Dermatologic Treatment of Cutaneous Graft Versus Host Disease

Cutaneous involvement in graft versus host disease (GVHD) after allogeneic hematopoietic cell transplant can be separated into acute GVHD (aGVHD), lichenoid chronic GVHD (cGVHD) and sclerodermatous cGVHD. It seems clear that these syndromes result from different mechanisms and entail different treatment approaches. Standard treatment of cutaneous aGVHD involves the intensification of immunosuppressive therapy with adequate topical supportive management. In skin-limited disease, phototherapy has shown promising results. In cutaneous cGVHD, the combination of corticosteroids and cyclosporine (ciclosporin) is the recommended therapy, and other immunosuppressants may be added depending on whether lichenoid or sclerodermatous lesions are present. High response rates to phototherapy have been found in lichenoid disease, while sclerodermatous disease responds better to etretinate or extracorporeal photochemotherapy. Localized cutaneous cGVHD may be treated with topical corticosteroids alone. Few reports on the effect of treatments in GVHD clearly describe the cutaneous involvement and the influence of the treatment on the skin. Therefore, dermatologists should be deeply involved in the diagnosis and treatment of GVHD, and good dermatologic grading systems should be developed. Theses changes will increase our knowledge of cutaneous GVHD, and relevant data in the evaluation of the effect of therapy in the disease will be obtained.     

Efficacy of Acitretin for Porokeratosis in a Child with Chronic Cutaneous Graft Versus Host Disease

Abstract: Porokeratosis is a rare disorder of epidermal keratinization that is regarded as a precancerous. Recipients of hematopoietic stem cell transplantation (HSCT) have a greater risk of skin cancer; chronic graft versus host disease (GVHD) is an additional risk factor. A 16‐year‐old boy who had received HSCT for acute myelogenous leukemia was referred to us for sclerodermoid chronic cutaneous GVHD. Two years later, he developed disseminated porokeratosis with a few atypical lesions. Despite cryotherapy, numerous lesions of porokeratosis recurred rapidly. Acitretin resulted in good clinical response and reduced the rate of onset of new lesions.     

Cutaneous Graft versus Host Disease in Pediatric Multivisceral Transplantation

Abstract: Multivisceral transplantation (MvTx) is the concurrent transplantation of the stomach, pancreaticoduodenal complex, and intestine, with or without the liver. Its use is increasing worldwide as it has been considered as a therapy for patients with functional disturbance of several organs. Graft‐versus‐host disease (GvHD) has been a relevant clinical problem in MvTx ever since the procedure was first performed, but little has been reported about its specific cutaneous features. Our study included all pediatric patients with clinical and histopathologic evidence of cutaneous GvHD who received MvTx between October 1999 and December 2010 in University Hospital La Paz. Seventeen children underwent MvTx at our center during this period of time. Five patients developed cutaneous GvHD (29.4%). The median onset was 45.2 days after transplantation. Acute cutaneous GvHD, consisting of symmetrical maculopapular exanthema with prominent acral erythema and accentuated lesions on the face and pinnae, was clinically suspected and pathologically confirmed in four patients (80%). Three children (60%) experienced disease progression to a formation and a positive Nikolsky sign. Only one girl (20%) showed lichenoid GvHD. The first therapeutic approach was steroids and tacrolimus adjustment; many other drugs were used in refractory cases. Three of the five patients (60%) died with concomitant GvHD, the immediate cause of death being another comorbid disease. Knowledge of the features of cutaneous GvHD in MvTx allows clinicians early recognition and prompt therapeutic intervention that may prevent progression to higher‐grade disease and improve outcomes for these patients.     

Graft‐versus‐Host‐Disease: Ein interdisziplinäres Problem aus der Sicht des Dermatologen

Graft‐versus‐host disease (GVHD) is a frequent complication following hematopoietic stem cell transplantation. Acute and chronic GVHD are identified based on the onset of clinical symptoms and signs. Whereas acute GVHD is relatively uniform in its appearance, chronic GVHD is characterized by a broad spectrum of clinical manifestations. The aim of this review is to introduce the reader to the pathophysiologic processes underlying GVHD, to demonstrate its manifestations in the skin and to review current therapeutic options.     

具有白癜风样表现的慢性移植物抗宿主病1例

患者男,21岁。口腔溃疡、全身发疹伴眉毛、睫毛变白2周。6个月前患者因"急性淋巴细胞白血病"行异体造血干细胞移植。查体示:躯干部弥漫性分布暗红斑,伴脱屑和苔藓样改变,口腔、舌黏膜白斑、糜烂出血,双侧眉毛和眼睫毛部分呈白色。诊断:慢性移植物抗宿主病。系国内首例局限于眉毛和睫毛的具有白癜风样表现的慢性移植物抗宿主病。     

硬皮病样慢性移植物抗宿主病3例

报告3例硬皮病样慢性移植物抗宿主病。患者均为男性，平均年龄48岁。因原发性血液系统疾病在化疗缓解后接受异体骨髓移植，移植后平均12．7个月出现仝身弥漫性硬化或泛发性硬斑，硬化严格以手腕、踝部和颈部为界，未累及肢体远端和面部，无雷诺现象和毛细血管扩张。其中2例伴有不同程度的肺间质病变。3例患者抗核抗体（ANA）、类风湿因子(RF）、抗Scl-70抗体和抗着丝点抗体均为阴性，组织病理检查显示真皮网状层胶原纤维束增粗、密集和肿胀。3例患者均接受中等剂量的糖皮质激素治疗，其中2例治疗1个月后皮肤明显软化，另1例软化不明显。     

皮肤混合瘤

报告1例皮肤混合瘤.患者男,78岁.右鼻唇沟一肤色结节,无自觉症状,病程1年.皮损组织病理检查:真皮内见一限局性肿瘤,在嗜碱性基质中可见上皮细胞构成的不规则囊腔和管腔.     

Giant Mixed Tumor of the Face

A 35-year-old Japanese man consulted our clinic with an eight year history of a 6 cm diameter subcutaneous tumor on the left cheek. Hematoxylin and eosin staining of the resected section showed histology corresponding to a benign chondroid syringoma. Keratin was positive in most of the constituent cells, and S-100 protein was positive in the cells distant from the lumens and in myxomatous cells. A benign chondroid syringoma of this size has only been very rarely reported in the literature.     

Toxic Epidermal Necrolysis Possibly Linked to Hyperacute Graft-versus-Host Disease after Allogeneic Bone Marrow Transplantation

Toxic epidermal necrolysis (TEN) is a severe blistering skin disease of high mortality. TEN may occur after bone marrow transplantation (BMT). In such cases, TEN have been attributed to graft-versus-host disease (GVHD) or an adverse drug reaction. It is very difficult to distinguish the causes of TEN after BMT. We report a 21-year-old Japanese man who developed TEN eight days after BMT, evaluate the differential diagnosis of hyperacute GVHD and an adverse drug reaction, and deduce that hyperacute GVHD was the more likely pathogenesis of TEN in this patient.     

皮肤混合瘤12例

对我院2008年至2020年6月12例皮肤混合瘤患者资料进行回顾性分析。12例患者中男7例,女5例,发病年龄为22~77岁。12例均发生于面部 (上唇部最多见),其中11例表现为单发无自觉症状的淡红色或肤色坚韧肿物,1例因外界因素出现破溃。组织病理示9例向大汗腺分化,3例向小汗腺分化。12例患者中,临床诊断为皮肤混合瘤仅1例,诊断为皮肤纤维瘤和表皮样囊肿的各3例,诊断为钙化上皮瘤、附属器肿瘤和皮角各1例,皮疹待查2例。12例患者均行手术切除,随访2个月~6年,均无复发。     

丙型肝炎病毒感染与原发性混合型冷球蛋白血症关系的研究

目的　探讨丙型肝炎病毒 (HCV)感染与原发性混合型冷球蛋白血症 (EMC)发病的关系。方法　采用酶联免疫吸附试验 (ELISA)、聚合酶链反应 (PCR)法检测 45例EMC患者血清HCV感染情况。结果　ELISA法检测HCV抗体阳性率为 75 .5 6% ,PCR法检测HCV RNA阳性率为 77.78%。结论　EMC的发生可能与HCV感染有关     

MCTD – Mixed Connective Tissue Disease

Mixed connective tissue disease is a disease entity characterized by overlapping symptoms of lupus erythematosus (LE), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA). Diagnostic criteria include high titers of antibodies against U1RNP as well as the presence of at least 3 of 5 of the following clinical features: edema of hands, synovitis, myositis, Raynaud phenomenon and acrosclerosis. In terms of the pathogenesis, genetic as well as infectious (viral) factors appear to play a role. The acceptance of MCTD as a distinct disease entity is controversial. Terms such as ”undifferentiated connective tissue disease“ or ”overlapping syndromes“ are not helpful. One‐quarter of MCTD patients transform into LE, while one‐third progress to SSc. Therapeutic recommendations are glucocorticoids in combination with immunosuppressive agents and endothelin receptor antagonists. Double blind studies are not available. The prognosis is relatively good. Causes of death include pulmonary hypertension, infections and both pulmonary and cardiac failure.     

Mixed vitiligo of Blaschko lines: a newly discovered presentation of vitiligo responsive to combination treatment

Vitiligo, depigmenting disorder of the skin and mucous membranes, affects up to 1% of the population worldwide. It is classified into four major types: segmental, non‐segmental, mixed, and unclassified type. Non‐segmental vitiligo refers to non‐dermatomal distribution of lesions, while dermatomal distribution of lesions is present in patients with segmental vitiligo. Segmental vitiligo can also follow Blaschko lines – pathways of epidermal cell migration and proliferation during the development of the fetus. Here, we present patient with segmental and non‐segmental vitiligo following Blaschko lines with excellent therapeutic response to combined therapy. Prior to our report, a case of segmental and non‐segmental vitiligo followed by Blaschko lines was never described, therefore we suggest the term “mixed vitiligo of Blaschko lines” to describe this entity. This is also a rare case in which 90% repigmentation was achieved in patient with segmental and nonsegmental vitiligo following Blaschko lines in only 2 months of combined therapy.     

Cutaneous Manifestations of Mixed Connective Tissue Disease: Study from a Tertiary Care Hospital in Eastern India

Context:

Mixed connective tissue disorder is an uncommon disease. Some scientists are reluctant to recognize it as a separate entity. Some others have defined this ailment. Cutaneous features of this condition are unique. Researchers from India have described these features to relate to those described in the studies from other parts of the globe.

Aims:

This study aims to delineate the skin manifestations of clearly defined mixed connective tissue disease (MCTD) patients, to compare them with those established as overlap syndrome, and to relate them with studies from other parts of the globe.

Settings and Design:

Successive patients who fulfilled the specific criteria for MCTD presenting in the skin outpatient department of a tertiary care hospital in eastern India were clinically examined from 2009 for 3 years.

Materials and Methods:

The number of participants was 23 and the dermatological features of these were compared with 22 patients with overlap syndrome. The antibody to uridine-rich U1 ribonucleoprotein was measured for all patients.

Statistical Analysis Used:

SPSS (Version 17) and MedCalc (Version 11.6).

Results:

The Male: Female ratio among the MCTD patients was 1:6.67 and that of the overlap syndrome was 1:10. Twenty patients of the MCTD group presented with synovitis as against only seven in the overlap group. Raynaud''s phenomenon was present in some of the subjects. Puffy fingers were rare in our study. Facial numbness was reported by four of those suffering from MCTD. Antinuclear antibody (ANA) was essentially of a speckled pattern in this disease

Conclusions:

Cutaneous indicators of MCTD are distinct from overlap syndrome. Knowledge of these manifestations prevalent in a region may lead to early diagnosis of the disease.     

Autologous Graft‐Versus‐Host Disease in a Child with Stage IV Neuroblastoma

Graft‐versus‐host disease (GVHD) is an underappreciated complication of autologous hematopoietic stem cell transplantation (AHSCT) that can affect the skin, gastrointestinal tract, and liver. The development of this rare condition is probably due to an impairment of immunologic tolerance that can occur spontaneously through T‐cell dysregulation, possibly from intensive conditioning chemotherapy regimens, or intentionally through administration of cyclosporine in the hopes of promoting an antitumor response. We present the case of a 2‐year‐old boy with metastatic neuroblastoma who spontaneously developed autologous GVHD after AHSCT. Severe pruritus and an inability to taper his oral steroids without a disease flare marked his disease. Eventually partial relief was achieved with initiation of cyclosporine and a strict soak and smear protocol using topical triamcinolone 0.1% ointment.