In animal models it has been shown that mesenchymal stromal cells (MSC) contribute to skin regeneration and accelerate wound healing. We evaluated whether allogeneic MSC administration resulted in an improvement in the skin of two patients with recessive dystrophic epidermolysis bullosa (RDEB; OMIM 226600). Patients had absent type VII collagen ...

Several studies have been performed to evaluate the efficacy of dietary n-3 fatty acid for patients with renal dysfunction. While about 40% to 80% of patients with end-stage renal disease (ESRD) complain about pruritus and xerosis, there are few reports on the effects of topical n-3 fatty acid on these ...

Four forms of calcinosis cutis exist: metastatic calcinosis, dystrophic calcinosis, idiopathic calcinosis, and subepidermal calcified nodules, usually referred to as cutaneous calculi because single, small, raised, hard nodules are present. Occasionally, there are two or three nodules, and in some instances there are numerous or even innumerable nodules. Most patients ...

We designed the current study to describe the spectrum of disease expression in systemic sclerosis (SSc) in a large cohort and to develop diagnostic criteria for SSc. We assessed patients in the Canadian Scleroderma Research Group Registry by standardized history, physical examination, and laboratory testing. We performed regression tree analysis ...

OBJECTIVES: To examine the 10-year outcome of affected body surface area (BSA), respiratory symptoms, and serum IgE in adult AD patients 6 years after a 4-year intervention with topical tacrolimus. METHODS: Patients who 10 years ago participated in a 4-year, open tacrolimus study (n = 65) were contacted for assessment ...

BACKGROUND: Sulfur mustard (SM) - a chemical agent - has both acute and chronic effects on skin. Xerosis, which is deemed to be due to the damage of hydrolipidic barrier of the skin, is the most common complaint of veterans exposed to the chemical. This study was designed to evaluate ...

OBJECTIVE: To investigate possible correlations between fingertip blood perfusion (FBP) status, assessed by laser Doppler flowmetry (LDF), and morphological microvascular abnormalities, detected by nailfold videocapillaroscopy (NVC), in patients with systemic sclerosis (SSc). The effects on FBP of intravenous (IV) treatment with the prostacyclin analog iloprost were also investigated. METHODS: Thirty-four ...

Formalin-fixed, paraffin-embedded skin tissue sections were collected from a matched cohort of 63 fibromyalgia syndrome (FMS) patients and 49 volunteers from the general population with both alpha1-antitrypsin (AAT) normal and deficiency variants. These tissues were examined for the expression of the broad-spectrum inhibitor AAT, the serine proteinases elastase and tryptase, ...

Lichen planus is a chronic inflammatory disease of mucosa and skin affecting approximately 1-2% of the adult population. Autoimmunity has been implicated in the etiology of this disease, and recently we detected antibodies directed against all six p63 isoforms in sera from 2 out of 20 patients diagnosed with oral ...

GeneScan (GS) analysis is a highly sensitive method for the early detection of cutaneous T-cell lymphoma (CTCL) and allows the identification of clonal heterogeneity, defined as the coexistence of two or more different T-cell clones in multiple samples from the same patient. We analyzed by GS the incidence and the ...

Sclerodermatous skin changes were observed in a patient with porphyria cutanea tarda (PCT) who initially was diagnosed as having progressive systemic sclerosis (PSS). In extremely rare circumstances, patients with PCT initially are misdiagnosed as having generalized morphea, or PSS, because they lack the typical skin findings of PCT, such as ...

A possible relationship between Takayasu arteritis (TA) and tuberculosis (TB) has been suggested. An increased frequency of tuberculin skin test (TST) was observed in TA patients. Quantiferon-TB Gold test (QFT) is a new in vitro assay measuring interferon-gamma response to M. tuberculosis antigens and helpful in diagnosing latent TB infection. ...

Ulceration of the lower limbs is a common debilitating complication of chronic venous hypertension. Detection of preulcerative skin changes would allow for identification of high-risk patients; early active treatment may prevent ulcer formation. Patients with isolated venous disease and volunteers attending outpatient clinics underwent assessment of their clinical, aetiological, anatomical ...

Xeroderma pigmentosum (XP) is a group of rare inherited human neurocutaneous diseases, and the group C (XPC) is the major group of patients with XP in Europe, North America, and South America. Current molecular diagnostic methods for XP require specialized, expensive, and time-consuming UV sensitivity and DNA repair assays followed ...

Hydroa vacciniforme (HV) is a chronic papulovesicular photodermatosis of childhood, with some cases persisting through adulthood. In children, the Epstein-Barr virus (EBV) has been detected in typical HV and in HV evolving into natural killer/T-cell lymphoma. No exploration of EBV infection has been performed in adult patients with HV with ...

The incidence of eczema has been increasing in developed countries. Environmental and hygiene factors have been incriminated. Although air and food pollution with heavy metals have been considered as possible culprits, these factors have never been investigated in Hong Kong. To evaluate if quality of life and eczema severity are ...

To evaluate the construct validity of the European Scleroderma Study Group (EScSG) activity index and to propose modifications if necessary. One hundred and thirty-one consecutive patients were investigated and re-evaluated 1 year later. Modified Rodnan skin score (MRSS), skin ulcers and joint contracture numbers, hand anatomic index (HAI), BMI, spirometry, ...

To obtain an objective, unbiased assessment of skin fibrosis in patients with SSc for use in clinical trials of SSc disease-modifying therapeutics. Skin biopsies from the dorsal forearm of six patients with diffuse SSc and six healthy controls, and skin biopsies from the forearm of one patient with diffuse SSc ...

INTRODUCTION: Incontinentia pigmenti (IP) is a rare complex X-linked genodermatosis in which skin changes are combined with anomalies of other organs. Mutations of the NEMO gene localized on chromosome Xq28 are responsible for IP. Clinical manifestations of IP according to evolution and prognosis can be considered as skin changes and ...

Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited skin disorder caused by mutations in the COL7A1 gene, which encodes collagen VII (COL7). Skin ulcers in RDEB patients are sometimes slow to heal. We describe here the therapeutic response of intractable skin ulcers in two patients with generalized RDEB to treatment ...

AIM: The aim of this study was to assess the correlation between osteogenesis imperfecta (OI) and dentinogenesis imperfecta (DI) from both a clinical and histological point of view, particularly clarifying the structural and ultrastructural dentine changes. DESIGN: Sixteen children (6-12 years aged) with diagnosis of OI were examined for dental ...

BACKGROUND: In daily dermatological practice, many dermatologists do not include demodicosis in their differential diagnoses, or the diagnosis of demodicosis is frequently masked by other skin diseases such as papulopustular or erythematotelangiectatic rosacea, seborrhoeic dermatitis, perioral dermatitis and contact dermatitis. There are two methods for measurement of the density of ...

The use of confocal laser scanning microscopy (CLSM) may be an eligible alternative for confirmation of the diagnosis of hypopigmented macules. Our purpose was to evaluate CLSM features for non-invasive imaging of vitiligo, nevus depigmentosus and postinflammatory hypopigmentation in vivo. A total of 68 patients with a clinical diagnosis of ...

We aimed to investigate the correlation of infrared thermography (IT) with laser Doppler perfusion imager (LDPI) among patients with primary Raynaud's phenomenon and healthy controls. Forty-seven individuals were included: we examined 25 patients with primary Raynaud's phenomenon and 22 age and gender matched healthy controls. IT of the volar surface ...

Interventional cardiac procedures not only lead to significant effective doses for the patient but also can potentially cause deterministic effects on the patient's skin. Information about the peak (maximal) skin doses (PSD) received by patients during percutaneous transluminal coronary angioplasty procedures were collected from three cardiac catheter rooms. Cumulative dose ...

Acute graft-versus-host disease (aGVHD), if not detected and treated early, is a common cause of morbidity and mortality. Drug hypersensitivity reactions (DHRs), the most frequent clinical and histopathological mimickers of early aGVHD, are often still distinguished from aGVHD by the presence of eosinophils within the inflammatory infiltrate on skin biopsy. ...

OBJECTIVES: To describe clinical and immunohistochemical findings in patients with cutaneous Langerhans cell histiocytosis (LCH) beginning in the first 3 months of life and to define predictors of disease evolution. DESIGN: Observational retrospective survey from July 15, 1989, to April 30, 2007. SETTING: Referral center in pediatric dermatology. PATIENTS: Thirty-one ...

BACKGROUND: Specific cutaneous lesions of Waldenstr?m macroglobulinemia are rare and include neoplastic cell infiltrates, IgM bullous disease, and so-called IgM-storage papules, which characterize cutaneous macroglobulinosis (CM). OBSERVATIONS: We report 2 patients with CM. In patient 1, CM started as small papules, as reported in most of the previously published case ...

BACKGROUND: Chronic venous insufficiency (CVI) comprises all symptoms caused by permanent venous and capillary hypertension. While the clinical manifestations of the disease have been well characterized, there is little knowledge on the skin barrier function in the affected patients. OBJECTIVES: The aim of the study was to assess noninvasively the ...

Eyelid bags are considered a sign of ageing, but they often appear prematurely due to the variety of causes that favor them. This brief report describes the case of a patient who was referred to us for the correction of a second degree bilateral palpebral ptosis that the patient had ...

Atopic dermatitis is a chronic inflammatory disease, usually associated with a personal or family history of atopic diseases such as AD, allergic rhinitis or asthma that most commonly arise in childhood and present with elevated IgE serum in up to 85% of patients. The severity of AD is based on ...

BACKGROUND AND OBJECTIVES: In recent years, the genus Malassezia has come to be considered important in the etiology of seborrhoeic dermatitis (SED). The aim of present study was identification of Malassezia species on the lesions of Iranian SED patients. METHODS: 100 patients with SED were enrolled in the study. The ...

Oral lichen planus (OLP) is a chronic autoimmune disease characterized by multiple clinical presentations and a relatively high prevalence in the population. This retrospective patient record study investigated the profile of OLP in a group of Brazilian patients seen between 1989 and 2009. The clinical records were analyzed and data ...

AIMS: To evaluate the presence of temporomandibular disorders (TMD) in systemic sclerosis (SSc) patients and its possible association with the severity of skin involvement. METHODS: The presence of TMD was evaluated in 35 SSc women and 30 age- and sex-matched healthy controls by means of the anamnestic (A(i)) and clinical ...

To determine if excretion of sorafenib in sweat is associated with hand-foot skin reaction in patients receiving sorafenib. Prospective pilot study. Outpatient clinic of a cancer research institution. Two patients who were receiving sorafenib and developed a hand-foot skin reaction of at least grade 1 and two healthy subjects (controls). ...

The aim of the study was to investigate the DNA polymorphic genotype in MMP-2 promoter gene as a potential candidate region for the development of the cutaneous T-cell lymphoma (CTCL) and/or its progression. A total of 89 Czech patients with CTCL (including 23 patients with large plaque parapsoriasis) were compared ...

Chronic graft versus host disease (cGVHD), the most common late complication of allogeneic haematopoietic stem cell transplantation (HSCT), may present with sclerodermatous lesions resembling in some cases the cutaneous involvement of systemic sclerosis (SSc). Certain pathogenetic findings connect the two diseases. In this report we describe ten subjects affected by ...

BACKGROUND: Patients with atopic dermatitis show a defective barrier function. In atopic skin, ceramide is significantly decreased and the secretion of lamellar bodies is also impaired. To mimic lamellar bodies, we prepared lipid granules composed of ceramide, fatty acids and cholesterol. Because these lipid granules contain multiple lamellar structures, it ...

Although the term, "trichothiodystrophy" (TTD) refers to the hair anomalies in this group of patients, this is a heterogeneous, multisystem disease in which any or every organ in the body may be affected. Neuroectodermal derived tissues are particularly likely to be involved. This term was introduced by Price et alin ...

OBJECTIVES: To characterise the clinical phenotype of Italian patients with adult-onset Still's disease (AOSD). METHODS: Sixty-six subjects who received a definite diagnosis of AOSD were seen and followed-up at our institution from 1991 to 2009. The diagnosis was made by a senior rheumatologist and confirmed by Yamaguchi's criteria for AOSD. ...

To develop and assess the psychometric properties of the Localized Scleroderma (LS) Skin Damage Index (LoSDI) and Physician Global Assessment of disease Damage (PGA-D). Damage was defined as irreversible/persistent changes (>6 months) due to previous active disease/complications of therapy. Eight rheumatologists assessed the importance of 17 variables in formulating the ...

Cutaneous Malassezia is an exacerbating factor in patients with atopic dermatitis. We analysed the Malassezia microbiota of adult patients with head and neck atopic dermatitis of different severities (mild, moderate and severe). Of the nine human-associated Malassezia species, the number detected was similar (3.5-4.2 species per case) among the members ...

Epidermolysis bullosa pruriginosa (EBP) is a clinical variant of dominant or occasionally recessive, dystrophic epidermolysis bullosa (EB). Clinically, intense pruritus on a background of inherited skin fragility often leads to skin signs that resemble acquired inflammatory disorders such as hypertrophic lichen planus (LP) or nodular prurigo. Moreover, symptoms and signs ...

This study was performed on seven patients affected by the atrophic form of age-related macular degeneration (AF-ARMD). The patients under investigation belonged to a larger study aimed at evaluating the efficacy of rheopheresis treatment (RT) on the visual function of AF-ARMD patients. Following the protocol of the larger study, patients ...

We describe an 18 year-old male patient who was anti-Ro/SS-A and anti-La/SS-B positive and presented with recurrent annular plaques on the trunk, arms, face, and scalp with evidence of associated patchy alopecia. The skin biopsy revealed a superficial perivascular and periappendegeal lymphocytic infiltrate and focal areas of vacuolar alteration and ...

BACKGROUND: To date, there has been no systematic investigation of the detailed histological features of prurigo nodularis (PN) in a large cohort of patients. METHODS: This retrospective study includes skin biopsies of 136 patients (63 males, 73 females; mean age: 58.38 years) with PN. RESULTS: Highly characteristic for PN is ...

PURPOSE: This study examines the histology of conjunctival biopsy samples from patients with persistent allergic eosinophilic conjunctivitis (AEC) or non-allergic eosinophilic conjunctivitis (NAEC). METHODS: Fourteen patients with conjunctivitis and eosinophilia in cytology samples were included in the study. Seven had positive skin-prick tests (the AEC group) and seven had negative ...

Atopic myelitis is defined as myelitis with atopic diasthesis but the cause is still unknown. Toxocariasis is one of the common causes of hyperIgEaemia that may lead to neurologic manifestations. The purpose of this study was to evaluate the sero-prevalence of Toxocara specific IgG Ab among the atopic myelitis patients. ...

OBJECTIVE: Relaxin may potentiate the effect of topical estrogen treatment to eradicate post-incisional scarring in congenital adrenal hyperplasia (CAH) patients undergoing genitoplasty. The aim of this study was to determine whether CAH skin is capable of responding to relaxin. PATIENTS AND METHODS: Skin biopsies were obtained from four female CAH ...

We previously described a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America (El Bagre-EPF). On physical examination, the palms and soles of El Bagre-EPF patients reveal an edematous texture and mild hyperkeratosis, in comparison with the non-glabrous skin of the patients where blisters, pustules or other ...