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

After cutaneous and neurological manifestations, bone and joint involvement are the most common manifestations in Hansen’s disease (HD). Sometimes, these are undiagnosed and rarely reported.

A 68-year-old Filipino, male, presented with an 8-month history of digital joint swelling on both hands. He was initially managed by Rheumatology for arthritis, partially responsive to Methotrexate and Prednisone. Immunology and lupus panel tests were unremarkable. Skin biopsy done on a solitary erythematous plaque on the back revealed granulomatous dermatitis, tuberculoid type. Fite-Faraco, AFB, PAS stains were negative. Four weeks later, he developed multiple erythematous papules and plaques on the face, ears, lower back, gluteal area, and lower extremities. Arthritis was persistent but hypoesthesia, hypohidrosis or alopecia were not reported. A repeat skin biopsy showed epithelioid to macrophagic cell granulomatous reaction compatible to HD. AFB stain and slit skin smear were positive. He was managed as a case of Borderline (BB) to Borderline Lepromatous (BL) leprosy and was started on multibacillary drug treatment (MDT) of Rifampicin, Clofazimine and Dapsone. One month later, the patient reported resolution of skin lesions and arthritis.

Approximately 75% of individuals with HD experience arthritis, which can be one of the early signs of disease. A high index of suspicion is required as it closely mimics other rheumatologic conditions.

Human ; Male ; Aged: 65-79 Yrs Old ; Arthritis ; Rheumatology

Introduction: Psoriasis and some of its treatments such as methotrexate have been linked to the development of non-melanoma skin cancers including cutaneous squamous cell carcinoma (SCC). Chronic maque psoriasis, Koebnerization, and prolonged methotrexate therapy are some of the concerns that may impact wound healing and graft uptake when treating these patients. Case Report: We report a case of a 64-year-old male with a 32-year history of moderate to severe psoriasis continuously self-medicating with methotrexate for 30 years who presented with a solitary indurated tumor with ulceration on the right anterior leg. Histopathology result revealed acantholytic cSCC. The patient concomitantly has generalized psoriatic plaques that complicated the selection of donor site for the skin graft, and raised concerns on wound healing and graft uptake. He underwent wide excision surgery with gastrocnemius (medial head) flap and split thickness skin graft. Platelet-rich plasma (PRP) injections were utilized post-operatively to increase graft survival and donor site regeneration. Discussion The main risk factors for the development of cSCC for this patient are the history of chronic plaque psoriasis and chronic methotrexate therapy. These two can also complicate the success of grafting and wound healing for this patient. PRP was utilized to for better graft survival, faster wound healing, and prevention of Koebnerization.
Platelet-Rich Plasma ; Neoplasms, Squamous Cell ; Psoriasis ; Methotrexate