Journal of Cutaneous Medicine and Surgery最新文献

Xanthoma disseminatum (XD) is a rare normolipidemic mucocutaneous xanthomatosis within the spectrum of cutaneous non-Langerhans histiocytosis. Managing XD poses substantial challenges, with limited available data. This study aims to comprehensively evaluate existing literature on clinical features of XD and treatment outcomes. A systematic search of MEDLINE, Embase, and PubMed was performed, using "xanthoma disseminatum" and "Montgomery syndrome" as search terms, without restrictions. Screening was performed in duplicate by 2 reviewers. One hundred fifty-one studies met the inclusion criteria, yielding 166 cases of XD (106 females, 60 males), mean age at diagnosis 35.3 years (range: 9 months-87 years). XD typically presented as yellow-to-brown coalescing papules/plaques and nodules. Distribution affects mainly the face (n = 116/166), flexures (n = 45/166), trunk (n = 65/166), and genitalia/inguinal areas (n = 63/166). Most cases (99.4%; n = 165/166) exhibited extracutaneous manifestations, including the pituitary gland and the oropharynx. Treatment options rendered low complete response rates (CRRs). Treatments with reported outcomes included surgical resection (n = 17/99), systemic steroids (n = 40/99), immunosuppressants/immunomodulators (n = 73/99), energy-based devices (n = 7/99), lipid-lowering agents (n = 24/99), cryotherapy (n = 6/99), lasers (n = 10/99), topical steroids (n = 6/99), oral retinoids (n = 2/99), and radiotherapy (n = 5/99), with CCRs of 23.5% (n = 4/17), 5.0% (n = 2/40), 9.6% (n = 7/73), 14.3% (n = 1/7), 4.2% (n = 1/24), 16.7% (n = 1/6), 10.0% (n = 1/10), 0% (n = 0/6), 0% (n = 0/2), and 0% (n = 0/5), respectively. The most promising therapy is cladribine, with the highest CRR of 27.1% (n = 6/22) and the lowest no response rate (9.1%; n = 2/22) of all reported treatments. This review confirms the high prevalence of systemic manifestations in XD. Treatment options vary widely; thus, further research is needed to establish management strategies for this challenging condition.

Background: The understanding of genital vitiligo among Thai individuals is limited.

Objectives: This study evaluated the clinical presentation, quality of life, and sexual health consequences of genital vitiligo.

Methods: This cross-sectional, questionnaire-based study involving vitiligo patients aged 18 years or older with past or present genital involvement was conducted at Siriraj Hospital. It also measured aspects of sexual health and quality of life.

Results: The mean age of the 41 participants was 48.2 years, and 24 (58.5%) were males. All participants presented with genital vitiligo. In males, the penile shaft (45.8%), scrotum (45.8%), and glans (33.3%) were predominantly affected. In females, the mons pubis (64.7%), labia majora (23.5%), and labia minora (23.5%) were frequently involved. Both sexes reported afflictions in the pubic area (41.5%), inguinal region (36.6%), buttocks (34.1%), and oral mucosa (34.1%). Itching was the principal symptom in 26.8% of the patients. The median Dermatology Life Quality Index scores were significantly different (females 6, males 3.5). Compared with their male counterparts, females exhibited lower self-esteem (41.2% vs 29.2%), greater apprehension about marriage (11.8% vs 8.3%), and embarrassment about sexual activities (23.5% vs 16.7%). Remarkably, 65.9% of patients had not discussed their genital vitiligo with their doctors, and 51.2% of physicians had not inquired about or examined for genital involvement.

Conclusions: Genital vitiligo adversely impacts quality of life and self-esteem, particularly among female patients. The lack of discourse between patients and physicians highlights a need for increased awareness and proactive clinical investigations to enhance patient care and satisfaction.