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

A 31-year-old woman presented with progressive deep linear induration on her lower abdomen, forearms and thighs. These symptoms developed three years after allogenic stem cell transplantation. Furthermore, the patient showed multiple hypopigmented lichenoid papules on the extensor surfaces of the forearms consistent with lichen sclerosus. Histological analysis of a biopsy specimen from her left thigh showed dermal sclerosis extending into the fascia, thus establishing the diagnosis of a rare combination of superficial and deep sclerodermoid chronic graft-versus-host disease. After 7 cycles of extracorporeal photopheresis, a marked resolution of the indurations and a reduction of the modified Rodnan skin score from 12 to 7 were noted.
 ...
PMID:[A rare case of a sclerodermoid chronic graft versus host disease. Successful treatment with extracorporeal photopheresis (ECP)]. 2030 12

The spectrum of clinical and histopathologic features associated with chronic graft-versus-host disease (GVHD) is broad, with recognized variants simulating scleroderma, lichen sclerosus, eosinophilic fasciitis, and de novo diffuse melanoderma. We report a case of a patient with multiple myeloma who presented approximately 1 year after his allogeneic hematopoietic stem cell transplantation with lesions of chronic lichenoid GVHD that harbored features of hypertrophic lupus erythematosus (LE) and that was initially mistaken for a superficial well-differentiated squamous cell carcinoma (SCC). However, in 4 years of follow-up, the patient failed to develop any evidence of cutaneous or systemic LE, actinic damage, or SCC, and the lesions cleared with topical and systemic treatments appropriate for chronic GVHD. For proper interpretation of the histologic findings of GVHD, it is important for the dermatopathologist to be aware of unusual manifestations. Knowledge of the occurrence of hypertrophic LE and familiarity with its histologic features is also important to avoid an erroneous diagnosis of SCC in immunosuppressed patients.
 ...
PMID:Chronic cutaneous graft-versus-host disease simulating hypertrophic lupus erythematosus--a case report of a new morphologic variant of graft-versus-host disease. 2230 31

Lichen sclerosus et atrophicus (LSA) is an inflammatory disease that affects the genitals, which was first described by Hallopeau in 1887 and is of unknown etiology. Only 15% of patients have an associated extra-genital form, and 2.5% have an isolated extra-genital form. LSA treatment remains poorly codified and mostly empirical. Here, we report a case of LSA, of mainly cutaneous form, which was effectively treated using extracorporeal photochemotherapy (ECP). Remission was achieved quickly, after the fourth session, with excellent treatment tolerance. ECP is now recognized as an effective treatment for erosive lichen planus, graft-versus-host disease (GVHD), and scleroderma. Thus, we began ECP treatment for our cases of LSA based on clinical and/or anatomopathological similarities between LSA and these commonly ECP-treated disorders. The fact that ECP is effective in LSA, GVHD, erosive lichen planus, and scleroderma strengthen the hypothesis that there is a common link between these four conditions.
 ...
PMID:A case of cutaneous lichen sclerosus et atrophicus effectively treated by extracorporeal photochemotherapy. 2365 Dec 76

Beside to traditional use, dermoscopy is more and more used in the assessment of other "general" dermatologic conditions, namely scalp and hair disorders (trichoscopy), nails abnormalities (onychoscopy), skin infections and infestations (entomodermoscopy), and cutaneous inflammatory diseases (inflammoscopy). Among the list of new applications of dermoscopy, the study of inflammatory dermatoses is probably the most promising topic in terms of development and usefulness, considering the large number of such disorders and the frequent problems in their differential diagnosis which the dermatologist encounters in own daily clinical practice. In this paper, we report selected relatively common clinical differential diagnosis issues concerning inflammatory dermatoses (and some clinically related noninflammatory conditions), analysing them by a dermoscopic point of view in order to assist their noninvasive resolution according to the available literature data and our personal experience, including papulosquamous dermatoses (guttate psoriasis, pityriasis lichenoides chronica, pityriasis rosea, lichen planus, lymphomatoid papulosis, classic pityriasis rubra pilaris, papulosquamous sarcoidosis, disseminated forms of porokeratosis and papulosquamous chronic GVHD), dermatoses presenting with erythematous-desquamative patches/plaques (plaque psoriasis, eczematous dermatitis, pityriasis rosea, mycosis fungoides, subacute cutaneous lupus erythematosus), palmar psoriasis vs. chronic hand eczema, scalp psoriasis vs. seborrheic dermatitis, erythematous-desquamative disorders typically involving the elbows (psoriasis vulgaris, circumscribed juvenile pityriasis rubra pilaris, dermatomyositis/Gottron's sign), itchy papulonodular dermatoses (hypertrophic lichen planus, prurigo nodularis, nodular scabies and acquired perforating dermatosis), common facial inflammatory skin diseases (rosacea, seborrheic dermatitis and demodicidosis), lichen sclerosus vs. morphea, urticaria vs. urticarial vasculitis and common inflammatory cicatricial alopecia (discoid lupus erythematosus, lichen planopilaris and folliculitis decalvans).
...
PMID:The practical usefulness of dermoscopy in general dermatology. 2608 12

"Vulvar dystrophy" is characterized by chronic alterations of vulvar trophism, occurring in both physiological (menopause) and pathological (lichen sclerosus, vulvar graft-versus-host disease) conditions. Associated symptoms are itching, burning, dyspareunia and vaginal dryness. Current treatments often do not imply a complete remission of symptoms. Adipose-Derived Stem Cells (ADSCs) injection represents a valid alternative therapy to enhance trophism and tone of dystrophic tissues. We evaluated efficacy of ADSCs-based therapy in the dystrophic areas. From February to April 2013 we enrolled 8 patients with vulvar dystrophy. A biopsy specimen was performed before and after treatment. Digital photographs were taken at baseline and during the follow-up. Pain was detected with Visual Analogue Scale and sexual function was evaluated with Female Sexual Function Index. All patients received 2 treatments in 3 months. Follow-up was at 1 week , 1 and 3 months, and 1 and 2 years. We obtained a significant vulvar trophism enhancement in all patients, who reported pain reduction and sexual function improvement. Objective exam with speculum was easy to perform after treatment. We believe ADSCs-based therapy finds its application in the treatment of vulvar dystrophies, since ADSCs could induce increased vascularization due to their angiogenic properties and tissue trophism improvement thanks to their eutrophic effect.
...
PMID:The Use of Human Adipose-Derived Stem Cells in the Treatment of Physiological and Pathological Vulvar Dystrophies. 2688 Sep 44

We describe two patients who received haematopoietic stem cell marrow transplantation, and developed male genital lichen sclerosus (MGLSc), one of whom also had squamous carcinoma in situ (Bowen disease). MGLSc has previously been associated with graft-versus-host disease. Various aetiological factors for LSc have been proposed, including a role for chronic occluded epithelial exposure to urine. A number of factors imply that the risk of malignant transformation in this bone marrow transplant group is likely to be higher than the overall figure of 2-9% cited for MGLSc. It is vital, therefore, that clinicians involved in the care of those with haematological malignancies are adequately prepared to examine the genitals of their patients, and to recognize and refer any suspect penile lesions.
...
PMID:Male genital lichen sclerosus in recipients of bone marrow transplants. 2693 88

Over the last few years, dermoscopy has been shown to be a useful tool in assisting the noninvasive diagnosis of various general dermatological disorders. In this article, we sought to provide an up-to-date practical overview on the use of dermoscopy in general dermatology by analysing the dermoscopic differential diagnosis of relatively common dermatological disorders grouped according to their clinical presentation, i.e. dermatoses presenting with erythematous-desquamative patches/plaques (plaque psoriasis, eczematous dermatitis, pityriasis rosea, mycosis fungoides and subacute cutaneous lupus erythematosus), papulosquamous/papulokeratotic dermatoses (lichen planus, pityriasis rosea, papulosquamous sarcoidosis, guttate psoriasis, pityriasis lichenoides chronica, classical pityriasis rubra pilaris, porokeratosis, lymphomatoid papulosis, papulosquamous chronic GVHD, parakeratosis variegata, Grover disease, Darier disease and BRAF-inhibitor-induced acantholytic dyskeratosis), facial inflammatory skin diseases (rosacea, seborrheic dermatitis, discoid lupus erythematosus, sarcoidosis, cutaneous leishmaniasis, lupus vulgaris, granuloma faciale and demodicidosis), acquired keratodermas (chronic hand eczema, palmar psoriasis, keratoderma due to mycosis fungoides, keratoderma resulting from pityriasis rubra pilaris, tinea manuum, palmar lichen planus and aquagenic palmar keratoderma), sclero-atrophic dermatoses (necrobiosis lipoidica, morphea and cutaneous lichen sclerosus), hypopigmented macular diseases (extragenital guttate lichen sclerosus, achromic pityriasis versicolor, guttate vitiligo, idiopathic guttate hypomelanosis, progressive macular hypomelanosis and postinflammatory hypopigmentations), hyperpigmented maculopapular diseases (pityriasis versicolor, lichen planus pigmentosus, Gougerot-Carteaud syndrome, Dowling-Degos disease, erythema ab igne, macular amyloidosis, lichen amyloidosus, friction melanosis, terra firma-forme dermatosis, urticaria pigmentosa and telangiectasia macularis eruptiva perstans), itchy papulonodular dermatoses (hypertrophic lichen planus, prurigo nodularis, nodular scabies and acquired perforating dermatosis), erythrodermas (due to psoriasis, atopic dermatitis, mycosis fungoides, pityriasis rubra pilaris and scabies), noninfectious balanitis (Zoon's plasma cell balanitis, psoriatic balanitis, seborrheic dermatitis and non-specific balanitis) and erythroplasia of Queyrat, inflammatory cicatricial alopecias (scalp discoid lupus erythematosus, lichen planopilaris, frontal fibrosing alopecia and folliculitis decalvans), nonscarring alopecias (alopecia areata, trichotillomania, androgenetic alopecia and telogen effluvium) and scaling disorders of the scalp (tinea capitis, scalp psoriasis, seborrheic dermatitis and pityriasis amiantacea).
 ...
PMID:Dermoscopy in General Dermatology: A Practical Overview. 2761 97

Phototherapy is an effective treatment strategy for a variety of sclerosing skin conditions. There are a number of phototherapeutic modalities used for the treatment of sclerosing skin conditions, including ultraviolet (UV)A1, broadband UVA, psoralen plus UVA, and narrowband UVB phototherapy. As controlled trials with validated outcome measures are lacking for these therapies, existing evidence is largely level II for morphea and is even more minimal for scleroderma and other sclerosing disorders (scleroderma, lichen sclerosus, and chronic graft-versus-host disease, among others). Studies do suggest that phototherapy may be effective for many of these disorders, including those that have been unresponsive to other therapies. Phototherapy remains an attractive therapeutic option for patients due to its efficacy and favorable risk-versus-benefit profile. Phototherapy also offers a therapeutic alternative to systemic immunosuppressives for patients who cannot tolerate these medications.
...
PMID:Phototherapy for sclerosing skin conditions. 2763 41

Cutaneous chronic graft-versus-host disease (GvHD) has a broad spectrum of clinicopathological presentations, the most common ones being poikiloderma, lichen planus-like eruptions, lichen sclerosus-like lesions, morphea-like plaques, and deep sclerosis. New forms of chronic cutaneous GvHD with different clinicopathological characteristics have been described, most of them mimicking cutaneous manifestations of autoimmune diseases. We report the case of a 35-year-old man who underwent allogenic stem cell transplantation for a therapy-associated acute myeloid leukemia and developed an acute GvHD with involvement of skin and gastrointestinal tract. He subsequently presented with chronic sclerodermatous cutaneous GvHD, followed by the appearance of indurated erythematous papules and plaques located on his back, resembling the nodular/keloidal form of cutaneous scleroderma on both clinical and histopathological grounds. This peculiar clinicopathologic presentation of chronic cutaneous GvHD was never described previously.
 ...
PMID:Nodular Sclerodermatous Chronic Cutaneous Graft-Versus-Host Disease (GvHD): A New Clinicopathological Variant of Cutaneous Sclerodermatous GvHD Resembling Nodular/Keloidal Scleroderma. 2847 13

Lichen sclerosus (LS), also known as lichen sclerosus et atrophicus, is a chronic inflammatory mucocutaneous disease affecting the genital and/or extragenital areas. Although LS usually occurs alone, its coexistence in morphea patients has been reported in 5.7% and 38.0% (genital LS) of cases, in two series (1). A 74-year-old woman presented with a 6-month history of multiple asymptomatic, shiny , indurated, brownish large flat plaques located on the abdomen (Figure 1, a-b) and back, intermingled with slightly atrophic, white-colored, guttate, and patchy areas (Figure 1, d-e). Both punch biopsies of the sclerotic plaques on the back and abdomen showed findings consistent with morphea (Figure 1, c, f). Furthermore, the punch biopsy of a well-demarcated white plaque on the back revealed findings compatible with LS (Figure 1, f). Remarkably, there were also multiple white-colored lesions on the sites of pregnancy-induced striae distensae (SD) (Figure 2, a-b) on the lower abdomen and an old appendectomy scar (Figure 2, c). There was no anogenital involvement. A diagnosis of morphea-LS overlap was established and white lesions located on the surgical scar and SD were clinically evaluated as LS. Methotrexate (15 mg/week) achieved a partial regression of morphea plaques in three months. However, white LS lesions remained unchanged. Our patient presented with coexistence of LS and morphea on different sites of the trunk and on the same lesion. Additionally, one of the isolated LS lesions was located on a surgical scar. Occurrence of LS on skin grafts, irradiated areas, injection sites, or burn/surgical scars has been attributed to the Koebner phenomenon, also called isomorphic response, defined as "the formation of the skin lesions in the same morphology of the existing disease on the areas of various cutaneous injuries" (2). LS is classified under the Koebner category-III (occasional lesions) (2). However, in a case of morphea with features of LS that developed in 1 month following a herpes zoster infection has been suggested to represent "Wolf's isotopic response" (3), which was originally defined as "the occurrence of a new skin disease at the site of another, unrelated and already healed skin disorder" with a time interval between the first and second diseases ranging from months to several years (4). Remarkably, typical morphea plaques in our patient did not involve the surgical scar, in contrast to a cohort in which 16% of 329 patients developed initial morphea lesions at sites of prior (surgery) or ongoing/repetitive (chronic friction) skin trauma (5). SD appear on skin as atrophic linear bands mostly due to rapid weight changes, pregnancy, Cushing syndrome, or prolonged use of corticosteroids (6). The mechanism underlying the occurrence of several diseases on striae is still elusive. Blunt trauma occurring during the development of striae has been suggested to cause the Koebner phenomenon in patients with vitiligo, psoriasis, and lichen planus (7), but it has been suggested that the occurrence of leukemia cutis on SD in a patient reflects Wolf's isotopic response (8). Although chronic graft-versus-host disease, urticarial vasculitis, keloid, lupus erythematosus, diffuse normolipemic plane xanthoma, and drug-induced cutaneous eruptions have been reported to occur on striae (6,9), such an association with LS as in our patient has not been previously documented in the literature. Concomitant occurrence of LS patches on different previous lesions such as a surgical scar and SD in our patient raises the possibility of a common underlying mechanism. As mentioned above, the terms "Koebner phenomenon" or "Wolf's isotopic response" have been used to designate the development of some diseases in injured areas. However, Happle and Kluger (10) claimed in a recent statement that "there is no clear-cut criterion to distinguish isotopic response from Koebner phenomenon and all reactions of this kind represent examples of Koebner phenomenon", which seems to be the best way to describe the site-specific occurrence of LS lesions in our patient.
 ...
PMID:Lichen Sclerosus on the Sites of Striae Distensae and a Surgical Scar in a Patient with Coexistent Morphea. 3103 93


<< Previous 1 2 3 Next >>