfully treated by chemotherapy and radiotherapy in 1984. In 1987 he showed PLC lesions which responded well to puva therapy, later also in combination with etretinate. Until 1988 repeated skin biopsies revealed a non-specific eczematous pattern. In 1989 the recalcitrant PLC eruptions finally revealed a pleomorphic non-Hodgkin lymphoma of the skin with medium-sized cells. The third patient had a PLC for about 9 years when Hodgkin's disease stage Ia was diagnosed. At the beginning the skin biopsy showed an eczematous pattern, but 2 years later, in 1990, skin infiltrations of a large-cell, anaplastic non-Hodgkin lymphoma were seen. These cases show that PLC in rare cases may either represent a paraneoplastic skin disease or may itself develop into cutaneous lymphomas.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
7/9. Premalignant melanosis of the conjunctiva and the cornea in xeroderma pigmentosum.xeroderma pigmentosum is a rare autosomal recessive dermatosis. The neoplastic changes in sunlight-exposed areas of the skin and eyes may be related to the impaired replication of ultraviolet radiation-damaged DNA. A 38-year-old Greek woman is reported with a mild form of xeroderma pigmentosum and primary acquired melanosis with atypia of her right limbal conjunctiva and cornea. The development of this precursor of conjunctival malignant melanoma in a xeroderma pigmentosum patient may support the putative role of sunlight exposure in malignant transformation of conjunctival melanocytes.- - - - - - - - - - ranking = 0.55137577466369keywords = dermatosis (Clic here for more details about this article) |
8/9. Acute febrile neutrophilic dermatosis (Sweet's syndrome) and myeloproliferative disorders.Acute febrile neutrophilic dermatosis (ND) consists of the abrupt onset of red, tender, cutaneous plaques on the face, extremities, and upper trunk, accompanied by fever, malaise, and neutrophilic leukocytosis. Histologically, there are distinctive, dense, dermal infiltrates of neutrophils. Response to systemic steroids is dramatic. This report describes four patients with leukemia or preleukemia and ND (LND), reviews reports of 12 similar patients, and compares LND with ND in otherwise healthy individuals (idiopathic ND, IND). Although lesions of LND more frequently had vesiculobullous appearances or location on mucous membranes, this study showed no consistent difference between LND and IND with regard to cutaneous signs, symptoms, histologic findings, and response to therapy. The first episode of LND either preceded or followed documentation of the myeloproliferative disorder, and the most common associated hematologic conditions were acute myeloid or myelomonocytic leukemia. Moderate to severe anemia was present in nine of ten patients whose first episode of LND preceded the discovery of the hematologic condition by eight months or less. The presence of anemia is the most obvious and readily detectable difference between LND and IND. The possibility of an underlying myeloproliferative disorder should be considered in all patients with ND, and LND should not be confused with infectious complications in patients known to have myeloproliferative disorders.- - - - - - - - - - ranking = 2.7568788733185keywords = dermatosis (Clic here for more details about this article) |
9/9. Follicular mucinosis associated with scarring alopecia, oligoclonal T-cell receptor V beta expansion, and staphylococcus aureus: when does follicular mucinosis become mycosis fungoides?A diagnosis of alopecia mucinosa, occurring as a single scalp lesion, was made in a 40-year-old white woman who had a history of trauma. Follicular mucinosis, staphylococcus aureus, and oligoclonal expansion of the T-cell receptor V beta chain genes 6 and 7 were present in the skin. Epidermotropic T-cell skin diseases with oligoclonal T-cell proliferations may be the result of HLA- and cytokine-determined reaction patterns to persistent antigens.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |