1.Cutis marmorata telangiectatica congenita
Malaysian Journal of Dermatology 2012;28(-):27-29
Cutis marmorata telangiectatica congenita
(CMTC) is a rare congenital disorder with
persistent cutis marmorata, telengiectasia, and
phlebectesia, which may be associated with
cutaneaus atrophy and ulceration of the involved
skin. We herewith report a three month old male
baby with CMTC at birth involving left side of
the face, upper limbs, both flanks, and left gluteal
and left leg with ulceration over the extensor
aspects of the left knee joint. The baby had a
reticulated bluish purple skin changes all over the
body including the face and limb. Although it
resembled physiological cutis marmorata, it was
strikingly pronounced and defi n i t e ly was
unvarying and permanent. A variety of vascular
malformation has been described along with this
disorder. Etiology is not very clear. Prognoses in
uncomplicated cases are good.
2.Neonatal lupus erythematosus presenting as multiple photosensitive annular plaques with skin atrophy
Malaysian Journal of Dermatology 2012;28(-):30-33
Neonatal lupus erythematosus (NLE) is an
autoimmune disease affecting the fetus as a
result of transplacental transfer of anti-Ro
autoantibodies. Typically, it presents in the first
few months of life with an annular form of
subacute cutaneous lupus erythematosus. We
report an infant of NLE presenting at birth with
multiple annular erythematous plaques with skin
atrophy involving the face, head, and upper trunk.
Histopathology of skin biopsy was consistent
with subacute cutaneous lupus. The mother was
clinically free of disease and had no family
history of autoimmune disease. Serology (extranuclear
antigens) was positive in both the baby
and the mother. This is a rare presentation of a
rare disease.
3.Detection of BCR-ABL T315i Mutation in Imatinib Resistant Chronic Myeloid Leukemia Patients
Medicine and Health 2019;14(1):145-156
Chronic myeloid leukemia (CML) patients who have BCR-ABL T315I mutation,
usually present in the advance phase of the disease with overall survival (OS) shorter
than those without the mutation. This study aimed to determine the prevalence
of T315I mutation amongst imatinib mesylate (IM) resistant CML patients and to
compare the OS between T315I-mutated and non-T315I-mutated patients. Sixty
consecutive CML patients who were treated with IM for at least 18 months and
their treatment responses, were recorded. The mutation analysis was done using
allele-specific oligonucleotide reverse transcriptase-polymerase chain reaction
(RT-PCR) assay followed by direct sequencing technique. Forty-two patients (70%)
were found to have IM-resistance. Five out of 42 patients had detectable T315I
mutation. Median OS of IM-resistant T315I-mutated patients was 96 months
(95% CI:54-138) compared to 84 months (95% CI:48-120) in non T315I-mutated
patients, although this was found to be statistically insignificant (p = 0.43). The
present study showed a higher prevalence of T315I mutation as compared to a few
local studies. Median OS of T315I-mutated patients were observed to be longer
than non-T315-mutated patients. Further studies encompassing larger cohort of
patients are required to confirm this finding
4.Treatment of Infantile Haemangiomas with 585nm pulsed dye laser
Sabeera BKI ; Mardziah A ; Gangaram HB
Malaysian Journal of Dermatology 2010;25(-):15-20
Haemangiomas usually develop within the first few weeks of life, most regressing spontaneously
before the age of 7 to 10 years. Some may ulcerate or compromise a vital function, in which case
systemic corticosteroids, surgery or radiotherapy may be helpful. All of these treatment modalities
are associated with significant morbidity. Treatment with 585nm flashlamp pulsed dye laser is safe
and effective in the management of ulcerated and superficial proliferating haemangiomas. We report
a retrospective review of 33 children under the age 12 months, who were treated at our centre with
585nm pulsed dye laser over a period of 4 years. Forty eight percent of these children presented with
rapidly proliferating haemangiomas causing functional impairment, 40% with ulcerated
haemangiomas and others for re-growth after stopping oral treatment. Patients were treated with the
585nm pulsed dye laser (fluence: 5.5-7J/cm2; spot size: 7mm and duration: 0.45s). Patients received
treatment until the lesion was almost clear or until lesion failed to respond. All lesions ulcerated
haemangiomas healed after an average 3 treatment. Both the physician and parental perception of
improvement were analysed based on three parameters, which include reduction in redness,
thickness and size. All the haemangiomas showed significant reduction in size, thickness and colour.
Less than 1% of patients had atrophic scaring. We conclude that the flashlamp-pulsed dye laser may
successfully prevent enlargement and promote involution of superficial haemangiomas with minimal
adverse effect. Therapy is most appropriate for patients with ulcerated haemangiomas and
haemangiomas at sites of potential functional impairment.
5.Childhood atopic dermatitis: a measurement of quality of life and family impact.
M S Noor Aziah ; T Rosnah ; A Mardziah ; M Z Norzila
The Medical journal of Malaysia 2002;57(3):329-39
Atopic dermatitis is the commonest skin disease in children, causing psychological, social and functional disability to them and their families. This study assessed the family impact and quality of life using the translated Malay version of The Dermatology Family Impact (DFI) and The Children's Dermatology Life Quality Index (CDLQI) questionnaires. Seventy-two children, aged between 6 months and 16 years attending the Paediatric Dermatology Clinic at the Paediatric Institute and the Dermatology Department, Hospital Kuala Lumpur participated in this study. Thirty-nine patients (54.2%) were males and 33 patients (45.8%) were females. The median age of the patients was 74 months (Q1 6, Q3 104 months). The median age of diagnosis was 22 months (Q1 1, Q3 36 months). The median disease duration was 44 months (Q1 3, Q3 65). The severity of eczema was assessed using the SCORAD severity index (maximum score = 83). The median SCORAD (European Task Force On Atopic Dermatitis) score was 36 (n = 72, SD = 16.2). The majority of patients in this study suffered from moderately severe eczema (n = 40, mean, SCORAD = 29.3) followed by severe eczema (n = 27, mean SCORAD = 54.3). The mildly affected patients formed the minority group (n = 5, mean SCORAD = 9.0). The family impact was shown to be greater in severe atopic dermatitis compared to moderate atopic dermatitis (Anova, p = 0.02). The children's quality of life impairment was also greater in severe atopic dermatitis compared to moderate atopic dermatitis (Anova p = 0.08). This study confirms that quality of life and family impact are related to the severity of atopic dermatitis.
Dermatitis, Atopic
;
month
;
Quality of life
;
Family
;
Median Statistical Measurement
6.Synthesis and characterization of strontium-doped hydroxyapatite powder via sol-gel method.
Mardziah CM ; Sopyan I ; Hamdi M ; Ramesh S
The Medical Journal of Malaysia 2008;63 Suppl A():79-80
Improvement of the mechanical properties of hydroxyapatite (HA) can be achieved by the incorporation of metal. In addition, incorporation of strontium ion into HA crystal structures has been proved effective to enhance biochemical properties of bone implant. In this research, strontium-doped HA powder was developed via a sol-gel method to produce extraordinarily fine strontium-doped HA (Sr-doped HA) powder. XRD measurement had shown that the powder contained hydroxyapatite phase only for all doping concentration except for 2%, showing that Sr atoms have suppressed the appearance of beta-TCP as the secondary phase. Morphological evaluation by FESEM measurement shows that the particles of the Sr-doped HA agglomerates are globular in shape with an average size of 1-2 microm in diameter while the primary particles have a diameter of 30-150 nm in average.
7.Efficacy and safety of tacrolimus ointment in patients with moderate to severe atopic dermatitis - Malaysian experience
Ng TG ; Mardziah A ; Roshidah BB ; Heng YH ; Najeeb A ; Lo Kang SC ; Pubalan M ; Loh LC ; Suraiya HH
Malaysian Journal of Dermatology 2009;22(-):47-54
Objectives To evaluate the efficacy and safety of tacrolimus ointment 0.1% in adult and 0.03% in
pediatric patients with moderate to severe atopic dermatitis in Malaysia.
Methods This is an open-labeled and single arm multi-center study. 36 adult and 37 pediatric
patients were enrolled. Tacrolimus ointment is applied twice daily for four weeks. The primary
efficacy outcome is based on the Physician’s Global Evaluation of Clinical Response (PG) at Week
4. The secondary efficacy outcomes are Eczema Area and Severity Index (EASI) score, changes
from baseline in individual scores of signs and symptoms and body surface area affected and
Patients Assessment of Treatment Effects.
Results Overall success rate were 97.1% and 91.2% in the adult and pediatric groups respectively.
The decline in EASI, percentage of total BSA affected and patient’s assessment of pruritus were
significant (P<0.001). Of adults and pediatric patients, 97.2% and 75.7% respectively reported
adverse effect. The most common adverse effect reported was skin burning sensation in 91.7% adult
patients and pruritus in 67.6% pediatric patients.
Conclusion Tacrolimus ointment 0.1% in adult and 0.03% in pediatric patients is effective for the
treatment of moderate to severe atopic dermatitis in Malaysia.
8.A study on the knowledge, attitudes and practices of Southeast Asian dermatologists in the management of atopic dermatitis.
Yuin-Chew CHAN ; Yong-Kwang TAY ; Titi-Lestari SUGITO ; Siti Aisah BOEDIARDJA ; Duong-Dinh CHAU ; Khac-Vien NGUYEN ; Kim-Chye YEE ; Mardziah ALIAS ; Suraiya HUSSEIN ; Ma Victoria DIZON ; Francisca ROA ; Yiong-Huak CHAN ; Siriwan WANANUKUL ; Preya KULLAVANIJAYA ; Srisupalak SINGALAVANIJA ; Wai-Kwong CHEONG
Annals of the Academy of Medicine, Singapore 2006;35(11):794-803
INTRODUCTIONThis study evaluated the knowledge, attitudes and practices of Southeast Asian dermatologists in the management of atopic dermatitis (AD).
MATERIALS AND METHODSA questionnaire survey of 255 dermatologists in Indonesia, Malaysia, the Philippines, Singapore, Thailand and Vietnam.
RESULTSFamiliarity with diagnostic criteria varied considerably. The usage of moisturisers by the respondents from Vietnam and Indonesia was significantly less frequent than the other countries. Most respondents (91% to 100%) used topical corticosteroids in children with mild-to-moderately severe dermatitis. Some respondents in the Philippines (17% to 19%) and Vietnam (11% to 25%) only used topical corticosteroids for severe disease. For infected eczema, most respondents would prescribe systemic antibiotics for mild-to-moderate infection. A minority in the Philippines (14%) and Vietnam (11%) did so only for severe infection. The top 4 systemic antibiotics prescribed most frequently were: erythromycin, cloxacillin, cephalosporin and amoxicillin/clavulanic acid. In Indonesia, a large proportion of the respondents (47%) prescribed amoxicillin most frequently. The majority of respondents (60% to 100%) prescribed both sedating and non-sedating oral antihistamines. Most respondents used oral corticosteroids to treat severe AD. Some in Malaysia, Singapore and Vietnam used cyclosporin (7% to 58%), azathioprine (5% to 31%) and methotrexate (5% to 14%). With the exception of those in Singapore, the majority of respondents (71% to 97%) did not use phototherapy.
CONCLUSIONFamiliarity with diagnostic criteria, the early and judicious use of moisturisers and topical corticosteroids, as well as the treatment of Staphylococcus aureus superinfection with penicillinase-stable antibiotics should be emphasised in this region.
Anti-Bacterial Agents ; therapeutic use ; Asia, Southeastern ; epidemiology ; Dermatitis, Atopic ; diagnosis ; drug therapy ; epidemiology ; Drug Administration Routes ; Glucocorticoids ; administration & dosage ; therapeutic use ; Health Knowledge, Attitudes, Practice ; Histamine H1 Antagonists ; therapeutic use ; Humans ; Hypnotics and Sedatives ; therapeutic use ; Outcome Assessment (Health Care) ; Population Surveillance ; Severity of Illness Index