ARTICLE
A 13-month-old boy was hospitalized in our ward with the presence of
erythematous-purpuric lesions, roundish in shape, not itchy, 1/2 to 6-7
cm in diameter, localized on the face, ears and lower limbs (Fig.
1a, b). His history revealed that the eruption developed suddenly
the previous week and was not followed by infection of the upper respiratory
tract or by the use of any drug. During hospitalization, a mild fever
and diarrhea appeared. The overall general condition was good.
The haematological parameters were within normal limits, and white cell
count was the following: leukocytes 7,000 with 31.4% neutrophils, 54.6%
lymphocytes, 7.8% monocytes, 4.3% eosinophils, 0.8% basophils; erythrocyte
sedimentation rate 26 mm/h; coagulation tests normal. Electrophoresis:
64.8% albumin, 4.8% *1, 12.2% *2, 11.1%ß, 7.1% *. Immunoglobulin
count: 405 mg/dl IgG, 27 mg/dl IgA, 83 mg/dl IgM. Stool examination showed
only a small number of colonies of Candida Albicans, while salmonella
and other faecal parasites were absent. The tests for anti-nuclear, anti-liver,
anti-heart, anti-gastric parietal cells, and anti-thyroid antibodies were
negative.
Skin biopsy specimens of a lesion of the lower limb revealed acanthosis,
focal spongiosis and parakeratosis; the papillary dermis was characterized
by a modest lymphohistiocytic perivascular infiltrate with scattered eosinophils.
Furthermore, limited extravasation of erythrocytes was present (Fig.
2). Immunofluorescence findings were negative for deposits of
IgG, IgA, IgM and C3. Urinanalysis was normal.
Two weeks after admission, the disease resolved without any sequelae.
Comments
Acute haemorrhagic oedema (AHO) of the skin is a cutaneous disorder
that almost exclusively affects children under two years of age. The course
of the disease is acute and the evolution is benign, with resolution occurring
spontaneously within one to three weeks. First described by Snow in 1913
[1] and subsequently by Finkelstein in 1938 [2], AHO is a rare disease.
The majority of cases have been reported by French authors, because for
many years the English-language authors regarded this pathology as an
analogue of Schönlein-Henoch purpura occurring in the very young,
and not as an individual pathology with its own clinical entity [3].
AHO is characterized by the appearance of erythematous-edematous purpuric
lesions, typically cockade or "rosette" in appearance and localized mainly
on the face and limbs. Systemic involvement is very rare and the coagulation
values are within normal limits.
The cause of the AHO is, unfortunately, still unknown. The use of drugs,
vaccinations, respiratory infections have all, in turn, been hypothesized
[4]. Immunological pathogenesis was postulated by Lambert in 1979 [5],
and immunological tests sometimes show the presence and sometimes the
absence of circulating immune-complexes with normal complement levels.
The most striking feature of this disease is the contrast between the
acuteness of the cutaneous signs, which are typical and unmistakable,
and the general condition of the patient, which is good [6]. Visceral
involvement and death is a rare exception [7] and usually there is complete
resolution in two to three weeks.
The histopathological examination is very rarely performed and the results
vary from a typical leukocytoclastic vasculitis with or without fibrinoid
necrosis to less specific findings (for example, lymphohistiocytic perivascular
infiltrate with extravasation of erythrocytes) [8]. Immunofluorescence
may or may not reveal fibrinogen and C3, IgA and IgM in and around the
dermal vessels [6-9].
In our case, the patient's age, the absence of haematuria, visceral
and mucous involvement and the good general condition excluded Schöenlein-Henoch's
disease and multiform erythema. The absence of pruritus and the haphazard
distribution of the cutaneous lesions excluded urticaria, while the absence
of antinuclear antibodies made diagnosis of neonatal lupus improbable.
Furthermore, the patient's age, the presence of typical cockade cutaneous
lesions, the benign evolution and the histological findings were all typical
of AHO [10].
Cases like this one, presenting no clear-cut vasculitis but only less
specific histological findings, should be borne in mind in order to better
understand the disease and to diagnose it correctly. This is essential
for preventing the use of drugs which will not modify the evolution of
the dermatitis, which is in any case benign.
References
1. Snow IM. Purpura, urticaria and angioneurotic edema of the
hands and the feet in a nursing baby. JAMA 1913; 61: 18-9.
2. Finkelstein H. Lehrbuch der Säuglingskrankheiten.
4th ed. Amsterdam, 1938: 814-30.
3. Amitai Y, Gillis D, Wasserman D, Kochman RH. Henoch-Schonlein
purpura in infants. Pediatrics 1993; 92: 865-7.
4. Gelmetti C, Barbagallo C, Cerri D, et al. Acute hemorrhage
edema of the skin in infants: clinical and pathogenic observations in
seven cases. Pediatr Dermatol News 1985; 4: 23-34.
5. Lambert D, Laurent R, Bouilly D, et al. Œdème
aigu hémorragique du nourrisson. Données immunologiques
et ultrastructurales. Ann Dermatol Venereol 1979; 106: 975-87.
6. Legrain V, Lejean S, Taïeb A, et al. Infantile
acute hemorrhagic edema of the skin. Study of ten cases. J Am Acad
Dermatol 1991; 24: 17-22.
7. Larrègue M, Lorette G, Prigent F, et al. Œdème
aigu hémorragique du nourrisson avec complication léthale
digestive. Ann Dermatol Venereol 1980; 107: 901-5.
8. Ince E, Mumcu Y, Suskan E, Yalcinkaya F, Tumer N, Cin S. Infantile
acute hemorrhagic edema: a variant of leukocytoclastic vasculitis. Pediatr
Dermatol 1995; 12: 224-7.
9. Larrègue M, Laesage B, Rossiner A. Edema agudo hemorragico
del lactante (EAHL) (purpura en escarapela con edema post-infeccioso de
Seidemayer) y vascularitis alergica. Med Cutanea 1974; 11: 165-74.
10. Saraçlar Y,Tinaztepe K, Adahoglu G, Tuncer A. Acute
hemorrhagic edema of infancy (AHEI): a variant of Henoch-Schönlein
purpura or a distinct clinical entity? J Allergy Clin Immunol 1990;
86: 473-83.
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