Darier disease is an autosomal dominant manner, with complete penetrance and variable expressivity. The condition is caused by mutations in the ATP2A2 gene, which encodes SERCA2. Family history is often overlooked due to subtle clinical presentations and presentation is common at around 20 years of age.

Case Report:This is the case of a 79-year-old elderly female who presented with a three year history of intensely pruritic erythematous hyperkeratotic papules on the face, trunk, and all extremities aggravated by heat and sweating. She reported of similar skin lesions seen on her father and brother, however they were undiagnosed and not treated. Multiple medications, including Prednisone, topical steroids, and antibiotics, provided temporary relief. Skin punch biopsy was done which revealed Acantholytic acanthoma consistent with Darier’s disease. CBC, SGPT, SGOT, Alkaline Phosphatase, BUN, Creatinine, Lipid Profile, FBS were requested and she was managed with topical corticosteroids, isotretinoin, Urea 10% lotion, and oral anti-histamines.

Darier disease is an autosomal dominant genodermatosis caused by mutations in the ATP2A2 gene, often presenting during puberty with chronic symptoms like hyperkeratotic lesions and nail abnormalities. Diagnosis relies on clinical and histopathologic correlation, aided by family history, though variable expressivity can complicate it. Coexisting infections must be evaluated due to potential morbidity. Treatment options include systemic retinoids, immunomodulators, and topical therapies like retinoids and calcineurin inhibitors. Personalized therapies have shown promise. Patient education on trigger avoidance and genetic counseling is crucial for managing recurrence risk, while life expectancy remains comparable to the general population.

Human ; Female ; Aged: 65-79 Yrs Old ; Darier Disease ; Keratosis Follicularis

Mycosis fungoides is the most common type of cutanoues T-cell Lymphoma. It is a mature T-cell non-Hodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. This case reports a 24-year-old female who presented with a two-year history of progressively increasing hypopigmented macules and patches. Skin punch biopsy and immunohistochemical stains done were suggestive of a malignant T-cell process. The patient was managed with a short course topical corticosteroids and maintained on a topical moisturizer. The patient underwent multiple sessions of narrowband UV-B Phototherapy at 2-3 times per week and showed significant improvement of the skin lesions hence, was advised to be maintained on phototherapy.

Human ; Female ; Young Adult: 19-24 Yrs Old ; Mycosis Fungoides ; Phototherapy

Introduction: Narrowband ultraviolet B (NBUVB) phototherapy is a well-established treatment option for a variety of dermato- logic conditions. The initial dosage is obtained either by determining the patients’ minimal erythema dose (MED) or their Fitzpat- rick skin phototype (SPT). MED determination is a better way to establish the proper initial dose as it is more objective. However, in practice, SPT is more commonly used as it is more convenient, and MED data in Filipinos are scarce. Objectives: To establish data determining the MED values of Filipino adults that can serve as a basis to standardize the initial dose of NBUVB phototherapy in a tertiary hospital. Methods: We enrolled 86 volunteers in a cross-sectional analytical study to determine their MED and assess if there is any asso- ciation between their MED and the participants’ age, sex, skin prototype, ancestry, and daily duration of sun exposure. Results: The median MED of the participants is 800 mJ/cm2 (IQR 600-800 mJ/cm2 ). A majority of 38 participants (44.19%) have a MED of 800mJ/cm2 followed by 600mJ/cm2 for 23 (26.74%) participants. There was also a significant association between study partici- pants’ MED with respect to their Fitzpatrick skin type (p=<0.001) and ancestry (p=0.03), but with no association with regards to age (p=0.291), sex (p=0.245), and daily duration of sun exposure (p=0.237). Conclusion Majority of the participants have a median MED value of 800 mJ/cm2. Based on this MED value, the initial dosage of NBUVB at 50-70% of the MED would translate to an initial dose of 400-560 mJ/cm2.
Erythema ; Phototherapy

Background: Ultraviolet radiation has been proven to result in unwarranted effects on the skin through reactive oxygen species (ROS) and direct DNA damage. Lycopene, a naturally occurring substance, acts as an antioxidant by neutralizing ROS. Objective: The objective of the study was to determine the efficacy of oral lycopene supplementation for photoprotection in adult Filipino patients seen in a tertiary hospital in Makati City. Design: The study design involves single-blind, parallel, randomized controlled trial. Methods: Thirty-six Filipino patients aged 18 years old and above with Fitzpatrick Skin Phototype (FSP) III–V were divided into two groups using a computer-generated randomization. Group A received lycopene 500 mg/ soft gel capsule two capsules per orem once daily for 12 weeks, while Group B received no intervention during the entire observation period. Minimal erythema dose (MED) of patients from both groups was assessed by a single treatment-blinded reader at baseline, week 6, and week 12. Results: Group A showed a significant increase in MED across periods, with a 20.83% increase from baseline at week 6 and a 43.06% increase at week 12. Group B MED remained constant from baseline to week 6 and to week 12. These results show that there is a significant effect in the increase in MED as compared to the control group. Conclusion Oral lycopene is effective in increasing the MED of patients and may be used for photoprotection among patients with FSP III–V.
Lycopene