Treatment of morphea with narrowband ultraviolet B: A case series

Introduction@#Morphea, is a rare autoimmune disease presenting with fibrotic changes in the dermis and subcutis. It is a benign condition associated with significant atrophy and sclerosis leading to disfigurement, flexure contractures, and impaired function. Ultraviolet A1 and photochemotherapy are highly effective treatment options but are not readily available in the country. Narrowband ultraviolet B (NBUVB), on the other hand, is readily available, affordable, and safe to use. @*Case summary@#Three patients diagnosed with different variants of morphea (bilateral generalize morphea, unilateral generalized morphea, and circumscribed morphea). underwent 30 sessions of NBUVB. Treatment response was assessed using tightness and itch Visual Analogue Scale (VAS), Modified Skin Score (MSS), photographic comparison, ultrasonographic measurement, and histopathologic analysis. NBUVB treatment resulted to 14-60% decrease in the tightness and itch VAS. MSS was also reduced by 35-50%. The size, pigmentation, and erythema of the lesions also decreased. Ultrasonography showed an improvement in the thickness of lesions after treatment. Histopathologic study showed less packed collagen with increase in inter-bundle spaces.@*Conclusion@#Response to treatment was influenced by the age of the lesion and anatomical location. More chronic lesions tend to have less response. Lesions on the face exhibited the greatest improvement while lesions on the lower extremities had the least improvement. This is the first case series study in the country that uses NBUVB as treatment for morphea. The improvement of the sclerotic and atrophic lesions treated with narrowband UVB treatment may be an acceptable substitute for UVA1 and PUVA.

Bullous systemic lupus erythematosus and membranous lupus nephritis in a Filipino child

@#<p style="text-align: justify;">Bullous eruptions are rare cutaneous manifestations of systemic lupus erythematosus. We report a case of an 8-year old Filipino girl with vesiculobullous systemic lupus erythematosus (SLE) and membranous lupus nephritis on kidney biopsy who presented with clinical nephrotic features of generalized edema, proteinuria, hypoalbuminemia and hyperlipidemia. The 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE were met. Immunohistopathologic examination of the skin lesion revealed a sub-epidermal split with neutrophilic infiltrates along the dermo-epidermal junction, moderate perivascular, periadnexal and interstitial infiltrates composed of predominantly neutrophils with neutrophilic dusts, lymphocytes, plasma cells, rare eosinophils and increased dermal mucin. Direct immunofluorescence showed strong continuous linear IgG deposits along the basement membrane and weak linear IgM and IgA deposition along the basement membrane zone (BMZ). To our knowledge, this is the first report of vesiculobullous SLE in a Filipino child. This case is a rare form of cutaneous lupus in children. Bullous SLE (BSLE) should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.</p>

Multiple neurofibromas with hypertrichosis in a Filipino patient with neurofibromatosis 1

@#<p style="text-align: justify;">This is a case of an 11-year-old male who presented with multiple neurofibromas with hypertrichosis. Classic cutaneous neurofibromas, café-au-lait macules, axillary freckling, Lisch nodules, and scoliosis were also present fulfilling a diagnosis of Neurofibromatosis type 1. This is the first report of multiple neurofibromas with hypertrichosis in the Philippines. Hypertrichosis overlying a neurofibroma is rarely reported and the mechanism remains to be elucidated.</p>

Multiple eruptive myxoid dermatofibroma in a male with chronic hepatitis B infection

@#<p style="text-align: justify;">We report the first published case of multiple eruptive myxoid dermatofibroma (MEMDF) in a male with chronic hepatitis B infection presenting with eruptive lesions showing marked deposits of dermal mucin. Alcian blue and immunohistochemistry confirmed the diagnosis of myxoid dermatofibroma. Further work-up showed asymptomatic chronic hepatitis B infection without cirrhosis.This case highlights an extremely rare histologic variant and the importance of screening for altered immunity in patients with eruptive dermatofibromas.</p>