Weber-Christian Disease (WCD), or relapsing febrile nodular panniculitis is a rare form of subcutaneous fat inflammation commonly affecting women aged 40-75 years old. It is rarer in children with only 1 previously reported case in the Philippines. It presents as erythematous nodules, frequently affecting the lower extremities. There is no standard treatment, but reportedly improves with steroid therapy, cyclosporine, and immunomodulators.

This is a case of a 12-year-old-female, who presented with a 4-month history of palpable facial nodules, thigh pains, and recurrent fever. Work-up for hypersensitivity reaction, autoimmune condition, hematologic problem, or infectious etiology yielded unremarkable findings, and corresponding management had no significant response. Patient developed firm lesions on the abdomen which was sent for biopsy and showed results consistent with WCD. Steroid, hydroxychloroquine, and topical indomethacin were then started and noted gradual improvement of the lesions. Patient was then discharged improved with lesions noted to progressively decrease in size and with no appearance of newer lesions upon follow-up.

WCD is a rare form of nodular panniculitis, more so in the younger age group. It is characterized by presence of cutaneous lesions associated with systemic symptoms. Skin biopsy is necessary to confirm its diagnosis. Visceral organ involvement and failure to respond to treatment may result to poor prognosis, and occasionally leads to death.

Human ; Male ; Child: 6-12 Yrs Old ; Linear Iga Bullous Dermatosis ; Immunoglobulin A ; Skin Diseases

Weber-Christian Disease (WCD), or relapsing febrile nodular panniculitis is a rare form of subcutaneous fat inflammation commonly affecting women aged 40-75 years old. It is rarer in children with only 1 previously reported case in the Philippines. It presents as erythematous nodules, frequently affecting the lower extremities. There is no standard treatment, but reportedly improves with steroid therapy, cyclosporine, and immunomodulators.

This is a case of a 12-year-old-female, who presented with a 4-month history of palpable facial nodules, thigh pains, and recurrent fever. Work-up for hypersensitivity reaction, autoimmune condition, hematologic problem, or infectious etiology yielded unremarkable findings, and corresponding management had no significant response. Patient developed firm lesions on the abdomen which was sent for biopsy and showed results consistent with WCD. Steroid, hydroxychloroquine, and topical indomethacin were then started and noted gradual improvement of the lesions. Patient was then discharged improved with lesions noted to progressively decrease in size and with no appearance of newer lesions upon follow-up.

WCD is a rare form of nodular panniculitis, more so in the younger age group. It is characterized by presence of cutaneous lesions associated with systemic symptoms. Skin biopsy is necessary to confirm its diagnosis. Visceral organ involvement and failure to respond to treatment may result to poor prognosis, and occasionally leads to death.

Human ; Behavior ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Diabetic Foot ; Foot Ulcer

INTRODUCTION: The COVID-19 pandemic has changed care provision models, with a rapid increase in the adoption of telemedicine to reduce in-person visits. Although there are many benefits to teledermatology, there are also factors that hinder its widespread adoption. We aimed to examine patients' perceptions of teledermatology to identify the barriers to its adoption. METHODS: A prospective study was conducted from 15 June to 14 August 2020. Patients were invited to complete a questionnaire in an outpatient dermatology clinic via direct approach by clinical staff or posters posted at the door of consultation rooms. RESULTS: Out of 2,276 clinic attendances, 997 survey responses (43.8%) were collected over a 3-month period. When asked if they would change their subsequent visit to teledermatology, 294 (29.5%) patients were keen, 166 (16.6%) were unsure and 537 (53.9%) declined. Significant factors for declining teledermatology were lack of prior exposure to videoconferencing ( P < 0.01) and lower educational level ( P = 0.019). Patients also raised concerns regarding the ability of teledermatology to address medical concerns (32.1%) and indicated a preference for face-to-face consultation (29.7%). CONCLUSION Factors that influence patients' decision to adopt teledermatology, such as concerns about its ability to address medical issues, lack of IT literacy or experience in teleconferencing, are modifiable. Targeted strategies such as careful patient selection, a dedicated teleconsultation workflow, and the use of a novel 'teledermatology patient journey' (including a clinic walkthrough at the first visit) and an intuitive audio-enabled user interface, may improve patient perceptions and adoption of teleconsultation service.
Humans ; COVID-19/epidemiology* ; Dermatology/methods* ; Telemedicine ; Male ; Female ; Prospective Studies ; Middle Aged ; Adult ; Surveys and Questionnaires ; SARS-CoV-2 ; Aged ; Perception ; Young Adult ; Pandemics ; Skin Diseases/diagnosis* ; Videoconferencing

Stiff skin syndrome is a very rare non-inflammatory reactive skin disease, characterized by skin sclerosis and limited joint mobility. The paper reports one case of child with stiff skin syndrome and treated with combined therapy of acupuncture and cupping. Acupuncture was used at the lateral line 1 of vertex (MS8) on the right side, Jiaji (EX-B2) of L₂ to L₄, Huantiao (GB30), ashi point, Juliao (GB29), Fengshi (GB31), Weizhong (BL40), and etc. on the left side. After deqi, the electrodes of KWD-808Ⅰimpulse electronic therapeutic device were attached to Jiaji (EX-B2) of L₄ and Huantiao (GB30), Fengshi (GB31) and Yanglingquan (GB34) on the left side respectively, at disperse-dense wave, a frequency of 2 Hz/100 Hz, and a current of 2 mA. The needles were retained for 20 min. Acupuncture was operated once every 2 days, 3 interventions a week. When acupuncture was completed in each intervention, moving cupping was followed till the skin turned to be red, along the distribution of the governor vessel, foot-shaoyang gallbladder meridian and foot-taiyang bladder meridian on the left side, of the lumbar region and leg. Moving cupping was delivered once every 2 days, 3 times a week. Once a week, after moving cupping, the cups were retained on the areas with skin stiffness for 8 min to 10 min. One course of the combined therapy of acupuncture and cupping was composed of 6 treatments. After 2 courses of treatment, the skin stiffness on the left buttock region and the lateral side of the lower limb was ameliorated, the swelling on the left lower limb relieved and the walking improved; and the patient could walk continuously for 2 000 m. The combined therapy of acupuncture and cupping provides a new idea for the clinical treatment of stiff skin syndrome.
Child ; Humans ; Acupuncture Points ; Acupuncture Therapy ; Contracture/therapy* ; Cupping Therapy ; Skin Diseases, Genetic/therapy*

The paper reports one case of primary cutaneous amyloidosis (PCA) treated by tapping with plum-blossom needle combined with sulfur ointment and local irradiation. PCA in this case was manifested as generalized erythema, papules, plaques, lichenification, and severe pruritus. In treatment, tapping with plum-blossom needle was delivered at typical lesions to induce local congestion, redness, and minimal bleeding. After cleaned with sterile gauze for 10 s, 25% sulfur ointment was evenly applied, followed by local irradiation with a TDP lamp for 15 min. This session was repeated twice a week. In 1 month of treatment, the lesions turned flat and the skin was soft as the normal, with pigmentation and mild pruritus left. In 3 months of follow-up, no papules recurred, and mild pruritus presented occasionally.
Humans ; Ointments/administration & dosage* ; Sulfur/administration & dosage* ; Skin Diseases, Genetic/radiotherapy* ; Middle Aged ; Amyloidosis, Familial/radiotherapy* ; Male ; Acupuncture Therapy/instrumentation* ; Female ; Combined Modality Therapy

Tissue-resident memory T (TRM) cells are a recently defined subtype of non-recirculating memory T cells with longevity and protective functions in peripheral tissues. As an essential frontline defense against infections, TRM cells have been reported to robustly patrol the tissue microenvironment in malignancies. Accumulating evidence also implicates that TRM cells in the relapse of chronic inflammatory skin diseases such as psoriasis and vitiligo. In light of these developments, this review aims to synthesize these recent findings to enhance our understanding of TRM cell characteristics and actions. Therefore, after providing a brief overview of the general features of the TRM cells, including precursors, homing, retention, and maintenance, we discuss recent insights gained into their heterogeneous functions in skin diseases. Specifically, we explore their involvement in conditions such as psoriasis, vitiligo, fixed drug eruption - dermatological manifestations of drug reactions at the same spot, cutaneous T cell lymphoma, and melanoma. By integrating these diverse perspectives, this review develops a comprehensive model of TRM cell behavior in various skin-related pathologies. In conclusion, our review emphasizes that deciphering the characteristics and mechanisms of TRM cell actions holds potential not only for discovering methods to slow cancer growth but also for reducing the frequency of recurrent chronic inflammation in skin tissue.
Humans ; Skin Diseases/immunology* ; Memory T Cells/immunology* ; Animals ; Vitiligo/immunology* ; Psoriasis/immunology* ; Immunologic Memory

Case 1 was a 7-year-old girl; Case 2 was her 3-year-old younger brother. Both children developed pink urine shortly after birth and exhibited blistering on photo-exposed areas (face and hands), followed by ulceration, crusting, scarring, and joint contractures leading to impaired mobility. Genetic testing in both patients identified a homozygous variant in the UROS gene, c.776T>C (p.Leu259Pro), confirming autosomal recessive congenital erythropoietic porphyria due to UROS mutations. This case report highlights that congenital erythropoietic porphyria should be considered in infants and young children with unexplained hemolytic anemia, pink urine, and severe photosensitive dermatitis. Early genetic testing is recommended to facilitate timely intervention and improve outcomes.
Humans ; Porphyria, Erythropoietic/genetics* ; Female ; Child ; Child, Preschool ; Male ; Siblings ; Mutation ; Uroporphyrinogen III Synthetase/genetics*

Skin fibrosis is primarily characterized by excessive fibroblasts proliferation and aberrant extracellular matrix accumulation, leading to pathological conditions such as hypertrophic scars, keloids, and systemic sclerosis. This dynamic and complex process involves intricate interactions among various resident skin cells and inflammatory cells, ultimately resulting in extracellular matrix deposition and even invasive growth. The maintenance of cellular phenotypes and functions relies on dynamic metabolic responses, and cellular signal transduction is closely coupled with metabolic processes. Given that the coupling of cell metabolism and signaling in the skin fibrosis microenvironment plays a critical role in inflammatory responses and fibrotic activation, modulation of these metabolic pathways may offer novel therapeutic strategies for inhibiting or even reversing the progression of skin fibrosis. This review systematically summarizes the metabolic characteristics of various cell types involved in skin fibrosis, with a focus on core metabolic reprogramming mechanisms such as hyperactive glycolysis, dysregulated fatty acid metabolism, cellular metabolic dysfunction and dysregulated mTOR/AMPK signaling. Furthermore, potential intervention strategies targeting these metabolic pathways are explored, thereby providing new research perspectives for the treatment of skin fibrosis.
Humans ; Fibrosis/metabolism* ; Skin/metabolism* ; Signal Transduction ; Fibroblasts/pathology* ; TOR Serine-Threonine Kinases/metabolism* ; Skin Diseases/pathology* ; Cellular Reprogramming ; Metabolic Reprogramming