Chicken-wire erythema, but not urticaria, as the presenting sign of hereditary angioedema

ARTICLE

Chicken-wire erythema, but not urticaria, as the presenting sign of hereditary angioedema

Auteur(s) : Deniz YUCELTEN¹, Sadiye KUS²

¹ Department of Dermatology, Marmara University, Medical Faculty, Istanbul, Turkey
² Department of Dermatology, Acibadem Hospital, Istanbul, Turkey
<skus@asg.com.tr>
<sadiyekus@superonline.com>

Hereditary angioedema (HAE) is a distinctive form of recurrent angioedema which is due to extravasation of plasma into deep cutaneous or mucosal layers, as a result of excessive amounts of C2 kinin and bradykinin [1, 2]. It is an autosomal dominant disease characterized by a quantitative or functional defect of the c1 esterase inhibitor (C1 INH) [3]. Angioedema without urticaria is the hallmark of HAE [2]. The cutaneous presentation of HAE is nonpitting and nonerythematous skin edema with ill-defined margins affecting the face, extremities and genitals. Recurrent abdominal pain caused by gastrointestinal wall edema and life threatening upper airway involvement may also be present [2, 3]. A prodromal rash may precede the angioedema [4, 5].
A 23-year-old man presented to our clinic with a 12 year history of attacks of truncal erythema, hand and feet swelling and concomittant abdominal pain recurring one or two times per month. On physical examination he had nonerythematous and non pitting edema with ill-defined margins on the dorsal side of both hands and a reticulate, serpentine, macular erythema on the trunk, mimicking chicken-wire appearance (figure 1). Complete blood count with differential and blood chemistry was unremarkable. Upper gastrointestinal endoscopy and abdominal ultrasonogrophy were normal. The laboratory values of complement levels were as follows: C4: < 5.7 (N:10-40), C2: 44 (N:80-120), CH50: < 10 (N:80-120), C1INH: 6.37 (N:16-33), repeat C1INH level (in a different laboratory): 0.06 (N:0.15-0.35). When investigated, his 2 year old son, who had recurrent abdominal pain, was also found to have low C1INH level: 5.7 (N:16-33).
Presence of angioedema without urticaria, low levels of C2, CH50, and C1INH, early presentation of the symptoms, absence of an associated disease, and existence of a family member with low C1INH levels accompanying recurring abdominal pain enabled us to diagnose hereditary angioedema in our patient.
Danazole 400 mg/day was started and then reduced to 200 mg. The patient has been followed for four years during which he experienced infrequent mild attacks without preceding reticular erythema.
In 26% to 50% of patients, an asymptomatic, mild and transient, non-urticarial figurated erythema on the trunk (most commonly) or extremities precedes the typical symptoms of HAE [5, 6]. As in our patient, this prodromal erythema enables the early recognition of an attack by the patient. The figurated erythema usually starts in childhood and may be observed in other family members of the patient [4]. A flat, ring-shaped, erythematous lesion enlarges and produces polycyclic serpiginous or reticulate «chicken-wire» eruption [4, 6, 7]. The border of the erythema may be slightly raised and so called «erythema marginatum». It usually resolves with the onset of the attack [4]. The exact pathogenetic mechanism in its development is not known. Conventional histopathology shows minimal abnormalities [8]. Heavy deposition of bradykinin in the dermal stromal tissue and endothelial cells, suggests that bradykinin may be important in its development [5].
We report this case as clinicians should consider the diagnosis of hereditary C1INH deficiency, a rare and seldom fatal disease, in the setting of recurrent angioedema with concurrent abdominal pain sometimes preceded by a characteristic prodromal eruption «chicken wire erythema». It is crucial to recognise this disease in order to avoid triggering factors or to provide prophylaxis before minor and major procedures. n

References

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