Widespread Erosions Research Articles

Mesozoic-Cenozoic Exhumation Processes of the Harlik Mountain (East Tianshan), NW China: Evidence from Apatite (U-Th)/He Thermochronology

Abstract The Tianshan Mountains, which formed through the prolonged and complex subduction-accretion of the paleo-Asian ocean, experienced multiple widespread erosions and reactivations during the Mesozoic-Cenozoic period. Thus, the Tianshan has become a crucial area to explore the process and mechanism of intracontinental orogeny. The Harlik Mountain located in the easternmost part of Tianshan has special significance in revealing crustal deformation and far-field stress propagation. In this study, we use the apatite (U-Th)/He method to investigate the near-surface exhumation process of Harlik Mountain, which is the result of tectonic events and erosion. The apatite (U-Th)/He data and thermal history modeling reveal three enhanced exhumation events in the Harlik Mountain, i.e., the early Cretaceous, the late Cretaceous-Paleocene, and the middle Eocene. These events are consistent with the exhumation patterns observed throughout the entire Tianshan. Notably, the exhumation rate and amplitude in the early Cenozoic period were significantly higher in the Harlik Mountain compared with the Mesozoic period. Our findings reveal that the exhumation of the Harlik Mountain and the entire Tianshan displayed pronounced spatial-temporal variations throughout the Mesozoic-Cenozoic period. These variations can be attributed to the combined effect of uneven northward compression of the southern Asian margin, the heterogeneous rheological properties of the lithosphere, and the complex structure of the Tianshan.

P25 TB or not TB? An unusual case of inflammatory arthritis in an anti-CCP positive patient

P25 TB or not TB? An unusual case of inflammatory arthritis in an anti-CCP positive patient

Widespread Erosions in Intertriginous Areas

Widespread Erosions in Intertriginous Areas

Follicular lymphoma manifests as multiple erosive and proliferative lesions of the oral mucosa: case report and brief literature review

BackgroundErosion is one of the most common and basic lesions of oral mucosal diseases. Long-term refractory oral erosions, induced by autoimmune blistering diseases, infectious diseases, malignant diseases, and some rare conditions, may substantially reduce the quality of life of patients or even constitute a life-threatening condition, resulting in a clinical dilemma regarding the accurate diagnosis and precise management of these diseases. As a special type of malignant lymphoma, most lesions of follicular lymphoma (FL) in the oral mucosa present as masses or swelling of the oral mucosa, while emerging novel presentations lead to intractable diagnoses. Hence, diagnostic algorithms for such diseases are clinically required. Case presentationA 55-year-old female patient presented to the clinic with long-lasting oral mucosal erosions and proliferative lesions. Blood tests, pathological examinations of oral lesions including haematoxylin–eosin (HE) staining, and direct immunofluorescence precluded all of the potential diagnoses described previously. Unexpectedly, positron emission tomography/computed tomography (PET/CT) and abdominal CT of the patient revealed a dense mass in the retroperitoneal area, and the final diagnosis of the retroperitoneal mass was FL. After three courses of chemotherapy conducted by the haematologist, the erosion and proliferative lesions in the patient's oral mucosa had significantly improved. HE and immunohistochemical staining results of intraoral lesions also confirmed it as oral FL. The successful diagnosis of FL in this case is of great clinical significance, as the oral and abdominal FL were treated in a timely manner to avoid unfavourable outcomes.ConclusionsTo the best of our knowledge, this is the first case of FL that exhibited widespread erosions interspersed with proliferative lesions. Clinicians should be aware of oral FL or seek systemic factors in the presence of similar refractory oral erosions when treatment is non-responsive and the diagnosis is intractable.

A Fatal Case of Febrile Ulceronecrotic Mucha-Habermann Disease in a 10-Year-Old Boy.

A Fatal Case of Febrile Ulceronecrotic Mucha-Habermann Disease in a 10-Year-Old Boy.

A Sinister Disease Hides Behind Recalcitrant Mucocutaneous Disorder

Paraneoplastic pemphigus (PNP) is a rare autoimmune disorder associated with underlying benign or malignant neoplasia. Its signs and symptoms may be the first presentation of a concealed malignancy. Due to late diagnosis, prognosis of PNP is not good, so early diagnosis and treatment is of paramount importance. We present a case of 22 years old female who presented to our out patient department (OPD) with history of recurrent, severe, recalcitrant, painful oral ulcers; lichenoid lesions over the hands and feet and widespread blistering and erosions involving the soles. Histopathological examination was consistent with paraneoplastic pemphigus and showed features of lichenoid dermatitis. CT scan revealed retroperitoneal mass suggestive of Castleman disease. The patient was referred to surgical team for further management.

Superadded chromomycosis in recessive dystrophic epidermolysis bullosa: An easily misdiagnosed entity

Recessive dystrophic epidermolysis bullosa (RDEB) (Hallopeau Siemens) is one of the most severe forms of epidermolysis bullosa (EB) with generalized vesiculation over the skin and mucous membrane resulting in extensive scarring with exuberant granulation tissue. Because of widespread erosions, patients are prone to secondary infection. We are reporting a 10-year-old male child having RDEB who presented with a nonhealing ulcer with verrucous growth over bilateral knees for 2 years which was considered as part of the disease by many dermatologists and was later diagnosed to be superadded chromoblastomycosis based on histopathology. We are highlighting this case to increase awareness among dermatologists regarding the occurrence of superadded deep fungal infections like chromoblastomycosis in patients of RDEB which is usually missed or misdiagnosed as part of the disease.

Ingenol mebutate induces a tumor cell-directed inflammatory response and antimicrobial peptides thereby promoting rapid tumor destruction and wound healing

BackgroundIngenol mebutat (IM)-gel is effective for the topical treatment of epithelial tumors, including actinic keratoses (AKs) or anogenital warts (AGW). AK patients treated with IM develop intensified inflammatory reactions on sights of prior clinical visible or palpable AKs as compared to the surrounding actinically damaged skin, suggesting the induction of a tumor cell-directed inflammation. AGW patients treated with IM develop even stronger inflammatory reactions with large erosions, suggesting a directed inflammatory response against HPV-infected keratinocytes. Of note, even widespread erosions heal very fast without any superinfections. Here, we set out to elucidate underlying molecular and cellular mechanisms of these clinical observations.MethodsThe effects of IM (10−9–10−5 M) on the expression and translation of a comprehensive set of chemokines (CXCL1, CXCL8, CXCL9, CXCL10, CXCL11, CXCL14, CCL2, CCL5, CCL20, CCL27) and antimicrobial peptides (AMP) (HBD1, HBD2, HBD3, LL37, RNase7) were analyzed in primary human epithelial keratinocytes (HEK) and a set of epithelial cancer cell lines by RT-qPCR and ELISA in vitro. To study the possible effects of different concentrations of IM on migratory, respectively wound healing responses, an in vitro scratch assay was conducted on HEK.ResultsIngenol mebutat significantly and dose-dependently induced the expression of proinflammatory chemokines (CXCL8, CCL2) and AMP (RNase7, HBD3) in HEK and epithelial cancer cell lines. A significantly stronger induction of CXCL8 and CCL2 was observed in our tested tumor cells as compared to HEK. We did not observe any significant effect of IM on HEK migration, respectively wound healing responses in vitro for any tested concentration (10−9, 10−8, 10−6 M) except 10−7 M, which induced a significant inhibition.ConclusionsOur data suggest that tumor cells are more susceptible to IM as compared to differentiated HEK. This is evident by a stronger IM-mediated induction of proinflammatory chemokines in tumor cells, which may result in a tumor cell-directed inflammatory response and rapid tumor destruction. In addition, IM induces AMP in keratinocytes and seems not to severely interfere with keratinocyte migration, which contributes to a fast and uncomplicated wound healing. Surprising is a selective inhibition of keratinocyte migration by IM at the concentration of 10−7 M pointing to very dose depending biological effects, induced by IM.

Paraneoplastic Pemphigus: A Case Report

Paraneoplastic pemphigus (PNP) is a rare autoimmune bullous disease associated with underlying neoplasms. Targetoid lesions, intractable stomatitis and refractory course of disease are some of the characteristic features of paraneoplastic pemphigus. It is usually associated with lymphoproliferative disorders, and rarely with solid tumors. We present a case of a 35 years old female with a six-months history of recurrent, severe, recalcitrant stomatitis and widespread blistering and erosions with involvement of palms and soles. A provisional diagnosis of PNP was made based on clinical features. Investigations for an underlying neoplasm revealed presence of solid lesion of size 6.7 x 6.4 cm in left adnexa, likely an ovarian tumor. PNP should be considered in the differential diagnoses in severe atypical mucocutaneous manifestations of pemphigus vulgaris and diagnostic screening for search for an underlying tumor should be performed.

Gastroduodenal involvement in disseminated strongyloidiasis.

Here, we report the case of a 57-year-old Peruvian man on long-term corticosteroid therapy for a drug allergy and proton pump inhibitors for chronic dyspepsia symptoms. Upper gastrointestinal endoscopy showed multiple white granular lesions and widespread erosions in the stomach. Our findings indicate that gastric strongyloidiasis should be carefully considered in high-risk patients even if endoscopic findings are nonspecific.

A New Mouse Model of Junctional Epidermolysis Bullosa: The LAMB3 628G>A Knockin Mouse

Junctional epidermolysis bullosa (JEB) is a group of recessively inherited genodermatoses, characterized by tissue separation in the epidermal basement membrane due to defective anchoring proteins. The lethal Herlitz type of this disorder is caused by absence of laminin332. Affected individuals suffer from widespread erosions of skin and mucous membranes and very often die within the first year of life. Mouse models lacking the a3or g2-chain of laminin-332 have been developed and a spontaneous s3-knockout mouse exists, but all die shortly after birth. We generated a new mouse model of JEB by knockin of the point mutation 628G>A (p.E210K) in the gene encoding the laminin-332 s3chain, LAMB3. In compound heterozygous humans, this mutation has always been associated with lifelong skin blistering without reduced life expectancy. Fourteen homozygous LAMB3 628G>A knockin mice were analyzed. Most of them showed skin blistering with tissue separation in the basement membrane soon after birth. Laminin-332 was almost completely absent. None of the homozygous LAMB3 628G>A knockin mice survived longer than 72 hours. LAMB3 gene expression levels in heterozygous and homozygous LAMB3 628G>A knockin mice, however, were similar to wild-type mice. Analysis of the LAMB3 transcript revealed alternative splicing in homozygous LAMB3 628G>A knockin mice: a 64 base-pair deletion of exon 7 led to a frame-shift and a premature termination codon. Due to alternative splicing, the phenotype of this new mouse model resembles that of knockout mice. The LAMB3 628G>A knockin mouse may contribute to a better understanding of the molecular basis of JEB. Authors’ details Department of Pediatrics and Adolescent Medicine, University Hospital Erlangen, Erlangen, Germany. Department of Biology, Nikolaus-FiebigerCenter for Molecular Medicine, Friedrich-Alexander-University ErlangenNurnberg, Erlangen, Germany.

A new mouse model of junctional epidermolysis bullosa: the LAMB3 628G>A knockin mouse

Junctional epidermolysis bullosa (JEB) is a group of recessively inherited genodermatoses, characterized by tissue separation in the epidermal basement membrane due to defective anchoring proteins. The lethal Herlitz type of this disorder is caused by absence of laminin-332. Affected individuals suffer from widespread erosions of skin and mucous membranes and very often die within the first year of life. Mouse models lacking the α3- or γ2-chain of laminin-332 have been developed and a spontaneous s3-knockout mouse exists, but all die shortly after birth. We generated a new mouse model of JEB by knockin of the point mutation 628G>A (p.E210K) in the gene encoding the laminin-332 s3-chain, LAMB3. In compound heterozygous humans, this mutation has always been associated with lifelong skin blistering without reduced life expectancy. Fourteen homozygous LAMB3 628G>A knockin mice were analyzed. Most of them showed skin blistering with tissue separation in the basement membrane soon after birth. Laminin-332 was almost completely absent. None of the homozygous LAMB3 628G>A knockin mice survived longer than 72 hours. LAMB3 gene expression levels in heterozygous and homozygous LAMB3 628G>A knockin mice, however, were similar to wild-type mice. Analysis of the LAMB3 transcript revealed alternative splicing in homozygous LAMB3 628G>A knockin mice: a 64 base-pair deletion of exon 7 led to a frame-shift and a premature termination codon. Due to alternative splicing, the phenotype of this new mouse model resembles that of knockout mice. The LAMB3 628G>A knockin mouse may contribute to a better understanding of the molecular basis of JEB.

Pemphigus Vulgaris: Pentingnya Diagnosis Dini, Penatalaksanaan yang Komprehensif dan Adekuat (Laporan Kasus)

Pemphigus vulgaris is an autoimmune–mediated disease of skin and mucous membran leading to progressive blistering and chronic erosions. It often begins with blister formations which easily rupture. The characteristic feature is positive nikolsky sign which may or not be presented simultaneously. Establishment of early definite diagnosis is critical and requires correlation of clinical and histopathological findings. Because of this conditon is a potentially life-threatening, the risk of complications and mortality rate increases if initial management is non comprehensive and inadequate. Treatment is directed at supression of autoimmune process, typically administration of corticosteroids. This article report a case in a 51 years old woman who had painful chronic oral ulcer and poor general health condition. Prior to the visit to Oral Medicine clinic, patient was treated by her general practitioners for several months, without either established diagnosis nor comprehensive and adequate management, so that she had no clinical improvement. Clinical examination at the first visit in Oral Medicine clinic Cipto Mangunkusumo Hospital, revealed bula in the skin, conjunctivitis, easily bleed oral mucosae, widespread erosions and ulcerations of the lips, gingiva, tongue, and buccal mucosae. Biopsy of one of skin erosions demonstarting suprabasal intraepithelial acantholysis. Multi divisions in Hospital, such as Oral Medicine, Dermatology, Internal Medicine, Ophtalmology, ENT, were involved in treating this case. Patient received high dose methylprednisolone sistemically and prednisone topically for the lips. General remission achieved in several days. Thus, establishment of early definite diagnosis and adequate management are important in management of Pemphigus vulgaris. DOI: 10.14693/jdi.v16i1.20

Inhibitory human antichimeric antibodies to rituximab in a patient with pemphigus

Inhibitory human antichimeric antibodies to rituximab in a patient with pemphigus

Junctional epidermolysis bullosa in a calf

A case of epidermolysis bullosa in a calf descendent from a Gir bull and a Gir crossbreed cow is reported. The calf presented with exungulation of all hooves, widespread erosions and crusts on the skin, and ulcers in the oral cavity. Histologically, the skin showed subepidermal separation with clefts occasionally filled with eosinophilic clear fluid, cellular debris, or neutrophils. Ultrastructurally, there was epidermal-dermal separation at the level of the lamina lucida, with the lamina densa attached to the papillary dermis. The hemidesmosomes were poorly defined and small. The clinical, histological, and ultrastructural findings are characteristic of junctional epidermolysis bullosa.

Congenital Lupus Erythematosus Presenting at Birth with Widespread Erosions, Pancytopenia, and Subsequent Hepatobiliary Disease

Neonatal lupus erythematosus is an uncommon disease caused by transplacental passage of maternal anti-Ro (SS-A), anti-LA (SS-B), or anti-U1RNP antibodies. Cutaneous findings of neonatal lupus are variable, but annular, erythematous plaques occurring within a few weeks of birth are most typical. Cutaneous lesions of congenital onset lupus erythematosus can differ from that of neonatal lupus erythematosus, presenting with atrophy or scarring, and less commonly, erosions. We report an unusual case of congenital lupus erythematosus presenting at birth with widespread erosions, pancytopenia, and subsequent hepatobiliary disease.

Congenital herpes simplex virus infection: Two unique cutaneous presentations associated with probable intrauterine transmission

Cutaneous manifestations of congenital herpes simplex virus (HSV) have been classically described as grouped vesicles on an erythematous base. We report two cases of HSV infection wherein both infants presented at birth with widespread erosions and an absence of vesicles or vesicopustules. The presence of skin lesions at birth, neurologic changes seen on radiographic imaging, and a cesarean section delivery in one case suggests intrauterine transmission in both neonates.

Nebenwirkungen der topischen photodynamischen Therapie

Photodynamic therapy (PDT) is gaining increasing significance in the treatment of skin cancers and precancerous lesions. Most of the studies dealing with this procedure focus on efficacy. Whereas treatment results are a decisive parameter, before any therapy is accepted the evaluation should also consider side effects in daily practice. We treated 2031 patients with PDT between 2001 and 2006, and evaluated the side effects during and after treatment with a specially designed protocol. The most prominent side effect was pain, which occurred in 92% of patients, followed by erythema/edema (89%) and scaling in conjunction with itching (80%). Less common unwanted effects included crusting (9%), pustules (6%), large erosions (1.2%), pigmentary changes (1%) and infections (0.4%). Especially widespread erosions and pustules caused significant anxiety for the patients. Patients should be informed in detail about the side effects of PDT prior to treatment, since most of the unwanted effects are easily managed, heal without scarring and do not influence the therapeutic response.

Cicatricial pemphigoid vegetans

A 58-year-old woman with ulcerative colitis of 5 years' duration was first seen in March 2002 with a 5-month history of widespread erosions on the palate and gingiva, subtle scarring of the conjunctiva, and xerostomia accompanying a flare of ulcerative colitis. No skin lesions were observed. Direct immunofluorescence study performed on the oral mucosa showed a linear distribution of immunoglobulin G (IgG) and immunoglobulin A (IgA) at the dermal-epidermal junction. Indirect immunofluorescence demonstrated the presence of circulating IgG anti-basement membrane zone antibody. This patient was successfully treated with sulfasalazine 3 g/day. Two years later, however, she developed extensive, well-defined pustules and vegetating erosions on the skin, symmetrically localized in the inguinal and axillary folds and on the upper inner thighs (Fig. 1). At that time, mucous membrane involvement, except for subtle scarring conjunctivitis, was not observed. Laboratory findings were normal, except for colonoscopy, which disclosed cobblestoning and histologically well-circumscribed granulomas, confirming the diagnosis of ulcerative colitis. On the basis of the clinical features and the flare of coexistent ulcerative colitis, this patient was suspected of having pyoderma gangrenosum. Skin biopsy specimens showed acanthosis, papillomatosis, pseudo-carcinomatous hyperplasia, and subepidermal blisters overlying an infiltrate of leukocytes and eosinophils, which also extended into the lower epidermis (Fig. 2). Direct immunofluorescence of the perilesional skin demonstrated a linear distribution of IgG and IgA at the dermal-epidermal junction. In vivo-bound IgG within the basement membrane zone was documented using laser scanning confocal microscopy, which disclosed the presence of immunoglobulins above collagen type IV and below laminin-5 (Fig. 3). Indirect immunofluorescence study showed the presence of circulating IgG anti-basement membrane zone antibody reacting with the roof and floor of salt-split skin. Characterization of the target antigen (courtesy of Professor Hashimoto of Kurume University, Kurume, Japan) was unsuccessful, as Western immunoblot study on both epidermal and dermal extracts was negative, as was immunoblot with purified laminin-5 and enzyme-linked immunosorbent assay with the NC16a epitope of BP180. Laser scanning confocal microscopy confirmed the diagnosis of cicatricial pemphigoid. Therapy consisting of prednisone 40 mg each morning and sulfasalazine 1.0 g three times daily led to rapid improvement of the skin lesions, but did not affect the conjunctival lesions. After 4 weeks, prednisone was tapered and sulfasalazine was continued at a reduced dose of 0.5 g three times daily. At that time, only post-inflammatory hyperpigmentation and atrophic scars were evident.

Paraneoplastic Pemphigus in a Dog with Splenic Sarcoma

Paraneoplastic pemphigus (PNP) is an autoimmune blistering skin disease of humans that consists of characteristic skin lesions associated with concurrent neoplasia. In this study we provide histologic and serologic evidence to support a diagnosis of PNP in a dog with splenic sarcoma. Skin lesions consisted of widespread erosions involving haired skin, mucocutaneous junctions, and oral mucosa. Microscopic examination of skin and mucosae revealed lesions consistent with both pemphigus vulgaris and erythema multiforme. Immunoprecipitation confirmed that circulating IgG autoantibodies from this patient recognized five distinct antigens, presumed to represent epidermal plakins. Clinical, histopathologic, and immunologic findings in this patient were similar to those observed in human patients with PNP. The splenic neoplasia in this dog was diagnosed as a phenotypically variable spindle cell sarcoma. To date, only one other dog has been reported with PNP. This is the second reported case of canine PNP and the first patient in whom skin lesions were identified in association with splenic neoplasia.