Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 39-year-old woman with coexistent lichen planus, lichen sclerosus, and generalized morphea with ulcerations is described. Three additional cases were found in the files of the Mayo Clinic from 1950 to 1983, and these are summarized. Eight cases reviewed in the literature are also summarized. Coexistent lichenoid and sclerodermatous eruptions in graft-versus-host disease after bone marrow transplantation are noted as a model for this combined inflammatory and sclerotic dermatosis.
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PMID:Coexistence of lichen sclerosus, morphea, and lichen planus. Report of four cases and review of the literature. 400 88

The aim of the study was to compare alterations of various regions of the basement membrane zone (BMZ) in lichen sclerosus (LS) using laser scanning confocal microscopy. The study included three cases of bullous LS, one case of bullous LS that developed in the course of graft-versus-host disease (GVHD), and six cases of non-bullous LS. Three cases of morphea served as a control. Biopsies from patients' skin and control biopsies from normal human skin were cut into 30-microm thick slides and labeled with antibodies against beta4-intergin (lamina lucida marker), collagen IV, and the N-terminal end of collagen VII (lamina densa markers) and the C-terminal end of collagen VII (sublamina densa marker) using routine immunofluorescence (IF). Three-dimensional (3D) reconstruction of various regions of the BMZ showed a decrease in the number and size of the dermal papillae in LS and morphea as compared with normal skin. In LS numerous invaginations and holes were present in the BMZ at the level of the lamina lucida and lamina densa. Computer animation of 3D projections revealed that the thickness of the lamina densa observed under the light microscopy is an optical artifact dependent on periodical tortion of the lamina densa along its axis. Torsions and invaginations of the BMZ are equally responsible for the phenomenon of artificial reduplication of the lamina densa observed at the ultrastructural level. IF labeling with antibody against the N-terminal end of collagen VII disclosed the presence of a large hole (up to 25 microm) in the lamina densa and the presence of granular material in deep dermis suggestive of partial degradation of lamina densa at the level of anchoring fibers. An IF mapping study showed blister formation below the lamina densa in three patients with bullous LS, whereas in a case of LS associated with GVHD, a blister formed through the basal layer of the epidermis. In morphea, there was flattening of BMZ at the level of lamina lucida, lamina densa, and sublamina densa but the continuity of BMZ was preserved. Three-dimensional reconstruction of dermal-epidermal junction in LS revealed alterations of the BMZ, most pronounced at the level of the lamina densa and sublamina densa.
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PMID:Alterations of basement membrane zone in bullous and non-bullous variants of extragenital lichen sclerosus. 1502 89

Chronic cutaneous graft-versus-host disease (GVHD) is classically divided into two major clinical categories--lichenoid and sclerodermoid. Although diffuse areas of sclerosis as in scleroderma characterize the more advanced stages of the sclerodermoid form, the initial circumscribed plaques would be more correctly described as morpheaform. Eosinophilic fasciitis (EF) (a fibrosing disorder related to deep morphea) and lichen sclerosus (LS) have also been reported as manifestations of sclerodermoid GVHD. However, these two presentations of GVHD have not been emphasized in the dermatologic literature. We describe 6 patients, all of whom developed LS and two of whom also developed EF in the context of chronic GVHD. Each patient presented clinically with hypopigmented plaques that exhibited wrinkling, scaling, and follicular plugging. These lesions demonstrated the classic histologic features of LS including epidermal atrophy; a subepidermal zone of pale-staining, homogenized collagen; and a bandlike lymphocytic infiltrate. Although all patients eventually developed morpheaform and/or sclerodermoid GVHD, LS was a prominent part of the initial presentation of chronic cutaneous GVHD in every case. The LS lesions tended to occur on the neck and upper to mid aspect of the trunk, whereas morpheaform lesions favored the lower aspect of the trunk. EF involved the extremities (sparing the hands and feet), and was characterized clinically by an acute onset of pain and edema followed by induration with a rippled appearance. This case series serves to expand the spectrum of sclerodermoid GVHD, with LS as the most superficial and EF as its deepest manifestation.
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PMID:Lichen sclerosus and eosinophilic fasciitis as manifestations of chronic graft-versus-host disease: expanding the sclerodermoid spectrum. 1701 Jul 68

Pimecrolimus is an ascomycin macrolactam. It is a specific calcineurin inhibitor that allows topical application. The highly lipophilic nature of this compound reduces the risk of systemic absorption through normal and inflammed skin. Pimecrolimus shows activity not only against T-cell activation, but also against mast cells and pruritus. Pimecrolimus 1% cream is approved for atopic dermatitis, and also has a great potential in other inflammatory skin diseases. Clinical trials have been performed in contact- and seborrhoeic dermatitis, genital lichen sclerosus, intertriginous psoriasis and cutaneous lupus erythematosus. In other diseases, the available data are limited to small case series, or individual cases of graft-versus-host disease or Netherton's disease. Although the use of calcineurin inhibitors in the treatment of vitiligo is promising, detailed studies with pimecrolimus and ultraviolet-irradiation are necessary and there is a need for prospective randomised, double-blind controlled trials.
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PMID:Topical pimecrolimus for skin disease other than atopic dermatitis. 1702 Apr 22

Despite advances in the procedure and posttransplantation immunosuppressive therapy, more than half of allogeneic hematopoietic stem cell transplant (HSCT) recipients develop graft-versus-host disease (GVHD), which remains a major cause of morbidity and mortality. Modern HSCT protocols have resulted in substantial alterations in the timing and relative incidences of acute and chronic GVHD, making traditional classification schemes obsolete. This article reviews major changes in HSCT during the past decade, evolving concepts of acute and chronic GVHD (including new diagnostic criteria) and the expanding spectrum of cutaneous GVHD. It focuses on observations that have led to a better delineation of the full constellation of skin findings in chronic cutaneous GVHD, including lichen sclerosus, morpheaform lesions, and eosinophilic fasciitis. Recent insights into pathogenesis of GVHD, lessons from GVHD arising in settings outside HSCT, and therapeutic advances also are highlighted.
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PMID:The changing face of graft-versus-host disease. 1717 39

There are numerous dermatoses which may cause cicatricial alopecia when localized on the scalp, such as chronic discoid lupus erythematosus (DLE), lichen planus, graft-versus-host disease, dermatomyositis, scleroderma, cicatricial pemphigoid, porphyria cutanea tarda, follicular mucinosis, perifolliculitis capitis abscedens, lichen sclerosus et atrophicus, necrobiosis lipoidica, sarcoidosis, etc. Histologically, cicatricial alopecia is characterized by dermal scarring, along with absent or reduced hair follicles and reduced number of erector pili muscles. According to working classification of cicatricial alopecia by the North American Hair Society, primary cicatricial alopecia may be divided into the following categories: lymphocytic group (e.g., DLE, lichen planopilaris, classic pseudopelade (Brocq), central centrifugal cicatricial alopecia); neutrophilic group (e.g., folliculitis decalvans, dissecting cellulitis); and mixed group (e.g., folliculitis keloidalis). Over a 5-year period, 36 patients with cicatricial alopecia were hospitalized at our Department: DLE (n = 27), pseudopelade Brocq (n = 3), mucinosis follicularis (n = 2), and lichen planopilaris, folliculitis decalvans, folliculitis abscedens and folliculitis keloidalis (one patient each). Clinical evaluation was compared with histopathologic analysis of follicular architecture, as well as with the type, localization and extent of inflammatory infiltrate. Scalp biopsy was considered mandatory in all cases. Our experience indicates the need of more complex research to extend the knowledge about the etiopathogenesis and treatment options for cicatricial alopecia. We hope that this type of alopecia may attract more attention and research in the future.
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PMID:Cicatricial alopecia as a manifestation of different dermatoses. 1731 39

Graft-versus-host disease (GVHD) often complicates allogeneic stem cell transplantation (SCT) and affects mainly the gut, liver, lung and skin. The microscopic morphological features of late-phase sclerodermatous chronic GVHD in the skin, namely epidermal atrophy, lymphoplasmacytic infiltration, dense dermal fibrosis and adnexal atrophy, are histologically indistinguishable from those in sporadic systemic sclerosis, morphoea and the related condition of lichen sclerosus. Mucosal orifices including those of the genitourinary system may be severely affected. We present three SCT recipients with chronic GVHD and severe posthitis leading to phimosis requiring surgery. The excised prepuces showed features of lichen sclerosus including epidermal atrophy and a subepidermal zone of eosinophilic, homogeneous and hyalinized collagen above a band-like lymphoplasmacytic infiltrate. These cases add further evidence to support the notion that penile lichen sclerosus should be included within the expanding sclerodermoid spectrum of late-stage cutaneous chronic GVHD.
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PMID:Penile lichen sclerosus after allogeneic stem cell transplantation. 1850 92

Graft-versus-host disease (GVHD) is the main complication of allogeneic bone marrow transplantation and the skin is the most commonly involved organ. The clinical presentation is varied and may resemble autoimmune diseases, such as scleroderma, lichen planus or lichen sclerosus. Chronic GVHD presenting with a butterfly malar rash mimicking lupus erythematosus is uncommon. We report a series of five patients with cutaneous lichenoid GVHD that presented with a butterfly malar rash. Two of our patients had positive antinuclear antibody titres. The evolution was poor with development of sclerodermatous GVHD lesions in three patients and relapse of their haematological disease in two.
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PMID:Cutaneous lichenoid graft-versus-host disease mimicking lupus erythematosus. 1853 15

The most common cutaneous side effects of radiotherapy include radiodermatitis and radiation fibrosis. These are influenced by the type, dose, and pattern of delivery of the treatment. Distinct from these is postirradiation morphea (localized scleroderma), an idiosyncratic treatment-related phenomenon. Within the last 20 years, approximately 31 examples of postirradiation morphea after treatment for breast cancer were reported. We describe 5 new cases of this entity and integrate our findings with those in the literature. The mean age of the patients at the time of diagnosis of cancer was 58 years; all were left-sided and treated by local excision of the tumor, ipsilateral axillary lymph node dissection, and local radiotherapy. After an interval of 4 to 12 years, the patients developed morphea in the radiation portals, with extension beyond it in one instance. Recurrent breast carcinoma was suspected clinically in 2 cases. Microscopically, changes of morphea involved the dermis in all cases and the subcutis in 2. There was associated lichen sclerosus et atrophicus in 2 cases. Our data about management and outcome are limited, but 1 patient treated with potent topical steroids experienced gradual softening of the affected skin over a 5-year period, whereas another had a mastectomy for relief of painful induration of the breast. Our findings support existing theories about the pathogenesis of this condition and link it to those of sclerodermoid graft-versus-host disease. The purpose of our communication is to draw attention to this underrecognized complication of treatment for breast cancer.
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PMID:Postirradiation morphea: an underrecognized complication of treatment for breast cancer. 1865 34

A major complication following hematopoietic stem cell transplantation is graft versus host disease. Cutaneous manifestations of chronic graft versus host disease (cGVHD) are varied and this condition impacts patient outcomes and quality of life. We describe two cases of lichen sclerosus et atrophicus-like cGVHD developing in patients after hematopoietic stem cell transplantation. Both patients presented clinically with patches of pigmentary changes and scaling that displayed classic histologic features of lichen sclerosus et atrophicus. The skin is a frequent target organ of cGVHD and often the presenting location of the disease, making dermatologists key in recognition and management. It has been proposed that cutaneous cGVHD is a spectrum of disease and the lesions may evolve through various stages. Lichen sclerosus et atrophicus-like cGVHD may represent a phase in this continuum or a distinct sub-type of disease. Remaining cognizant of the potential manifestations of disease is key for prompt recognition and proper treatment.
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PMID:Lichen sclerosus et atrophicus-like graft-versus-host disease post stem cell transplant. 1993 Sep 91


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