1/29. Correction of bone marrow failure in dyskeratosis congenita by bone marrow transplantation.dyskeratosis congenita is recognized by its dermal lesions and constitutional aplastic anemia in some cases. We report successful allogeneic bone marrow transplantation in two siblings with this disease from their sister, and their long term follow-up. We used reduced doses of cyclophosphamide and busulfan for conditioning instead of total body irradiation. Also, we report late adverse effects of transplantation which are not distinguishable from the natural course of disease.- - - - - - - - - - ranking = 1keywords = keratosis (Clic here for more details about this article) |
2/29. Atrichia, ichthyosis, follicular hyperkeratosis, chronic candidiasis, keratitis, seizures, mental retardation and inguinal hernia: a severe manifestation of IFAP syndrome?A boy with congenital atrichia, ichthyosis follicular, keratitis, cutaneous infections and a huge inguinal hernia, but without deafness is reported. We believe it represents a new case of a rare X-linked recessive syndrome known as ichthyosis follicularis, alopecia, photophobia syndrome (IFAP). The differential diagnosis from keratitis ichthyosis deafness is discussed. The cutaneous infections seen in our case suggest the possibility of considering a genetic link between these syndromes.- - - - - - - - - - ranking = 0.8keywords = keratosis (Clic here for more details about this article) |
3/29. Keratosis lichenoides chronica: report of a new case, with success of puva therapy.Keratosis lichenoides chronica is a rare disorder. It consists of the progressive development of asymptomatic verrucosities on the limbs and the trunk, with a partially linear distribution. We report the case of a 78-year-old woman who presented with keratotic plaques and papules of the trunk and the limbs for several years. A diagnosis of keratosis lichenoides chronica was clinically and histologically evident. PUVA-therapy was started, with excellent results.- - - - - - - - - - ranking = 0.2keywords = keratosis (Clic here for more details about this article) |
4/29. Vascular variant of keratosis lichenoides chronica associated with hypothyroidism and response to tacalcitol and acitretin.Keratosis lichenoides chronica (KLC) is a rare chronic progressive cutaneous disease that is part of the heterogeneous group of lichenoid dermatoses. The typical clinical presentation is characterized by lichenoid hyperkeratotic papules and nodules arranged in a linear and reticular pattern on the trunk and extremities. Our case confirms the existence of a vascular variant of KLC. There is no consensus about its treatment, since it is refractory to many different treatment modalities. We report the effectiveness of acitretin in KLC in combination with tacalcitol. KLC is of unknown aetiology, but is perhaps associated with systemic diseases, most importantly glomerulonephritis and lymphoma. This is the second case associated with hypothyroidism.- - - - - - - - - - ranking = 0.8keywords = keratosis (Clic here for more details about this article) |
5/29. Human papillomavirus-negative ileostomal chronic papillomatous dermatitis.BACKGROUND: Papillomatous stoma-related skin lesions may result from irritant reactions or infection with epidermodysplasia verruciformis human papillomavirus (HPV) types. methods: We report upon a papillomatous lesion at the ileostoma of a 63-year-old male with familial adenomatous polyposis and colorectal adenocarcinoma. We thoroughly tested the lesion for HPV using immunohistochemistry, transmission electron microscopy, and polymerase chain reaction analyses. RESULTS: The lesion was a fleshy, multilobulated, and verrucous plaque, with hyperkeratosis, hypergranulosis, acanthosis and marked papillomatosis. The clinical and light microscopic features were suggestive of a condyloma. However, no HPV was detected. CONCLUSIONS: We suggest that the lesion most likely represents chronic papillomatous dermatitis, a reaction to mechanical and/or chemical irritation usually associated with urostomies and only rarely observed with ileostomies. This case highlights the clinical, diagnostic and therapeutic aspects of an unusual cutaneous morbidity associated with ileostomies.- - - - - - - - - - ranking = 0.2keywords = keratosis (Clic here for more details about this article) |
6/29. Keratosis lichenoides chronica. Possible variant of lichen planus.A chronic, asymptomatic, keratotic, papular eruption, present for 15 years and resistant to all modes of therapy, was observed in a 49-year-old woman. Many of the papules formed a reticulated configuration over the arms and legs. This case resembles several others that have been described in the literature under different names, ie, keratosis lichenoides chronica, porokeratosis striata, and lichen verrucosus et reticularis. It is likely that all of these names refer to the same condition, constituting a definite clinical entity, which, on the basis of histological study, may represent a variant of lichen planus.- - - - - - - - - - ranking = 0.4keywords = keratosis (Clic here for more details about this article) |
7/29. hydroa vacciniforme-like eruptions in a patient with chronic active EB virus infection.We report a case of chronic active Epstein-Barr (EB) virus infection (CAEBV) associated with skin eruptions mimicking hydroa vacciniforme (HV) in a 4-year-old boy. The patient had repeated episodes of vesiculo-necrotic eruptions on the face, scalp, and bilateral forearms one year before the first visit to our department. General symptoms including fever, hepatosplenomegaly, abnormal liver function, and cervical lymph node swelling were noted three months before the first visit. At the first visit, small, bean-sized, erythemic papules with central necrosis were observed on the face and anterior chest wall. thumb-sized ulcers with crust were present on the bilateral forearms. Histopathological examination of an erythematous lesion in the submandibular area revealed parakeratosis with a thick crust, mild spongiosis in the epidermis, and a dense infiltration of lymphoid cells into the dermis and perivascular space. Laboratory examination showed EBNA x 40, EBV VCA IgG x 1,280, and EBV dna (PCR) 8 x 10(4). EBV-encoded small nuclear rna (EBER) positive cells were detected in the dermis by an in situ hybridization (ISH) method. Large granular lymphocytes (65%) with the NK cell phenotype were found in the peripheral blood. A real time PCR method showed 171,741 copies/ micro g dna in CD 16 positive cells. Although latent EBV infection-associated eruptions have been documented, detailed skin manifestations in CAEBV are less well known.- - - - - - - - - - ranking = 0.2keywords = keratosis (Clic here for more details about this article) |
8/29. Follicular granular parakeratosis.An 83-year-old patient presented herself with a ten-year history of keratotic papules on her trunk. A biopsy of this process revealed granular parakeratosis confined to the infundibulum of a follicle. Exclusive follicular involvement in granular parakeratosis has not been previously described.- - - - - - - - - - ranking = 1.2keywords = keratosis (Clic here for more details about this article) |
9/29. A peculiar acantholytic dermatosis.This paper reports a peculiar case seen in a 36-year-old woman who every summer since the age of 30 has developed numerous solitary vesicles or crusted papules with intense pruritus in the seborrheic zones. There are no general symptoms and no hereditary relations. The individual rash disappears completely in a week to a month and there is no eruption in winter. The clinical picture resembles that of Darier's disease, but histopathological tests reveal intraepidermal cleft or bulla formation due to acantholysis but no dyskeratosis. Clinicopathological consideration indicates a disease akin to familial benign chronic pemphigus, but as our case does not correspond to any known disease, it is therefore reported as a peculiar acantholytic dermatosis.- - - - - - - - - - ranking = 0.2keywords = keratosis (Clic here for more details about this article) |
10/29. Idiopathic portal hypertension and chronic arsenic poisoning. Report of a case.We report a case of idiopathic portal hypertension which is related to chronic arsenic poisoning. Only 7 cases have been reported previously. The patient presented with bleeding esophageal varices. splenomegaly and hyperkeratosis of palms and soles were later noted and led to the discovery of chronic arsenic poisoning. The hemodynamic studies revealed a gradient between the splenic pulp pressure and hepatic wedge pressure which is consistent with presinusoidal hypertension. The liver histology revealed only mild portal fibrosis. arsenic poisoning is one cause of idiopathic protal hypertension.- - - - - - - - - - ranking = 0.2keywords = keratosis (Clic here for more details about this article) |
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