Introduction: Basal cell nevus syndrome (BCNS) (Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome) is a rare inherited multisystem and tumor-predisposing disorder caused by the patched tumor suppressor gene mutations and suppressor of fused gene. Its diagnosis follows a set of criteria based on specific cutaneous features and radiologic findings. Although an autosomal dominant disorder with a high degree of penetrance, BCNS has variable expression making its diagnosis difficult. The limited epidemiologic data among Asians especially in the Philippines hamper early detection or cause frequent misdiagnosis of the condition. Case report: A 56-year-old Filipino female with Fitzpatrick skin type V presented with early onset multiple basal cell carcinomas and bilateral palmoplantar pits. Radiologic investigation reveals odontogenic keratocyst, calcification of the falx cerebri, bridging of the sella turcica, bifid/splayed ribs and vertebral anomalies. The patient exhibits coarse facial features and bilateral cataracts. Cranial computed tomography scan shows cerebrocerebellar atrophy with ventricular dilatation. Management included wide excision of the nodular basal cell carcinomas (BCC), application of 5-flourouracil cream on the superficial BCC and electrodessication and curettage of the smaller lesions. Oral acitretin was also prescribed. Conclusion This is a case that highlights the approach to diagnosis, clinical features and management of BCNS in a Filipino patient. Since various phenotypic presentations may exist among dark-skinned individuals, early diagnosis poses a challenge among physicians. Epidemiologic and prevalence studies among Filipinos may be done to aid in the diagnosis and early management of this rare genodermatosis.
Basal Cell Nevus Syndrome ; Carcinoma, Basal Cell

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.
Scleroderma, Localized ; Phototherapy