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BACKGROUND: Primary cutaneous macular amyloidosis (PCMA) is a chronic pruritic cutaneous disease characterized by heterogeneous extracellular deposition of amyloid protein in the skin.
OBJECTIVE: This study aimed to evaluate the efficacy of topical 6% gabapentin cream for the treatment of patients with PCMA.
METHODS: In this triple-blind clinical trial, a total of 34 patients, who were diagnosed with PCMA, treated using two different strategies of topical gabapentin as the active group and vehicle cream as the control group.
RESULTS: Pruritus score reduction in both groups was statistically significant compared with the baseline value (p < 0.001). There was a significant pigmentation score reduction in intervention group compared with control group after 1 month of the study (p < 0.001). The differences of pigmentation score changes between the groups were not significant at month 2 (p = 0.52) and month 3 (p = 0.22).
CONCLUSIONS: The results of this study suggest that topical gabapentin cream may be effective as a topical agent in the treatment of pruritus associated with PCMA without any significant adverse effects. It is recommended to perform similar studies with a larger sample size and longer duration in both sexes.

Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-linked hereditary disorder characterized by the triad of follicular hyperkeratosis-photophobia-alopecia. The clinical heterogeneity makes the diagnosis difficult. To investigate the clinicopathologic and trichoscopic features of KFSD and to further clarify the essential requisites for the diagnosis, we conducted a retrospective study of patients with KFSD. The clinical information, histologic features, and trichoscopic findings were evaluated. Eight patients were from seven separate families. Two females were mother and daughter from the same family and the other six patients were male and represented sporadic cases. The average age of onset of alopecia was 21.25 years. Involvement of the scalp hairs leading to progressive scarring alopecia on the midline of the scalp with variable degrees of inflammation was the pathognomonic feature. It typically began after puberty. Vellus hair-associated follicular hyperkeratosis affected all of the patients. However, photophobia was not a constant feature. Histopathologic examination revealed disorders of the hair follicle with an acute-chronic inflammatory response. Follicular changes including fused infundibulum, the protrusion of the outer root sheath into the follicular canal, and a dilatation of the follicles at the isthmus level caused by the occlusion of keratin were observed. The trichoscopic features included perifollicular scaling, tufted hairs, and loss of follicular openings. In conclusion, terminal hair involvement, either scalp hairs, eyebrows, or eyelashes, and the hyperkeratosis of the follicle of vellus hairs is the diagnostic basis of KFSD. We hypothesize that follicular changes in histopathology are the primary event that trigger variable inflammation and further follicular destruction.

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OBJECTIVE: Stiff skin syndrome (SSS) is a rare disorder characterized by \"rock hard\" indurated skin affecting different body parts. The localized variant poses a diagnostic challenge, as it is frequently mistaken for other inflammatory connective tissue disorders. The aim of this study is to provide insightful clinical, radiologic and diagnostic data that might prove useful for the evaluation, management and treatment of pediatric patients with segmental SS.
METHODS: This single-center cohort study included patients ≤18 years diagnosed with localized SSS from 1988 to 2021 in a quaternary pediatric healthcare center in Toronto, Canada. Data included demographics, clinical, histopathologic and radiologic features, treatments, and clinical course. Data were summarized with descriptive statistics (mean, standard deviation, medians, interquartile ranges [IQRs]) and frequencies.
RESULTS: A total of 11 patients were included. The sclerotic changes were measured clinically and radiologically, by a total of 16 imaging studies: 13 magnetic resonance imaging (MRI) and 3 ultrasound. MRI readings showed abnormal high signal intensity of the affected tissue correlating with the anatomical site of involvement in all cases, specifically, in the shoulder/pelvic girdle with limb extension. Shear wave ultrasound elastography (SWE) demonstrated higher values within the dermis compared to the control site.
CONCLUSIONS: The presence of segmental sclerotic changes that affects the pelvic/shoulder girdle with extension to the extremities, in the absence of inflammation on biopsy and abnormal signaling intensity on imaging is suggestive of SSS. Skin SWE is a feasible, noninvasive, and objective instrument to evaluate and monitor sclerotic changes overtime, it could be potentially extrapolated to other pediatric skin sclerotic conditions.

BACKGROUND: Stiff skin syndrome (SSS) is a rare disease characterized by sclerosis of the skin. Cases of both widespread and segmental SSS have been reported.
OBJECTIVE: To report the clinical and histopathological characteristics of a large series of SSS.
METHODS: We retrospectively analysed the clinical and histopathological characteristics of widespread and segmental SSS collected from a dermatology department. We also compared histopathology between segmental SSS and morphea.
RESULTS: Thirty-one cases, including three widespread SSS and 28 segmental SSS, were collected. Skin lesions of widespread SSS generally showed skin sclerosis concentrating over the lumbar, buttocks, thighs, proximal part of limbs, and shoulders with specific abnormal gait and posture. Skin lesions of segmental SSS generally showed sclerotic plaques involving the thigh, lumbar area and buttocks, associated with hypertrichosis, hyperpigmentation and a cobblestone appearance. Segmental SSS did not typically cause joint limitation or serious physical discomfort. Histopathologically, SSS showed proliferation of fibroblasts and sclerosis of collagen in the dermis or subcutaneous tissue. Compared with morphea, SSS showed more prominent proliferation of fibroblasts and completely lacked lymphocyte infiltration.
CONCLUSIONS: Segmental SSS represents the major variant of SSS. Histopathologically, SSS shows proliferation of fibroblasts, sclerosis and an absence of inflammation.

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BACKGROUND: Different therapies have been used for dissecting cellulitis of the scalp (DCS) with poor results. Topical 5-aminolaevulinic acid photodynamic therapy (ALA-PDT) is effective for many inflammatory skin diseases.
OBJECTIVE: To evaluate the efficacy and safety of fire micro-needling plus 5% ALA-PDT on DCS.
METHODS: Forty-two male DCS patients were enrolled and treated by four sessions of fire micro-needling plus 5% ALA-PDT (occlusion time: 3 h, 100 mW/cm2 for 20-32 min, 633 ± 10 nm LED) with an interval of 1-2 weeks. The reduction of lesions was evaluated at each follow-up visit. The patients` symptoms were evaluated by Dermatology Life Quality Index (DLQI). All of the side effects were recorded.
RESULTS: Forty-one DCS patients finished the treatments and the marked effective rate (MER) reached 65.85%. Ten patients (24.39%) were cured and 17 patients (41.46%) achieved excellent improvement. The median of lesion numbers decreased significantly from 25 to 7 (p < 0.001) with DLQI from 13 to 2 (p < 0.001). Although all the ten cured patients had a relapse during the 12-month follow-up, 5/10 patients had a remission of more than 6 months. All the patients tolerated PDT well with mild adverse effects.
CONCLUSIONS: Fire micro-needling plus 5% ALA-PDT is effective for Chinese male DCS patients without the need to take systemic drugs.

BACKGROUND: Medicinal therapies such as systemic retinoids and antibiotics have shown efficacy in the treatment of dissecting cellulitis of the scalp (DCS), but refractory cases are common. 5-Aminolevulinic acid photodynamic therapy (ALA-PDT) has been successfully used in medicine-resistant cases in recent years.
METHODS: A total of 12 patients who received ALA-PDT in addition to formally failed medicinal therapies were interviewed by telephone regarding improvement of symptoms, satisfaction, and side effects.
RESULTS: Overall, 58.3 % patients achieved greater than 50 % improvement of at least one symptom, and 16.7 % had little improvement for any symptom. The proportion of patients who had greater than 50 % improvement for pain, pruritus, swelling and suppuration were 36.4 %, 66.7 %, 33.3 % and 58.3 % respectively. 41.7 % patients were either satisfied or very satisfied with the ALA-PDT. The side effects were mainly pain and crusting that were mostly mild and recovered quickly.
CONCLUSIONS: ALA-PDT was safe and partially effective in reducing DCS symptoms as an adjunct to systemic medicinal therapies.