INTRODUCTION: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disorder causing a mutation in the tumor suppressor genes, TSC1 or TSC2. Loss of function of these genes leads to dysfunction of hamartin and tuberin, resulting in hamartoma formation. It usually manifests with cutaneous manifestations at childhood. However, it also affects other organ systems. Based on the Philippine Dermatological Society Health Information System census, there have been 104 cases of TSC from 2011-2018. Currently, limited data is available regarding the treatment options in the local setting.

CASE REPORT: The case involves a 4 year-old boy, with a two year history of flesh-colored to dusky red fi rm papules on the centrofacial areas and neck. Lesions have been increasing in number since first appearance. He had a normal birth history. Family history was insignificant. However, delay in expressive speech development was noted. Physical examination revealed multiple well-defined angiofibromas on centrofacial areas and neck; fibrous cephalic plaque on the left temporal area, and several ash-leaf spots on the trunk. Periungual and subungual fibromas, confetti macules, shagreen patch and dental pits were absent. Based on the clinical manifestations, he was diagnosed with TSC. Histopathology of a papule on the chin was consistent with angiofibroma. Parents were concerned with the appearance of the lesions and preferred conservative management. Hence, topical sirolimus 0.2% ointment was applied once daily on the angiofibromas for 4 months. Monthly follow-up showed marked improvement, manifested by the decrease in number and by flattening of the lesions.

CONCLUSION: To the best of our knowledge, this is the fi rst case report of successful treatment of topical sirolimus for TSC in the Philippines.

KEYWORDS: tuberous sclerosis complex, topical, treatment success, conservative management, hematoma

Tuberous Sclerosis ; Conservative Treatment ; Hematoma

This is a case of an 18-year-old Filipino female with no known comorbidities, who presented with a three-year history of recurrent erythematous papules, vesicles, and nodules over her bilateral side of the chin. She was initially managed as a case of infected acne vulgaris, and was given oral antibiotics such as Cloxacillin, Rifampicin, and Clarithromycin, but with minimal improvement. She was then referred to Dermatology service for further evaluation. A 3-mm skin punch biopsy on an erythematous nodule on the left chin was done, and histopathologic findings were diffuse eosinophilic infiltrates, exhibiting flame figures admixed with few lymphocytes and neutrophils, consistent with Wells’ syndrome, otherwise known as eosinophilic cellulitis. Serology also revealed elevated IgE level at 949.2 IU/mL but normal eosinophil count. Patient was then given oral corticosteroid for eight weeks, resulting in complete resolution of lesions and no residual scarring.
Wells syndrome ; Cellulitis ; Eosinophilia

Introduction: Sneddon-Wilkinson disease (SWD) is a rare, recurrent neutrophilic dermatosis presenting as sterile pustules, with a predilection for flexural and intertriginous areas. Case summary: A 49-year-old Filipino female presented with a three-year history of recurrent pustules and papules on the flexural areas of trunk and extremities. Skin punch biopsy was done and histopathology was consistent with subcorneal pustular dermatosis/SWD. She was started on Dapsone but after two weeks of intake, the patient developed generalized erythematous desquamating plaques on the trunk and extremities, with palmoplantar involvement. The patient did not have fever, jaundice, lymphadenopathy, and abdominal tenderness. Laboratory investigation such as complete blood count and liver function tests were normal. The final diagnosis was SWD with hypersensitivity to Dapsone. Dapsone was immediately discontinued and she was shifted to oral colchicine. After six weeks of oral colchicine therapy, the lesions have completely resolved. Patient was in remission for six months thereafter. Conclusion SWD is rare and the drug of choice is dapsone. In instances where dapsone is not suitable, oral colchicine can be an ideal alternative treatment.
Skin Diseases, Vesiculobullous ; Dapsone ; Colchicine