Hubiche T, Boralevi F, Jouvencel P, Taïeb A, Leaute-Labreze C
Unité de Dermatologie pédiatrique, Hôpital Pellegrin-Enfants, Place Amélie Raba-Léon, 33076 Bordeaux Cedex.
Ann Dermatol Venereol. 2005 Mar;132(3):249-51. doi: 10.1016/s0151-9638(05)79255-3.
Hereditary angioedema is characterized by episodes of subcutaneous, digestive or laryngeal edema. In some cases, non-pruritic reticular erythema may precede the episodes of edema.
Every 4 to 6 weeks since infancy, a girl presented non-pruritic widespread reticular erythema, sparing the face. Two or three times every year, abdominal pain or edema of the lower limb joints followed the skin eruption. At 12 years of age, she was hospitalized because of an edema of the face associated with the eruption. Exploration of the complement confirmed the diagnosis of type I hereditary angioedema.
The mean delay before diagnosis of hereditary angioedema is of 7 years. Reticular erythema in hereditary angioedema is frequent (40p. 100 of cases) and it usually occurs early in childhood, even in the absence of any episode of angioedema. When present, these eruptions usually precede an episode of angioedema. Recognition of this eruption as a symptom of hereditary angioedema would shorten the delay before diagnosis and anticipate appropriate management of the episodes.
遗传性血管性水肿的特征是皮下、消化道或喉部出现水肿发作。在某些情况下，非瘙痒性网状红斑可能先于水肿发作出现。
自婴儿期起，一名女孩每4至6周就会出现一次非瘙痒性的广泛网状红斑，面部未受累。每年有两三次，皮肤出疹后会出现腹痛或下肢关节水肿。12岁时，她因面部水肿伴发疹而住院。补体检查确诊为I型遗传性血管性水肿。
遗传性血管性水肿的平均诊断延迟时间为7年。遗传性血管性水肿中的网状红斑很常见（占病例的40%），通常在儿童早期出现，即使没有任何血管性水肿发作。当出现这些皮疹时，通常会先于血管性水肿发作。将这种皮疹识别为遗传性血管性水肿的症状将缩短诊断前的延迟时间，并对发作进行适当的处理。
