Fernandez-Flores Angel, Cassarino David S
Department of Cellular Pathology, CellCOM-SB Group, Servicio de Anatomía Patologica, Hospital El Bierzo, Cellular Pathology, Ponferrada, Spain.
Department of CellCOM-SB Group, Biomedical Investigation Institute of A Coruña, CellCOM-SB Group, A Coruña, Spain.
Am J Dermatopathol. 2017 Dec;39(12):924-927. doi: 10.1097/DAD.0000000000000937.
The histopathological features of cutaneous hyperpigmentation in Addison disease have very occasionally been reported, and they include acanthosis, hyperkeratosis, focal parakeratosis, spongiosis, superficial perivascular lymphocytic infiltrate, basal melanin hyperpigmentation, and superficial dermal melanophages. We present a study on 2 biopsies from the arm and the thigh in a 77-year-old woman with a long clinical history of Addison disease as well as senile purpura and alopecia of female pattern. The patient presented diffuse hyperpigmentation of the skin, more pronounced on her face, and left upper forehead. The skin biopsies showed no remarkable dermal inflammatory infiltrate with melanocytic hyperpigmentation of the basal layer of the epidermis as well as a mild amount of melanophages in the papillary dermis. In addition, we found lipofuscin in the luminal pole of the secretory epithelium of the eccrine glands. In the perieccrine areas, there was Perls-positive pigment in the cytoplasm of macrophages most likely related to the senile purpura. An immunohistochemical study with Melan-A showed a melanocyte/keratinocyte ratio of 1:20 (5%) in the arm and of less than 1:50 (only 2 melanocytes in the whole section; <2%) in the thigh.
Addison病皮肤色素沉着的组织病理学特征偶尔有报道，包括棘层肥厚、角化过度、局灶性角化不全、海绵形成、浅表血管周围淋巴细胞浸润、基底黑色素沉着以及浅表真皮黑素细胞。我们对一名77岁女性的手臂和大腿进行了2次活检研究，该女性有Addison病、老年性紫癜和女性型脱发的长期临床病史。患者出现皮肤弥漫性色素沉着，面部和左上额更为明显。皮肤活检显示真皮无明显炎症浸润，表皮基底层有黑素细胞色素沉着，乳头真皮有少量黑素细胞。此外，我们在小汗腺分泌上皮的管腔极发现了脂褐素。在小汗腺周围区域，巨噬细胞胞质中有Perls阳性色素，很可能与老年性紫癜有关。用Melan - A进行的免疫组织化学研究显示，手臂的黑素细胞/角质形成细胞比例为1:20（5%），大腿的比例小于1:50（整个切片中只有2个黑素细胞；<2%）。
