Renal amyloidosis secondary to hidradenitis suppurativa
نویسندگان
چکیده
Hidradenitis suppurativa (HS), also known as Verneuil’s disease [1] or acne inversa, is characterized by hyperkeratosis of the follicular epithelium, resulting in obstruction of the follicular stoma and chronic perifollicular inflammation with remissions and relapses, usually involving skin overlying the axillary, inguinal, perianal, perineal and inframammarian regions. It usually presents in the second and third decades of life, has a female preponderance, no racial predilection and a prevalence of 4%. Some causing factors might be obesity and related hormonal changes, hyperandrogenism, immunologic and genetic status, diet and smoking [2]. The diagnosis is based on the presence of relapsing lesions in typical anatomical regions. HS may be localized with chronic, relapsing, superficial papulopustular lesions, as it may be associated with deep, widespread abscesses, sinus tracts, fistulas, malignancies like squamous cell carcinoma and systemic complications like arthropathy or anaemia [3]. Besides topical and surgical care, therapy with antibiotics, steroids, retinoids and anti-TNF agents is usually considered. Rarely, amyloidosis may complicate the cutaneous disease [4]. A 42-year-old male was referred to our hospital for nephrotic syndrome. Three years before he was diagnosed with hidradenitis suppurativa when papulopustular lesions over the inguinal, perineal, perianal and axillary regions appeared. Over these 3 years, he had relapses with fever, extensive perineal tenderness and partial remissions leaving fibrotic scar tissue following treatment with topical steroids, keratolytics and antibiotics. His temperature was 37.1°C, blood pressure 140/90 mmHg and heart rate 76/min. He had bilateral pretibial pitting oedema and active bilateral inguinal, perineal, perianal and left axillary papulonodular and pustular, hyperaemic, tender lesions of 2–3 cm diameter, some of which fluctuating and draining, scar tissue, sinus tracts of the older lesions and bilateral inguinal and left axillary tender lymphadenopathies, the largest of which being 2 cm in diameter (Figure 1).
منابع مشابه
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