1/61. Diffuse alopecia with stem cell folliculitis: chronic diffuse alopecia areata or a distinct entity?A 34-year-old woman presented with an 8-year history of slowly progressive diffuse nonscarring alopecia with loss of hair density. scalp biopsy specimens showed increased miniaturized follicles and an asymmetric wedge-shaped lymphocytic infiltrate concentrated on the stem cell-rich region at the point of entry of sebaceous ducts and at bulge-like regions of multiple follicles. Several hair bulbs emerging at the stem cell compartment also were inflamed, but the hair bulbs in the deeper dermis and subcutis were spared. I speculate whether these findings may represent a stem cell folliculitis similar to the reaction pattern previously observed in graft versus host disease and in androgenetic alopecia. The additional presence of peribulbar lymphocytic inflammation could indicate that the patient had a variant of alopecia areata. The clinical presentation of a slowly progressive diffuse alopecia without progression to clinically recognizable alopecia areata and the prominent lymphocytic inflammation involving the stem cell compartment may prompt a reexamination of similar cases currently classified as chronic diffuse alopecia areata. The concept that lymphocytes can inhibit stem cell function without destroying the stem cells themselves needs consideration.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/61. pemphigus vulgaris localized to the vagina presenting as chronic vaginal discharge.Involvement in pemphigus vulgaris of the female genital tract including the vulva, vagina and cervix has previously been described. In all these cases other cutaneous and mucosal sites have also been affected at some time. We describe a case of pemphigus vulgaris which only involved the vaginal mucosa. The patient presented with a persistent vaginal discharge and examination showed extensive vaginal erosions. histology of vaginal biopsies was non-diagnostic. The recognition that the vaginal changes may represent an immunobullous disease led to further vaginal biopsies on which direct immunofluorescence studies were performed. These biopsies showed IgG and C3 in the intercellular epidermis, suggesting a diagnosis of pemphigus vulgaris. During the 3-year period that the patient has been under review there have never been any other cutaneous or mucosal lesions. To our knowledge, this is the first case of pemphigus vulgaris localized exclusively to the vaginal mucosa. There was considerable delay in diagnosis and this case highlights how important it is to recognize that chronic mucosal lesions at genital sites may be caused by immunobullous diseases such as cicatricial pemphigoid and pemphigus, and to institute appropriate investigations.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
3/61. Chronic radiodermatitis following repeated percutaneous transluminal coronary angioplasty.We review three patients who developed chronic radiodermatitis subsequent to undergoing multiple percutaneous transluminal coronary angioplasties (PTCAs). All patients had had chronic ischaemic heart disease (IHD) and had undergone lengthy PTCA on several occasions. The skin eruption was characterized by an atrophic rectangular plaque on the left upper back, presenting as mottled hyper- and hypopigmentation with reticulate telangiectasia. Histologically, the eruption demonstrated epidermal atrophy, hyalinized and irregularly stained collagen, and telangiectasia of superficial vessels in the dermis. Although the risk of radiation injury in most patients undergoing cardiac catheterization is low, this danger should not be ignored. In particular, patients with long-standing IHD and numerous repeated catheterizations to only one or two occluded coronary arteries should be considered at high risk.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
4/61. Acral keratotic graft versus host disease simulating warts.A 55-year-old man experienced persistent oral graft versus host disease after receiving an HLA-matched allogeneic stem cell peripheral blood transplant for chronic myeloid leukaemia. Twelve months post-transplant, multiple keratotic lesions resembling warts developed on his fingers, on his palms and on the soles of his feet. Skin biopsy showed a lichenoid reaction under a hyperplastic and hyperkeratotic epidermis lacking signs of papillomavirus infection. The lesions partially regressed when the patient's oral prednisone was increased. This instance of acral keratotic lesions may represent an unusual expression of chronic graft versus host disease; however, the the hydroxychloroquine which had been used for 8 months to control the patient's oral graft versus host disease cannot be excluded as a cofactor.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
5/61. skin manifestations of a patient with mitochondrial encephalomyopathy with lactic acidosis and strokelike episodes (melas syndrome).skin abnormalities in a patient with newly diagnosed melas syndrome are reported. He presented with scaly, pruritic, diffuse erythema with reticular pigmentation on his entire body. Moderate hypertrichosis was also noted on the lower extremities. Thickened arrector muscles with longitudinal and cross sections were seen in the dermis. Analysis of mitochondrial dna in a biopsy specimen from the biceps brachii disclosed the presence of an A-to-G point mutation at nucleotide pair 3243. Electron microscopy revealed marked increase in the number of morphologically abnormal mitochondria in the arrector muscles of the involved skin. This is the first report of a melas syndrome patient with skin manifestations and evidence of morphologically abnormal mitochondria.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
6/61. Invisible mycosis fungoides: A diagnostic challenge.We describe a 76-year-old woman who presented persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patient's course. Skin biopsies obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
7/61. Macroscopic and microscopic mucinosis in chronic sclerodermoid graft-versus-host disease.Secondary cutaneous mucinosis is a well-recognized feature of connective tissue diseases such as lupus erythematosus and dermatomyositis. We report the first three cases of dermal mucinosis in association with severe chronic cutaneous graft-versus-host disease of the sclerodermoid variety. One patient had clinical changes due to abundant mucin accumulation within the papillary dermis (mucinoma). In the other two patients histological examination revealed extensive deposits of mucin predominantly within the reticular dermis. The microscopic appearances were striking, with numerous vacuolated spaces interspersed between collagen bundles. We speculate that this appearance is the result of ground substance becoming trapped within grossly sclerodermoid connective tissue.- - - - - - - - - - ranking = 2keywords = dermis (Clic here for more details about this article) |
8/61. Linear IgA bullous disease limited to the eye: a diagnostic dilemma: response to intravenous immunoglobulin therapy.PURPOSE: To report on a diagnostic dilemma and treatment challenge in a patient with chronic cicatrizing conjunctivitis without involvement of skin and other mucous membranes persisting for 6 years and not responding to topical and systemic steroids. DESIGN: Interventional case report. methods: We performed direct immunofluorescence of the conjunctiva with fluorescein-conjugated rabbit antihuman antibodies against immunoglobulin a, G, and M, complement 3 component, and fibrinogen. To investigate the presence of circulating antibodies in patient's serum, indirect immunofluorescence using normal human conjunctiva, normal human skin, and monkey esophagus as substrate was done. In addition, we did immunoblot analysis using normal human epidermis as substrate to determine the molecular weight of an antigen. The patient was treated with intravenous immunoglobulin (IVIg). The correlation between the titer of circulating antibodies and the activity of conjunctival inflammation at various intervals during the course of IVIg therapy was demonstrated by immunoblot assay with serial dilutions of the patient's serum. The highest dilution at which the binding was visible was considered the titer. RESULTS: Direct immunofluorescence of the conjunctiva and indirect immunofluorescence with both salt split skin and conjunctiva as substrate disclosed linear deposition of immunoglobulin a (IgA) at the epithelial basement membrane. Immunoblot analysis demonstrated the presence of IgA circulating antibodies in patient's serum directed against a 97kDa protein in human epidermis. A continuous decrease in the titer of these antibodies correlating to improvement of clinical symptoms was observed during IVIg therapy. CONCLUSIONS: Use of a nonconventional diagnostic tool (immunoblot analysis), in addition to conventional immunohistologic studies, might be helpful in establishing the diagnosis of patients with chronic cicatrizing conjunctivitis. On the basis of results of these laboratory tests and clinical presentation, we believe that this patient has linear IgA bullous disease limited to the eye. IVIg therapy decreased the titer of circulating antibodies and induced a remission in this patient.- - - - - - - - - - ranking = 2keywords = dermis (Clic here for more details about this article) |
9/61. Treatment of elastosis perforans serpiginosa with the flashlamp pulsed dye laser.BACKGROUND: Elastosis perforans serpiginosa (EPS) is an uncommon and chronic dermatosis characterized by the transepidermal elimination of altered elastic fibers originating in the dermis. Frequently arranged in an arcuate or serpiginous pattern, the end stage of the disease is atrophic or stellate-shaped scars. OBJECTIVE: Prior therapy has primarily involved cryotherapy with liquid nitrogen which may cause scar or keloid formation. We present our experience using the flashlamp pulsed dye laser to treat EPS. methods: A young man with down syndrome was referred by his primary care physician for treatment of a chronic dermatosis unresponsive to topical therapy. The clinical findings were consistent with EPS, and a biopsy specimen and elastic tissue stain confirmed the diagnosis histologically. After a successful test spot, treatment was begun on the erythematous papules using a flashlamp pulsed dye laser (585 nm) with a 5 mm spot size and fluences of 6.0-7.0 J/cm2. RESULTS: Treated areas showed moderate to marked improvement overall. There was less erythema, thickness, and scarring in treated areas compared with active or end-stage lesions. CONCLUSION: The flashlamp pulsed dye laser may be an effective treatment method for EPS.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
10/61. Acral peeling skin syndrome.Peeling skin syndrome is a rare autosomal recessive disease characterized by widespread painless peeling of the skin in superficial sheets. We describe a 34-year-old man with a lifelong history of spontaneous asymptomatic peeling skin limited to the acral surfaces. This patient probably represents a localized variant of peeling skin syndrome, which has previously been described as a generalized condition. light and electron microscopic studies of biopsy specimens taken before and after immersion in water were performed. It was concluded that this patient has abnormal keratohyalin granules and inadequate aggregation of keratin filaments that caused the separation of the epidermis in the stratum corneum through the clear zone. Alternatively, unknown keratin species expressed in the clear zone may also cause the abnormality. (J Am Acad Dermatol 2000;43:1112-9.).- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
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