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1.
J Eur Acad Dermatol Venereol ; 32(9): 1499-1506, 2018 Sep.
Article in English | MEDLINE | ID: mdl-29430733

ABSTRACT

BACKGROUND: Psychological aspect and quality of life should be considered in treating patients with psoriasis. OBJECTIVE: We sought to ascertain which clinical characteristics including presence of exposed lesions are associated with impairment of health-related quality of life (HRQoL) in patients with psoriasis. METHODS: The EPI-PSODE study was a nationwide, multicenter, cross-sectional study conducted in Korea that included 1260 adult patients with psoriasis. In addition to clinical characteristics including presence of exposed lesions, data were collected using the Psoriatic Arthritis (PsA) Screening and Evaluation (PASE), Dermatology Life Quality Index (DLQI), MOS 36-Item Short-Form Health Survey (SF-36), Work Productivity and Activity Impairment Questionnaire Psoriasis (WPAI: PSO) and Medication Satisfaction Questionnaire (MSQ). RESULTS: Patients with a DLQI score > 5 (n = 990) were younger, had an earlier onset of psoriasis, scored higher on the Psoriasis Area and Severity Index (PASI), had higher body surface area (BSA) and had higher PASE scores than patients with DLQI ≤ 5 (n = 266). The group of patients with exposed lesions (n = 871) were younger and male predominance, earlier onset of psoriasis, longer disease duration, higher PASI/BSA score and a higher proportion with drinking and smoking history each than the group of patients without exposed lesions (n = 389). Presence of exposed lesions negatively influenced DLQI, 36-Item Short-Form Health Survey (SF-36) (mental component), presenteeism, total work productivity impairment and total activity impairment in the WPAI: PSO. In multiple regression model, PASI score was the only variable which was significantly associated with all HRQoL measures. Presence of exposed lesions was a significant factor affecting DLQI and SF-36 (mental). CONCLUSION: The presence of exposed lesions has a negative impact on quality of life, mental health and work productivity. Therefore, effective treatments are particularly needed for psoriasis patients with exposed lesions.


Subject(s)
Psoriasis/psychology , Quality of Life , Adult , Age of Onset , Alcohol Drinking/epidemiology , Arthritis, Psoriatic/diagnosis , Body Surface Area , Cross-Sectional Studies , Efficiency , Female , Humans , Male , Middle Aged , Presenteeism , Psoriasis/epidemiology , Republic of Korea/epidemiology , Severity of Illness Index , Sex Factors , Smoking/epidemiology , Surveys and Questionnaires
2.
Br J Dermatol ; 179(4): 836-843, 2018 10.
Article in English | MEDLINE | ID: mdl-29355904

ABSTRACT

BACKGROUND: Ingenol mebutate gel is a novel, field-directed topical treatment for actinic keratosis (AK). Most pivotal studies have targeted Western populations. No clinical study has been conducted to investigate its efficacy and safety in Asian populations. OBJECTIVES: To evaluate the efficacy and safety of ingenol mebutate gel for treating AK of face/scalp and trunk/extremities in a large Asian (Korean) population. PATIENTS AND METHODS: In this multicentre, open-label, interventional, parallel-group, prospective phase IV study (PERFECT, trial registration no.: NCT02716714), the eligible patients were allocated into either the face/scalp or the trunk/extremities group, according to their selected treatment area location. After application of ingenol mebutate gel, the participants were followed up for 6 months. The primary efficacy endpoint was complete clearance (CC) of AK lesions in the selected treatment area at day 57. Quality of life was evaluated using Skindex-29. Safety endpoints included local skin responses, scar, pigmentation, pain and adverse events. RESULTS: In total, 78·1% [95% confidence interval (CI) 66·86-86·92%] of subjects had CC at day 57, with 76·6% (95% CI 64·31-86·25%) in the face/scalp group and 88·9% (95% CI 51·75-99·72%) in the trunk/extremities group. Among them, CC was sustained in 88·9% (48 of 54, 95% CI 77·37-95·81%) at month 6. The local skin responses significantly increased 1 day after the treatment compared with baseline, and decreased afterwards. Among the total subjects, 7·8% (6 of 77) had hyperpigmentation on the application area. Scars were not reported. CONCLUSIONS: Ingenol mebutate is effective for the treatment of AK in Asians, with tolerable safety profiles.


Subject(s)
Diterpenes/administration & dosage , Facial Dermatoses/drug therapy , Keratosis, Actinic/drug therapy , Scalp Dermatoses/drug therapy , Administration, Topical , Adult , Aged , Aged, 80 and over , Diterpenes/adverse effects , Extremities , Facial Dermatoses/psychology , Female , Follow-Up Studies , Humans , Hyperpigmentation/chemically induced , Hyperpigmentation/epidemiology , Keratosis, Actinic/psychology , Male , Middle Aged , Prospective Studies , Quality of Life , Republic of Korea , Scalp Dermatoses/psychology , Torso , Treatment Outcome
3.
Opt Express ; 23(21): 26952-61, 2015 Oct 19.
Article in English | MEDLINE | ID: mdl-26480356

ABSTRACT

We demonstrate all-optical 4x10 Gbps NAND gate with an individual input data rate of 10 Gbps using single mode Fabry-Pérot laser diode. The proposed scheme is based on the principle of multi-input injection locking. All-optical NAND gate is one of the universal logic gates which can be used for realizing all other logic gates for optical communication and networks. The output performance of the proposed all-optical multi-input NAND gate is verified with output spectrum domain results, waveforms, rising-falling time, and eye diagram measurement at 10 Gbps input. The ON/OFF contrast ratio of 41 dB is measured at the spectrum domain output when all four input beams are logic high. In all other combinations of four inputs, maximum ON/OFF contrast ratio of 1.5 dB ON/OFF is measured. Clear output waveform, output eye diagram with an extinction ratio of about 11 dB, and rising-falling time of about 35 ps are obtained. The BER measurement is carried out and we found the power penalty of about 1.7 dB at BER of 10(-9).

4.
Dermatology ; 226(3): 212-6, 2013.
Article in English | MEDLINE | ID: mdl-23751255

ABSTRACT

The cutaneous manifestations of chronic active Epstein-Barr virus (EBV) infection can be diverse. Among them, hydroa vacciniforme-like eruption is one of the best-known features. Although rare, mucosal ulcers have been reported to be associated with EBV as a result of primary infection or immune suppression. We describe a 65-year-old female with recurrent necrotic papulovesicles on the face and both arms for 2 years. She also complained of recurrent oral and genital mucosal ulcers developing simultaneously with skin eruptions. They appeared periodically during the spring and summer and were triggered or aggravated by sun exposure. Skin biopsies from the face and genitalia showed identical findings with dense lymphocytic infiltrations. In addition, in situ hybridization revealed EBV-positive lymphoid cells in both specimens. To our knowledge, this is the first case of serologically and pathologically proven chronic active EBV infection presenting hydroa vacciniforme-like eruption and orogenital ulcers at the same time in one patient.


Subject(s)
Epstein-Barr Virus Infections/diagnosis , Herpesvirus 4, Human/isolation & purification , Oral Ulcer/virology , Skin Diseases, Vesiculobullous/virology , Skin Diseases, Viral/virology , Aged , Behcet Syndrome/diagnosis , Chronic Disease , Diagnosis, Differential , Epstein-Barr Virus Infections/complications , Epstein-Barr Virus Infections/drug therapy , Fatigue/etiology , Female , Humans , Skin Diseases, Viral/pathology , Vulvar Diseases/virology
5.
J Nanosci Nanotechnol ; 12(1): 227-35, 2012 Jan.
Article in English | MEDLINE | ID: mdl-22523970

ABSTRACT

A simple biotemplating method for the synthesis of silica (SiO2) and titania (TiO2) nanowires was designed on a fibrillar protein (alpha-synuclein) template. The diameter of SiO2 and TiO2 nanowires could be varied, between 20-100 nm, by varying the processing conditions. The nanowires were characterized by energy dispersive spectroscopy (EDS) and electron energy loss spectroscopy (EELS). Due to their high surface area and porosity, the nanowires were tested for potential applications in enzymatic biosensor design.


Subject(s)
Molecular Imprinting/methods , Nanostructures/chemistry , Nanostructures/ultrastructure , Silicon Dioxide/chemistry , Titanium/chemistry , alpha-Synuclein/chemistry , Macromolecular Substances/chemistry , Molecular Conformation , Particle Size , Surface Properties
6.
Clin Exp Dermatol ; 37(7): 744-8, 2012 Oct.
Article in English | MEDLINE | ID: mdl-22329727

ABSTRACT

Precise clinicopathological correlations of the clinical features of metastatic breast carcinoma with lymphatic-specific markers are rare. We classified 28 patients with metastatic breast carcinoma according to their clinical features. Immunohistochemical staining was performed using D2-40, CD31 and CD34. Of the 28 patients, 8 (28.6%) had inflammatory metastatic carcinoma, 6 (21.4%) had the telangiectatic type, 5 had the nodular type, 3 had the en cuirasse type, 3 had alopecia neoplastica, and 3 had a combination of features. D2-40 staining revealed dilated lymphatic channels (lymphangiectasia) in the upper dermis of all patients; in addition, 13 patients (46.4%) had intralymphatic tumour-cell emboli, which were common in those with the inflammatory and telangiectatic types. Intratumoral lymphatic invasion in the main tumour nodule was seen in 12 patients (42.9%). Our results suggest that cutaneous metastatic breast carcinomas have various clinical presentations, and that lymphatic vessels play an important role in all types of cutaneous metastasis.


Subject(s)
Breast Neoplasms/pathology , Carcinoma, Ductal, Breast/secondary , Lymphatic Vessels/pathology , Skin Neoplasms/secondary , Adult , Aged , Biomarkers, Tumor/metabolism , Carcinoma, Ductal, Breast/metabolism , Carcinoma, Ductal, Breast/pathology , Female , Humans , Immunohistochemistry , Lymphatic Metastasis , Middle Aged , Skin Neoplasms/metabolism
7.
Br J Dermatol ; 166(1): 115-23, 2012 Jan.
Article in English | MEDLINE | ID: mdl-21923752

ABSTRACT

BACKGROUND: Giant congenital melanocytic naevi (GCMN) are known risk factors for the development of melanoma. However, melanoma risk among Asians is rarely evaluated. OBJECTIVES: To evaluate the clinical characteristics and risk of melanoma development from GCMN in Koreans, we performed a nationwide retrospective cohort study in Korea. GCMN were defined as those comprising ≥5% body surface area in children or measuring ≥20cm in adults. METHODS: In total, 131 patients with GCMN were enrolled, with a mean age of 10·3years (range: birth-70years). RESULTS: The posterior trunk was the most common site (67, 51·1%), followed by lateral trunk, anterior trunk, legs, both anterior and posterior trunk, buttocks, and arms. Satellite naevi were present in 69 cases (52·7%), and axial areas were more commonly involved in patients with satellite naevi than in those without satellite lesions. Atypical features such as rete ridge elongation and bridges were seen, and, among these, pagetoid spread and ballooning cell changes were more common in patients <4years old. Proliferative nodules were found in three cases. Melanomas had developed in three of 131 patients (2·3%; a 6-year-old girl, a 14-year-old girl and a 70-year-old man), and the incidence rate was 990 per 100000 person-years. Melanomas in these three patients consisted of two cutaneous melanomas and one extracutaneous meningeal melanoma. CONCLUSIONS: We should be aware of melanoma development from GCMN, and lifelong follow-up is required due to the risk of melanoma arising in GCMN.


Subject(s)
Melanoma/epidemiology , Nevus, Pigmented/congenital , Skin Neoplasms/congenital , Skin/pathology , Adolescent , Adult , Age Distribution , Aged , Biopsy, Needle , Child , Child, Preschool , Female , Humans , Infant , Male , Melanoma/pathology , Middle Aged , Nevus, Pigmented/epidemiology , Nevus, Pigmented/pathology , Republic of Korea/epidemiology , Retrospective Studies , Risk Factors , Skin Neoplasms/epidemiology , Skin Neoplasms/pathology , Young Adult
8.
Br J Radiol ; 84(999): 210-5, 2011 Mar.
Article in English | MEDLINE | ID: mdl-21325363

ABSTRACT

OBJECTIVE: Neointimal hyperplasia is a major complication of endovascular stent placement with consequent in-stent restenosis or occlusion. Improvements in the biocompatibility of stent designs could reduce stent-associated thrombosis and in-stent restenosis. We hypothesised that the use of a diamond-like carbon (DLC)-coated nitinol stent or a polyethylene glycol (PEG)-DLC-coated nitinol stent could reduce the formation of neointimal hyperplasia, thereby improving stent patency with improved biocompatibility. METHODS: A total of 24 stents were implanted, under general anaesthesia, into the iliac arteries of six dogs (four stents in each dog) using the carotid artery approach. The experimental study dogs were divided into three groups: the uncoated nitinol stent group (n = 8), the DLC-nitinol stent group (n = 8) and the PEG-DLC-nitinol stent group (n = 8). RESULTS: The mean percentage of neointimal hyperplasia was significantly less in the DLC-nitinol stent group (26.7±7.6%) than in the nitinol stent group (40.0±20.3%) (p = 0.021). However, the mean percentage of neointimal hyperplasia was significantly greater in the PEG-DLC-nitinol stent group (58.7±24.7%) than in the nitinol stent group (40.0±20.3%) (p = 0.01). CONCLUSION: Our findings indicate that DLC-coated nitinol stents might induce less neointimal hyperplasia than conventional nitinol stents following implantation in a canine iliac artery model; however, the DLC-coated nitinol stent surface when reformed with PEG induces more neointimal hyperplasia than either a conventional or DLC-coated nitinol stent.


Subject(s)
Coated Materials, Biocompatible/therapeutic use , Iliac Artery/pathology , Neointima/prevention & control , Stents , Alloys/therapeutic use , Animals , Blood Vessel Prosthesis Implantation/instrumentation , Blood Vessel Prosthesis Implantation/methods , Dogs , Hyperplasia/prevention & control , Neointima/pathology , Polyethylene Glycols/therapeutic use , Stents/adverse effects
9.
Lupus ; 18(14): 1320-6, 2009 Dec.
Article in English | MEDLINE | ID: mdl-19884217

ABSTRACT

Dermatological examination is critical for the evaluation of lupus erythematosus. However, little is known about the epidemiology and clinical characteristics of the lupus erythematosus patients that visit dermatology clinics with the chief complaint of skin lesions, especially among Asian populations. We performed this study to determine the epidemiology of cutaneous lupus erythematosus for three subtypes: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus, and for lupus erythematosus non-specific skin disease. Also, we sought to determine the relationship between each type of lupus erythematosus, by the skin manifestations and systemic lupus erythematosus. The medical records of lupus erythematosus patients that were diagnosed by their clinical manifestations, skin biopsy results, and laboratory findings from January 1998 through December 2007 were reviewed. A total of 117 patients were diagnosed with lupus erythematosus; 62 cases had chronic cutaneous lupus erythematosus, 11 had subacute cutaneous lupus erythematosus, and 41 had acute cutaneous lupus erythematosus. The remaining three had systemic lupus erythematosus features with lupus erythematosus non-specific skin lesions such as Raynaud phenomenon, livedo reticularis/vasculitis, non-scarring alopecia, and periungual telangiectasia. The acute cutaneous lupus erythematosus subgroup showed extreme female predominance (9.2:1) whereas subacute and chronic cutaneous lupus erythematosus subgroups did not. Patients with chronic cutaneous lupus erythematosus tended to be older than other groups (peak incidence in the fifth decade). Incidence of laboratory abnormalities, including positive connective tissue markers such as antinuclear, double-strand DNA, and Ro/SS-A antibodies, were present in the order acute, subacute, and chronic cutaneous lupus erythematosus. Acute cutaneous lupus erythematosus almost always indicated systemic involvement of lupus erythematosus, whereas chronic cutaneous lupus erythematosus did not predict the development or existence of systemic lupus erythematosus and had a benign clinical course. Careful consideration of lupus erythematosus non-specific skin lesions may help detect systemic lupus erythematosus regardless of the diagnosis of cutaneous lupus erythematosus.


Subject(s)
Asian People/statistics & numerical data , Lupus Erythematosus, Discoid/epidemiology , Lupus Erythematosus, Discoid/pathology , Referral and Consultation/statistics & numerical data , Acute Disease , Adult , Aged , Child , Chronic Disease , Dermatology/statistics & numerical data , Female , Humans , Infant , Male , Middle Aged , Outpatient Clinics, Hospital/statistics & numerical data , Republic of Korea/epidemiology , Retrospective Studies , Skin/pathology
10.
Clin Exp Dermatol ; 34(8): e798-801, 2009 Dec.
Article in English | MEDLINE | ID: mdl-19778310

ABSTRACT

Dapsone has potent anti-inflammatory effects, and is used in the treatment of leprosy, cutaneous vasculitis, neutrophilic dermatoses, and dermatitis herpetiformis and other blistering disorders. However, it may cause severe adverse reactions such as hypersensitivity syndrome, which is characterized by fever, skin rash, hepatitis and lymphadenopathy. We report a 44-year-old female Korean patient with dapsone hypersensitivity syndrome (DHS) that presented as a bullous skin eruption. The patient had a 1-year history of urticarial vasculitis, treated with antihistamines, prednisolone and dapsone. Although the skin lesions improved, she reported fever, nausea, abdominal pain, jaundice, fatigue and skin rashes. On physical examination, there were generalized erythematous macules and purpura with facial oedema that developed into vesicles on the upper limbs. Histological examination of a skin biopsy of a vesicular lesion found subepidermal oedema with a mixed inflammatory cell infiltrate, including eosinophils in the dermis. Indirect immunofluorescence testing using normal foreskin as substrate revealed IgG deposits in the basement membrane zone. Circulating autoantibodies against antigens of 190 and 230 kDa were found by immunoblotting analysis using epidermal extracts. This case illustrates DHS with the formation of circulating autoantibodies.


Subject(s)
Dapsone/adverse effects , Dermatologic Agents/adverse effects , Drug Eruptions/immunology , Hypersensitivity/immunology , Vasculitis/drug therapy , Adult , Autoantibodies/immunology , Female , Humans , Syndrome
11.
Int J Cosmet Sci ; 30(5): 333-7, 2008 Oct.
Article in English | MEDLINE | ID: mdl-18822038

ABSTRACT

Sensitive skin is characterized by subjective symptoms that are hard to quantify. However, a neurobiological approach could improve our understanding of the nature of skin sensitivity. In this study, we measured the sensory perception of well-controlled electric currents on the skin that stimulated sensory nerve fibres such as the myelinated A fibre, A delta fibre and unmyelinated c-fibre. The sensory perception thresholds were obtained quantitatively from subjects with sensitive-prone skin and controls. Application of 0.075% capsaicin, known to stimulate the nociceptor c-fibre, was topically applied; then the sensory perception thresholds were measured to determine whether the exposure to nociceptive stimulation could affect the subsequent sensory perception. The results showed that the perception thresholds of skin sensitive-prone subjects were low for the c-fibre measurements at 5 Hz electric current stimulation. Furthermore, a wide variation in sensory perception was noted in the skin sensitive-prone subjects after topical application of capsaicin. In conclusion, the abnormal sensory perception in individuals with sensitive skin appears to be related to neurological instability, where c-fibre nociception plays a role. Thus, quantitative sensory perception threshold measurement was found to be a useful method for the identification of skin sensitive-prone subjects.


Subject(s)
Sensory Thresholds , Skin Physiological Phenomena , Capsaicin/pharmacology , Electric Stimulation , Humans , Skin/drug effects
14.
Clin Exp Dermatol ; 31(4): 545-7, 2006 Jul.
Article in English | MEDLINE | ID: mdl-16716160

ABSTRACT

We report a rare case of cutaneous abscess by Trichosporon asahii in an immunocompetent adult. A 31-year-old Korean woman presented to our hospital with a cutaneous abscess. She had received an intralesional steroid injection 4 months earlier on the site of a hypertrophic scar. Direct sequencing of the intergenic spacer regions of the rRNA genes identified T. asahii. The decreased local immunity after the steroid injection might have triggered the infection by T. asahii. A cutaneous abscess formation by T. asahii in an immunocompetent patient is an unusual cutaneous finding that to our knowledge has not been reported previously. The local immune reaction of the skin is important for the prevention of Trichosporon infection.


Subject(s)
Abscess/microbiology , Dermatomycoses/microbiology , Injections, Intralesional/adverse effects , Trichosporon , Abscess/immunology , Adult , Dermatomycoses/immunology , Female , Humans
16.
J Dermatolog Treat ; 15(1): 30-4, 2004 Jan.
Article in English | MEDLINE | ID: mdl-14754647

ABSTRACT

BACKGROUND: Atopic dermatitis is a chronically relapsing, common inflammatory skin disease, which significantly affects quality of life negatively in many respects. Topical steroids are the mainstay of atopic dermatitis treatment but they carry the risk of local side effects. A topical formulation of tacrolimus, a macrolide calcineurin inhibitor, has recently been developed. OBJECTIVE: To evaluate the efficacy and safety of 0.03% tacrolimus ointment for the treatment of moderate to severe atopic dermatitis in Korea. METHODS: An open, non-comparative, multi-center study with 4 weeks' follow-up was performed. A total of 180 patients (aged 2-57 years old) were enrolled. Tacrolimus ointment (0.03%) was applied to all involved areas twice daily. Efficacy was evaluated by an investigator's global assessment, the eczema area and severity index score, and by the patient's assessment of pruritus and clinical response at baseline, and after weeks 1, 2 and 4. Dermatology life quality index (DLQI), children's DLQI (CDLQI) and toddler's DLQI were assessed at baseline and at week 4. The safety assessment included monitoring all adverse events and clinical laboratory values. RESULTS: All efficacy parameters were improved. The mean EASI (eczema area and severity index) score was 19.7 at baseline and reduced to 8.0 at the end of the study. Moderate improvement was observed by the investigator's global assessment after 4 weeks' treatment. A marked decrease of pruritus was observed, and mild or moderate improvement was observed by patients' global assessments after the treatment period. Significant benefits in terms of quality of life in adults and children with atopic dermatitis were obtained. The most common adverse events associated with tacrolimus treatment were transient skin burning sensation (45.3%) and pruritus (41.6%) at the site of application. CONCLUSION: 0.03% tacrolimus ointment should be considered effective and safe in both Korean children and adults with moderate to severe atopic dermatitis.


Subject(s)
Dermatitis, Atopic/drug therapy , Immunosuppressive Agents/therapeutic use , Tacrolimus/therapeutic use , Administration, Cutaneous , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/adverse effects , Male , Middle Aged , Ointments , Tacrolimus/administration & dosage , Tacrolimus/adverse effects
19.
J Korean Med Sci ; 16(5): 615-8, 2001 Oct.
Article in English | MEDLINE | ID: mdl-11641532

ABSTRACT

The prevalence of Behcet's disease is the highest in the East Asian and the Mediterranean countries. Behcet's disease is also distributed in the Asian countries, but the nationwide survey has not been performed in Korea yet. The Korean Study Group for Behcet's Disease, founded in 1999, conducted a multicenter, retrospective survey on epidemiologic and clinical features of the patients with Behcet's disease from 20 hospitals around the nation from 1997 to 1999. Of 3,497 patients, 1,527 were classified into complete or incomplete type of Behcet's disease according to the revised Shimizu's classification. The sex ratio was 1:1.75 with the female predominance. Geographical distribution showed the highest frequency in Seoul (38.5%). Clinically, 98.8% had oral ulcers, 83.2% had genital ulcers, 84.3% had skin lesions and 50.9% had ocular lesions. As for the minor clinical manifestations, articular symptoms were the most frequent. The pathergy test showed positive in 15.4% of patients and revealed a higher positive rate in males (20.2%) than in females (12.7%). In conclusion, we performed the first multicenter study on Behcet's disease in Korea and revealed the female predominance, higher frequency of ocular lesions, and lower positivity of pathergy test in the patients.


Subject(s)
Behcet Syndrome/epidemiology , Adolescent , Adult , Aged , Behcet Syndrome/complications , Child , Child, Preschool , Female , Humans , Infant , Korea/epidemiology , Male , Middle Aged , Prevalence , Retrospective Studies , Sex Distribution
20.
Br J Dermatol ; 144(1): 182-5, 2001 Jan.
Article in English | MEDLINE | ID: mdl-11167703

ABSTRACT

We report a 56-year-old Korean woman with porphyria cutanea tarda (PCT), showing multiple scarring bullae and hypertrichosis on sun-exposed areas of skin with postinflammatory hyperpigmentation. Sclerodermoid changes were also found on both hands, the face and neck. The patient had suffered from CREST syndrome, manifesting with Raynaud's phenomenon and sclerodactyly, for more than 15 years. Anticentromere antibody was positive. She had presented with splenomegaly 3 years before the development of PCT, and was diagnosed as having idiopathic myelofibrosis, based on bone marrow biopsy. In summary, she had had CREST syndrome for 15 years and later developed idiopathic myelofibrosis and PCT. This is the first reported case of PCT in association with idiopathic myelofibrosis and CREST syndrome.


Subject(s)
CREST Syndrome/complications , Porphyria Cutanea Tarda/etiology , Primary Myelofibrosis/complications , Female , Humans , Middle Aged , Porphyria Cutanea Tarda/pathology
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