RESUMO
Lichen amyloidosis (LA) is a form of primary localized cutaneous amyloidosis (PLCA) characterized by bilateral intensely itchy domed scaly hyperkeratotic papules. Lichen amyloidosis is rare and affects men more than women. It is uncommonly seen in the western world but more prevalent in Asia. These papules most typically affect the shins and occasionally the arms and torso. Lichen amyloidosis has been reported in association with autoimmune disorders and after prolonged exfoliation and friction of affected skin. We present a 40-year-old woman with LA. In LA, the characteristic histological finding is apple-green birefringence of Congo red-stained preparations observed under polarized light. However, this is not always strongly positive, as in our patient. Other findings may include eosinophilia, periodic acid-Schiff positivity, staining with thioflavin T, and metachromasia after staining with crystal violet or methyl violet. Treatment of LA is difficult and complete clinical remission is seldom achieved. Recent trials revealed beneficial outcomes with topical calcipotriol, phototherapy, acitretin, cyclophosphamide, and laser treatments. A combination of acitretin, antihistamines, topical steroids, and hydrocolloid dressings have been beneficial in our patient with LA.
Assuntos
Amiloidose Familiar/patologia , Dermatopatias Genéticas/patologia , Pele/patologia , Adulto , Amiloidose Familiar/complicações , Amiloidose Familiar/diagnóstico , Feminino , Humanos , Prurido/etiologia , Dermatopatias Genéticas/complicações , Dermatopatias Genéticas/diagnósticoRESUMO
BACKGROUND: Hidradenitis suppurativa (HS) in children and young adults (CYA) (<18 years) is uncommon. No previous observational studies have been carried out in a UK CYA HS population. METHODS: This study was based on retrospective case note reviews of CYA HS patients attending tertiary-level care in the CYA HS service in a UK hospital. Patients <18 years old with a known diagnosis of HS were screened for inclusion. Exclusion criteria were those with less than one follow-up appointment. RESULTS: Twenty-eight CYA HS patients were identified, with an M:F ratio of 1:8.3. Mean BMI was 25.2 (SD: 7.6). 17 (61%) of cases had a relevant family history. Long-term antibiotic monotherapy was the most common treatment initiated. Lymecycline was the most commonly prescribed antibiotic, accounting for 23 (56%) of the 41 courses prescribed. Additional treatments initiated included dual therapy with rifampicin and clindamycin, isotretinoin, and adalimumab, which were more commonly prescribed in patients with Hurley Stage II or III. CONCLUSIONS: This group had a female predominance with an apparent strong genetic predisposition which is seen in other HS CYA cross-sectional research. Treatment was varied in this cohort, however long courses of antibiotics, including combined therapy with rifampicin and clindamycin, were the mainstay of treatment, similar to management in the adult population. This study therefore adds to the limited information on the demographics and management of the HS CYA population.