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An. Bras. Dermatol. vol.78 no.1 Rio de Janeiro Jan./Feb. 2003
Angiolymphoid hyperplasia with eosinophilia - report of two cases*
IDermatologist at the ICC, Fortaleza,
IIAssociate Professor and Head of Dermatology Service at UFC, Fortaleza, CE
IIIMasters student of Dermatology, UFRJ, Rio de Janeiro, RJ
IVAssociate Professor of Pathologic Anatomy at UFRJ, Rio de Janeiro, RJ
VAssociate Professor and Head of Dermatology Service at UFRJ, Rio de Janeiro, RJ
The authors present two cases of the relatively uncommon disease angiolymphoid hyperplasia with eosinophilia, which is usually located on the head and/or neck. One of the patients showed exuberant lesions and unusual site. Histopathology with HE and immunohistochemistry with factor VIII confirmed the diagnosis and the endothelial origin in both cases.
Keywords: eosinophilia; angiolymphoid hyperplasia with eosinophilia.
Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon vascular tumor with clinical and histopathologic characteristics that promptly permit its diagnosis.1 In 1969, when this disease was first described, Wells & Whimster 2 called it subcutaneous angiolymphoid hyperplasia with eosinophilia.
Solitary or multiple nodules or papules with an angiomatoid aspect characterize ALHE and its location is predominantly on the head, especially around the ears, forehead and scalp, and also the neck. Its origin is uncertain and it has been called epithelioid hemangioma, pyogenic pseudogranuloma, vascular angioplasia, atypical intravenous vascular proliferation and angiomatous inflammatory nodules. There is a frequent misdiagnosis with Kimura's disease.3-9
is a great variation in the histopathological findings of ALHE, it is probable
that, depending on the different stages of the disease and of the tissular reaction,6
it presents two main components:
1. vascular component, with marked proliferation of endothelial cells; and
2. cellular component, with intense infiltrate of lymphocytes, histiocytes and eosinophils.6,7
20-year-old black female, born and resident in Rio de Janeiro, noticed the onset of discretely pruritic papules on her right forearm. There was slow progression of the lesions for eight months before she sought medical advise at the Clementino Fraga Filho Hospital, of the Federal University of Rio de Janeiro.
The patient denied any past trauma on the site of the lesions, her past medical, physiologic, familial, and social history were inexpressive and there were no similar cases among her family or acquaintances. Blood eosinophils were normal in number.
At dermatological examination, a total of four nodular, round, erythemato-violaceous lesions, with 0.2 and 0.5 cm in diameter, were noticed on the anterior side of the right forearm. Some had a small central umbilication and were painful on palpation (Figures 1A, 1B and 2A).
A biopsy from one of these lesions (Figures 2B and 3A), was obtained and the histopathological examination showed a proliferation of blood vessels with various sizes, in a nodular arrangement, surrounded by an inflammatory infiltrate of lymphocytes and histiocytes, but mainly with a large number of eosinophils, in all extension of the dermis. These vessels had thickened walls composed of endothelial cells with large and eosinophilic cytoplasm, sometimes with vacuoles, protruding into the lumen. The diagnosis was ALHE. The immunohistochemical examination was positive for factor VIII, proving the endothelial origin of these cells (Figure 3B).
The patient was submitted to intralesional steroid injections with a good initial response. After six months, the lesions became resistant to this therapy and there was no more improvement. Cryosurgery with liquid nitrogen was then indicated with total disappearance of the lesions.
35-year-old white female, born and resident in Fortaleza, sought medical attention at the Walter Cantídio Hospital, of the Federal University of Cear´, because she noticed the onset of small pruritic violaceous nodules on and behind her left ear, which, she thought, began after insect bites at this site. The patient was not certain when the lesions began.
There were no significant problems in her past medical, physiologic, familial, and social history and she informed there were no members of her family or contacts with similar lesions. Eosinophils blood count was normal.
The dermatological exam showed two papulonodular, erythemato-violaceous lesions with angiomatoid aspect, on the superior portion of the left ear and left retroauricular region (Figure 4A).
The histopathological exam of the ear lesion (Figures 5A, 5B and 6A) revealed a dermal proliferation of irregular blood vessels, with thickened walls covered by dilated endothelial cells that projected into the lumen. These cells had an abundant eosinophilic cytoplasm and large nuclei. The dermis consisted of a fibrovascular stroma with lymphocytes, eosinophils and mastocytes. These characteristics are typical of ALHE and the immunohistochemical studies demonstrated positivity to the antigen related to factor VIII (Figure 6B).
The lesions were treated with an association of intralesional steroid and cryosurgery with liquid nitrogen (one session each three weeks). After six months of treatment, there was a partial involution of the larger lesion and total regression of the smaller one.
ALHE is a rare benign tumor that, in general, shows one or a few lesions on the head and neck of young adults.10 It grows slowly, is auto-limited and characterized by an intense endothelial cell proliferation.
It is believed that it is a malformation resulting from a subjacent arterio-venous shunt. Its pathogenesis is unknown, probably representing a true vascular neoplasia or a reactive cicatricial phenomenon after a variety of aggressions, such as trauma, infection, or humoral desequilibrium3-5 It is primarily a vascular process and its other alterations, as the presence of lymphoid follicles and eosinophils, seem secondary. Hyperestrogenemia states can be related to this disease.5,10
Our second patient attributed the onset of the nodules to insect bytes and it is known that deep biopsies reveal an arterio-venous shunt in a significant percentage of cases.4,5 Therefore, if we consider that ALHE represents a reactive hyperplastic process as a result of injury and reparation of a artery or vein,5 it is possible that insect bites can contribute to the development of the nodules.
There is great
confusion in the medical literature surrounding Kimura's disease and ALHE, names
used even as synonyms, and there are authors suggesting that Kimura's disease
is part of the spectrum of ALHE.5 Both have the tendency to produce
nodular lesions, involving primarily the head and neck, and also have the tendency
to persist. Kimura's disease shows normal skin color subcutaneous nodules while
ALHE presents reddish and/or violaceous nodules, reflecting its vascular nature.11
al.11 refer that the histopathological similarities are many,
as both can:
1. involve dermis and subcutaneous tissue;
2. have a infiltrate composed of lymphocytes and eosinophils;
3. have vessels paved by endothelial cells, which can be dilated and contain abundant cytoplasm;
4. have fibroplasia and many plasmatic cells; and
5. not affect epithelial and non-epithelial adnexal structures.
Its histopathological differential diagnosis is based on the following:11
1. does not
look like lymphoid tissue when observed under low magnification;
2. is predominantly a blood vessel disturbance;
3. shows structures similar to very dilated, abnormal veins, in the dermis and/or subcutaneous fat;
4. presents few or no lymphoid follicles;
5. has smooth muscle frequently on the walls of the structures similar to veins;
6. has mucin in abundance inside the walls of the structures similar to veins;
7. shows the dilated endothelial cells, some polygonal, projecting themselves deep into the lumen of the vessels;
8. commonly shows one of more vacuoles in the cytoplasm of the abnormal endothelial cells;
9. has a variation in the number of eosinophils from absent to numerous;
10. presents substitution of subcutaneous fat by fibrous tissue;
11. does not present fascia involvement.
1. is similar
to lymphoid tissue when observed under low magnification;
2. is predominantly a lymphoid follicle disturbance;
3. shows abnormal structures similar to veins without great dilatation;
4. presents many lymphoid follicles;
5. has no smooth muscle on the walls of the vessels;
6. has no mucin inside the walls of the vessels;
7. shows no projection of the endothelial cells into the lumen of the vessels;
8. shows no vacuoles in these endothelial cells;
9. eosinophils are always numerous;
10. shows frequent substitution of subcutaneous fat by fibrous tissue;
11. presents extension to fascia and sometimes even to the skeletal muscle.
The treatment of choice is surgical excision, although recurrences are frequent.6 Other therapeutic modalities have been proposed, among them: cryosurgery with liquid nitrogen, lasertherapy,12 local radiotherapy,13 aromatic retinoic acid,14 vvinblastine in patient with extensive cutaneous and mucous involvement,15 acitretin16 and/or intralesional steroids, especially in recurrent cases. Spontaneous remission may occur.5
The first patient, in whom there was recurrence of the lesions after the intralesional injection of steroid, did not show recurrence after cryosurgery for the following six months, and because of this, for her, this modality was a good therapeutic option. In the other patient, the association since the beginning of steroid and cryosurgery totally improved the smaller lesions but reduced only partially the larger lesion in the first six months of therapy. For this patient the possibility of complete surgical excision of the larger nodule is being considered, since larger lesions tend to persist unless the arterio-venous shunt is removed.17
We consider these are two very interesting cases because our first patient was a 20-year-old female presenting exuberant and pruritic lesions that by their aspect and location clinically mimic a keratoacanthoma. The fact that they were located on the right forearm is not consistent with the usual findings of ALHE. In our second patient also a female, the morphologic aspects, location and distribution, the presence of pruritus, gender and age of onset of the disease are in accordance with the findings of Wilson-Jones & Bleehen, in 1970,3 and of Olsen & Helwig, in 1985.5 The histopathology stained by HE and the immunohistochemistry with factor VIII confirmed the endothelial origin of the lesions in both patients. The fact that there was no eosinophilia does not invalidate the diagnosis, since this characteristic is found in not more than 10-15% of the cases.3,5
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Received in December, 12th of
Approved by the Consultive Council and accepted for publication in May, 22th of 2002.
* Work done at a "Serviço de Dermatologia e Curso de Pós-Graduação em Dermatologia", "Faculdade de Medicina e HUCFF-UFRJ", "Universidade Federal do Rio de Janeiro"; b "Instituto do Câncer do Ceará"; c "Serviço de Dermatologia, Faculdade de Medicina", "Universidade Federal do Ceará"; e d "Departamento de Anatomia Patológica", "Faculdade de Medicina e HUCFF-UFRJ", "Universidade Federal do Rio de Janeiro".