Extramammary Paget's disease (EMPD) is a rare disorder and may be found in the vulva, scrotum, penile area, perianal region and the groin. Frequently, it is associated with an underlying regional neoplasm or internal malignancy. We report 2 cases of EMPD; one involving the scrotal area and the other the vulva. Both were elderly patients who presented to the dermatologists with chronic eczematous lesions in the perineum that did not respond to topical treatment. Skin biopsies confirmed extramammary Paget's disease. Investigations for internal malignancies were negative. However, one of the patients defaulted treatment before surgery. The other patient had two excision surgeries with skin grafting to try to achieve tumour free margins. A long term follow-up was planned for him to look for recurrences. These cases emphasise that EMPD can mimic exudative dermatitis and present as a chronic non-healing lesion in the perineum for many years. Clinicians should have a high index of suspicion to pick up the disease early by biopsy. Various immunohistochemical markers not only can help differentiate other histological diagnoses but also help predict the presence of underlying malignancies. Management of EMPD included thorough search for occult or underlying malignancy followed by complete excision surgery with intraoperative frozen sections. Even then, recurrences are high for this disease and long term follow-up is advocated.
Extramammary Paget's Disease ; Malignant Neoplasms ; seconds ; Review [Publication Type] ; Lesion, NOS

Introduction: Cutaneous adverse drug reactions (cADRs) are common. There are only few studies on the incidence of cADRs in Malaysia. Objective: To determine the incidence, clinical features and risk factors of cADRs among hospitalized patients. Methods:A prospective study was conducted among medical inpatients from July to December 2014. Results: A total of 43 cADRs were seen among 11 017 inpatients, yielding an incidence rate of 0.4%. cADR accounted for hospitalization in 26 patients. Previous history of cADR was present in 14 patients, with 50% exposed to the same drug taken previously. Potentially lifethreatening severe cutaneous adverse reactions (SCAR), namely drug reaction with eosinophilia and systemic symptoms (DRESS: 14 cases) and Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN: 6 cases) comprise almost 50% of cADRs. The commonest culprit drug group was antibiotics (37.2%), followed by anticonvulsants (18.6%). Cotrimoxazole, phenytoin and rifampicin were the main causative drugs for DRESS. Anticonvulsants were most frequently implicated in SJS/TEN (66.7%). Most cases had “probable” causality relationship with suspected drug (69.8%). The majority of cases were of moderate severity (65.1%), while 18.6% had severe reaction with 1 death recorded. Most cases were not preventable (76.7%). Older age (> 60 years) and mucosal involvement were significantly associated with a more severe reaction. Conclusion: The incidence of cADRs was 0.4%, with most cases classified as moderate severity and not preventable. The commonest reaction pattern was DRESS, while the main culprit drug group was antibiotics. Older age and mucosal membrane involvement predicts a severe drug reaction.

Granulomatous facial skin lesions are a rare and challenging clinical problem. Differential diagnoses include cutaneous tuberculosis, sarcoidosis, granulomatous rosacea and acne agnimata. We reported a case of acne agminata presented with granulamatous facial papules.

background: Limited information exists regarding paediatric psoriasis and its association with body mass index (bMI) in Asia. Objectives: to determine the clinico-epidemiological profile and to compare the bMI of children with and without psoriasis. Methods: A case-control study of 92 children with psoriasis versus 59 with atopic eczema and 56 with non-inflammatory skin conditions. results: Psoriasis was more common in Malay and Indian children when compared to Chinese with odds ratios (Or) of 4.30 (95% CI, 1.85-9.99) and 3.00 (95% CI, 1.02-8.81) respectively. Prevalence of psoriasis was similar between Malay and Indian children (Or 1.43, 95% CI, 0.63-3.25). Male:female ratio was 1:1.09. the mean onset age of psoriasis was 7.9 years. Median onset age was earlier in males (6.5 years versus 9.0 years in females, p=0.05). Plaque psoriasis was the most common phenotype (89.1%) and 94.5% had scalp lesions. Arthritis was seen in 4.3%. Odds of excess adiposity, defined as bMI ≥85th percentile, was higher in children with psoriasis versus noninflammatory controls (Or 2.35, 95% CI 0.99-5.56, p= 0.052). No increased risk of adiposity was noted between children with psoriasis and eczema (Or 1.14, 95% CI 0.5-2.62, p=0.753). More children with psoriasis (17.4%) and eczema (20.3%) were underweight (bMI <5th percentile) compared to non-inflammatory controls (10.7%). Conclusion: Malays and Indians are three to four times more likely than Chinese to have psoriasis in multi-ethnic Malaysia. Plaque psoriasis is the most common phenotype. Odds of excess adiposity is about two times higher in children with psoriasis compared to non-inflammatory controls although this observation just missed conventional statistical significance.
Psoriasis

To report a rare case of acute onset of proptosis secondary to subcutaneous panniculitis-like T cell lymphoma (SPTCL). METHODS: A case report. RESULTS: A 27-year-old Malay man presented with history of acute onset of proptosis in the left eye for 2 weeks. It was associated with history of prolonged high fever. He also developed multiple erythematous swelling on his body and both thighs during this period. On examination, visual acuity in both eyes was 6/6. The left eye was proptosed and chemotic.The extraocular move-ment was limited in all directions.The cornea and the anterior segment were normal. Fundoscopy showed normal optic disc and retina.The examination of the right eye was unremarkable.His body temperature was 40.0℃.There was presence of multiple erythematous subcu-taneous lesions over the body mainly in the left axillary, right hypochondriac, both thighs and the suprapubic area.The inguinal lympnodes were also palpable.MRI of orbit and brain revealed generalized soft tissue swelling in the left extraconal and retro-orbital space suggesting of inflammatory reaction.The cavernous sinus and brain were normal.Skin biopsy from the erythematous lesion revealed as SPTCL.He was referred to the haematologist and was started on chemotherapy-CHOP regime. The patient responded well to the chemotherapy and the proptosis reduced. CONCLUSION: Proptosis secondary to SPTCL is very rare. This is a variant of a peripheral T cell lymphoma characterised by multiple subcutaneous nodules presented with proptosis and fever.

Generalized Pustular Psoriasis (GPP) is a dermatological emergency that often requires hospitalizationbecause of possible life-threatening complications, including heart failure, renal failure and sepsis. Itis a chronic recalcitrant disease in which acute pustular flares frequently recur on exposure to classictriggers. This review article is aimed to update the new insights into the genetic basis of GPP andhighlighted the central role IL1 and IL36 in the pathogenesis of GPP.

Background: Cutaneous vasculitis is common, yet the riskfactors for its chronicity have not been established.Objective: To describe the clinical spectrum and identify riskfactors for chronicity of cutaneous vasculitis.Methods: Retrospective data analysis of 275 patientsdiagnosed with cutaneous vasculitis from January 2008 toDecember 2013.Results: The mean age was 33.7 (±17.89) years, with femalepredominance. The majority of patients were Malays (67.3%).Skin biopsy was performed in 110 (40%) patients. Thecommonest sign was palpable purpura (30.6%). Theaetiology remained elusive in 51.3% of patients. Commonidentifiable causes include infection (19.7%) and connectivetissue disease (10.2%). Extracutaneous features were notedin 46.5% of patients. Erythrocyte sedimentation rate andantinuclear antibody were raised in 124 of 170 and 27 of 175patients with documented results respectively. Cutaneousvasculitis was the presenting symptom in seven patientswith newly diagnosed systemic lupus erythematosus. AntiStreptolysin O Titre was positive in 82 of 156 patients withdocumented results. Despite antibiotics, 31.7% of them hadchronic lesions. Prednisolone alone was used in 20% ofpatients while 16.4% needed steroid-sparing agents. Mostpatients who needed systemic therapy (62%) hadunidentifiable aetiology. Among the 155 patients whoremained under follow up, 36.4% had chronic disease, onepatient succumbed due to septicaemia, and the rest fullyrecovered within three months. The presence of ulcerativelesion was significantly associated with developing chronicvasculitis (p=0.003).Conclusion: The clinical spectrum of cutaneous vasculitis inour population was similar to other studies. Ulcerativelesion predicts a chronic outcome