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Biomedical subjects

Y K Tay

Publications and source records attributed to Y K Tay.

At least 73 records · Page 4Linked to original sources

Cutaneous mastocytosis in Singapore.

Mastocytosis is the collective name for a group of clinical syndromes whose signs and symptoms are due to the infiltration of various tissues by mast cells and to the release of chemical mediators by these cells. The skin is the most frequently affected organ. Skin manifestations include urticaria pigmentosa, mastocytoma, diffuse cutaneous mastocytosis and telangiectasia macularis eruptiva perstans. Seven cases of mastocytosis were seen over a 3-year period at the National Skin Centre from 1989 to 1992. All our patients were in the paediatric age group. There were four boys and three girls ranging in age from one year to five years. The mean age of onset of the disease was 2.3 months. Six patients presented with cutaneous signs and symptoms of urticaria pigmentosa and one patient had diffuse cutaneous mastocytosis. Itch was the most prominent symptom seen in all the patients. All the patients had a positive Darier's sign, pathognomonic for mastocytosis. None of the patients had a positive family history. Treatment was conservative and symptomatic, with the use of H1 antihistamines to control itching. A particularly important aspect of management is the avoidance of triggering factors. All our patients have remained well with only skin involvement. The prognosis for children with mast cell disease is good, with at least half of the children with urticaria pigmentosa experiencing reduction of symptoms and lesions by adolescence.

Biopsy↗

Anhidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome)--a case report and review.

We report two brothers with anhidrotic ectodermal dysplasia (AED). AED is characterised by a triad of inability to sweat dental abnormalities and hypotrichosis. Although rare, it is important to diagnose this condition as it may lead to considerable morbidity and mortality if unrecognised. The typical features of this entity as described in the literature and as seen in our patients are emphasized as increased awareness can lead to early diagnosis and better prognosis.

Ectodermal Dysplasia↗

Acute pyoderma gangrenosum does not require surgical therapy.

Pyoderma gangrenosum is an uncommon ulcerative skin disorder that is often associated with underlying systemic diseases, the most common of which is inflammatory bowel disease. We report an illustrative case of a 36-year-old woman with pyoderma gangrenosum occurring at surgical sites and at sites of trauma. She had associated Crohn disease. Multiple surgical procedures were performed on this patient, without improvement. Pyoderma gangrenosum can mimic a necrotizing soft tissue infection. Early recognition of the characteristic lesion may prevent unnecessary operations and facilitate effective control with appropriate medical therapy.

Acute Disease↗

Treatment of linear IgA bullous dermatosis of childhood with colchicine.

Linear IgA bullous dermatosis of childhood is the most common immunobullous disorder of childhood. First-line treatment includes dapsone or sulfapyridine. We report a child with linear IgA dermatosis who was treated successfully with colchicine after developing hemolytic anemia from dapsone. We recommend colchicine as an alternative therapy for linear IgA dermatosis of childhood in patients in whom first-line treatment is contraindicated or who fail first-line treatment or develop side effects from it.

Anemia, Hemolytic↗

Profile and outcome of childhood mycosis fungoides in Singapore.

Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma. It usually occurs in middle-aged and elderly persons, although several reports have described its occurrence in young children. The aim of this study was to review the profile and outcome of childhood MF in Singapore from 1989 to 1998. A total of nine patients (six males and three females) were diagnosed with MF before the age of 21 years. There were four Chinese, four Malay, and one Indian. The age at the time of histologic diagnosis ranged from 6 to 20 years (mean 14.3 years). Eight of the nine patients presented with hypopigmented patches and plaques. According to TNM staging, three were in stage 1A and six in stage 1B. The treatment modalities included psoralen plus ultraviolet A (PUVA) (n = 5), UVB (n = 2), and potent topical steroids (n = 2). We found that PUVA induced a faster clinical remission, but maintenance PUVA was required to prolong the relapse-free interval. This study also highlighted the need to consider MF in the differential diagnosis of hypopigmented dermatoses in dark-skinned individuals, especially if they occur on the buttocks.

Administration, Topical↗

Experience with UVB phototherapy in children.

Twenty children age 14 months to 12 years with photoresponsive dermatoses were treated with ultraviolet B (UVB) phototherapy over four years. Ten children had psoriasis, five had pityriasis lichenoids, and five had atopic dermatitis. All received short courses (average 34 treatments) of phototherapy with either no maintenance or short maintenance. Treatment was effective and well tolerated in most patients, and no serious side effects were seen. Patients with psoriasis and pityriasis lichenoides cleared completely. No patient with atopic dermatitis cleared completely, but all were moderately improved, with reduction of the extent of eczema and decreased pruritus. It appears that UVB phototherapy is a valuable and safe therapeutic option for selected children who do not respond to other treatments.

Child↗

Treatment of childhood cutaneous T-cell lymphoma with alpha-interferon plus PUVA.

All forms of cutaneous T-cell lymphoma are rare in childhood. We describe an 8-year-old boy with plaque-stage mycosis fungoides stage IIA whose cutaneous eruption had been present for 5 years. Histologic examination revealed the presence of a granulomatous infiltrate together with atypical lymphocytes within the dermis. The child had an excellent response to combination psoralen-UVA (PUVA) with interferon-alpha 2a treatment and is currently in remission.

Antineoplastic Agents↗

Epidermolysis bullosa simplex herpetiformis of Dowling-Meara with mottled pigmentation: the relationship between EBS herpetiformis and EBS with mottled pigmentation.

A 6-year-old girl with epidermolysis bullosa simplex (EBS) is described. Clinical features include generalized herpetiform blistering of the skin, healing without scars, intraoral blistering, nail dystrophy, palmoplantar keratoderma, and improvement with age. An unusual feature was the presence of a striking mottled pigmentation involving the arms, trunk, and legs. Histology, immunofluorescence, and electron microscopy of a fresh lesion showed an intraepidermal split within the basal keratinocytes, focal hyperpigmentation of the basal cells without an inflammatory infiltrate, and tonofilament clumping. Our patient illustrates the clinical features of Dowling-Meara EBS with the pigmentary changes of EBS with mottled pigmentation. The relationship between EBS of Dowling-Meara and EBS with mottled pigmentation is discussed.

Aging↗