毛囊角化病并发回状颅皮

A case of darier disease combined with cutis verticis gyrata

报告1例毛囊角化病并发回状颅皮。患者男,46岁。头皮、颈项部丘疹、斑块伴瘙痒30余年,泛发全身10余年。皮肤科检查:头皮皱褶呈深沟壑状,其上可见表面砂砾样的球状斑块,面部皱褶加深,可见针尖至粟粒大褐色丘疹,以口周为重。颈项部、前胸及后背正中线、四肢、腋窝密集分布多个褐色丘疹、斑块,部分融合成片,部分苔藓样变,可见散在抓痕、糜烂及结痂,皮损以四肢伸侧及胸背部正中线为著。头皮皮损组织病理检查示表皮灶状缺损,角化不全,见角化不良细胞,灶状颗粒层增厚,可见嗜碱性颗粒,灶状棘层松解。背部皮损组织病理检查示轻度角化过度,棘层肥厚,表皮突网状延伸,局部棘层松解形成裂隙,其下真皮浅层可见少许红染碎块状物沉积,刚果红染色(±),其余真皮上部小血管增生,血管周围淋巴细胞浸润,色素失禁。诊断:毛囊角化病并发回状颅皮。

A case of darier disease with cutis verticis gyrata is reported. A 46-year-old male presented with itchy papules and plaques on the scalp and neck for more than 30 years, and generalized skin lesions for more than 10 years. Physical ex amination showed deep gully-like scalp folds with grit-like globular plaques on the surface, deepened facial folds, and needletipto millet-sized brown papules with the perioral area as the focus. Densely distributed on the neck, front chest and midline of the back, limbs and armpits were multiple brown papules and plaques, some of which merged into pieces and some with mosslike changes. Scattered scratches, erosions, and scabs were seen, especially in the limbs and the midline of the chest and back. Histopathological examination of scalp lesions showed epidermal focal ulceration, erosive keratosis, dyskeratosis cells, focal granule layer thickening, basophilic granules, and focal spinous layer loosening. Histopathological examination of the skin lesions on the back showed mild hyperkeratosis, hypertrophic spine layer, reticulated extension of epithelial foot, fissures formed by partial spine layer loosening, a small number of red stained lumps deposited in the superficial dermis, congo red staining(± ), hyperplasia of small blood vessels in the upper part of the dermis, infiltration of peripheral lymphocytes, and incontinence of pigment. The diagnosis of darier disease combined with cutis verticis gyrate was made.