壞疽性膿皮症：特發群、副發炎群及血液疾病相關群的比較-十三例病例回顧分析-

Pyoderma Gangrenosum: A Comparison of the Idiopathic Group, the Parainflammatory Group, and those Associated with Hematological Disorders -Review of 13 Cases-

王佳珍(Chia-Chen Wang) ； 何信君(Hsin-Chun Ho) ； 曾興隆(Heng-Leong Chan)

《中華皮膚科醫學雜誌》 20卷4期 (2002/12) Pp. 271-279

https://doi.org/10.29784/DS.200212.0002

摘要

壞疽性膿皮症(PG)是一種罕見且原因不明的潰瘍性皮膚疾病。約50%的病例伴隨有相關疾病，其中以關節炎和發炎性腸疾最為常見。本研究回溯1987至2001年共13例(6例男性，7例女性)壞疽性膿皮症之病例，並著重於特發群(IPG)，副發炎群(PPG)，及血液疾病相關群(HPG)的臨床表現及病程的比較。在這些病例中，有5例屬顧IPG，4例(3例關節炎，1例潰瘍性結腸炎)為PPG，5例(4例IgA單源性伽瑪球蛋白病變，1例骨髓增生疾病)為HPG。腿部為好發部位。以病灶數而論，其中3例為單一病灶，4例有2至5個，其餘則多於5個病灶。長期追蹤顯示有4例只發作過一次，其餘病例則有次數不等的復發。有3例合併單源性伽瑪球蛋白病變之病患其皮膚病灶從未完全癒合。2例病例(皆屬HPG)死於肺炎。本研究顯示IgA單源性伽瑪球蛋白病變可能是台灣最常見之壞疽性膿皮症相關疾病。IPG和PPG其皮膚病灶數量，癒合所需時間，及復發率皆無顯著差距。HPG的預後可能較差。

並列摘要

Pyoderma gangrenosum (PG) is an uncommon, ulcerated cutaneous disorder of unknown etiol-
ogy. Approximately 50% of the patients have associated diseases, among which arthritis and inflame-
matory bowel disease are the most common. We retrospectively review 13 patients (6 male, 7 female)
of PG from 1987 to 2001 with an emphasis on the comparison of clinical presentations and courses of
the idiopathic group (IPG), the parainflammatory group (PPG) and those associated with hematologi-
cal disorders (HPG). Among these patients, 5 patients belong to IPG, 4(3 arthritis, 1 ulcerated colitis)
to PPG, and 5 (4 IgA monoclonal gammopathy, 1 myeloproliferative disorder) to HPG. The predilec-
tion sites are legs. Three patients had 1 lesion, 4 had 2-5 lesions, and the others had more than 5
lesions. Long-term follow-up revealed that 4 patients had only single attack, but others experienced
varied recurrences. Three patients with monoclonal gammopathy never healed completely. Two
patients (all HPG) expired due to pneumonia. Our study suggests that IgA monoclonal gammopathy is
probably the most common associated disease of PG in Taiwan, and no significant difference between
IPG and PPG in terms of lesion number, healing time and relapsing frequency. HPG possibly had a
poor prognosis.

並列關鍵字

Pyoderma gangrenosum ； Ulcerative colitis ； Arthritis ； Monoclonal gammopathy ； Myeloproliferative disorder