Ctmicat and Experimentat Dermatology 1990; 15: 65-67,
Angiolymphoid hyperplasia with eosinophilia limited to the vulva A.AGUILAR, P.AMBROJO, L.REQUENA, L.OLMOS AND E . S A N C H E Z Y\JS Department of Dermatology, Hospital Clinico de San Carlos, Universidad Complutense de Madrid, Spain Accepted for publication 2% June 1989
There was no evidence of previous injury to the site and the family and personal history were not contributory. A patient with angiolymphoid hyperplasia with eosinoThe patient was started on topical applications of philia is described. This is, to our knowledge, the first corticosteroid cream which produced no response. case report in which the lesions were located on the vulva. On physical examination, there were three red-purple vascular, painless lesions located on the external aspect of Angiolymphoid hyperplasia with eosinophilia (ALHE) is the labia majora (Fig. 1). The lesions ranged in size from a a benign, uncommon skin disease characterized clinically 2-mm slightly raised papule to 8-mm nodular domeby single or multiple intraderma! or subcutaneous shaped lesions which were compressible and emptied nodules located mainly on the head or neck region of partially on diascopy. There was no regional lymphadeyoung adults. Histologically, the striking features are nopathy and no relevant lesions were seen in other areas. proliferation of anomalous vessels with plump endothelial The remainder of the general medical examination was cells associated with a variable degree of inflammatory normal. infiltrate containing lymphocytes and eosinophils. The following laboratory studies were normal: full In 1969, Wells and Whimstcr' published a series of blood counts, erythrocyte sedimentation rates, scrum nine patients which they referred to under the title calcium, glucose, cholesterol, creatinine, uric acid, alka'subcutaneous angiolymphoid hyperplasia with eosino- line phosphatase, lactic dehydrogcnase, SGOT, SGPT, philia' but some isolated cases nf atypical cutaneous serum protein electrophorcsis and urinalysis. Chest Xvascular disorders with endothelial proliferation had been ray was within normal limits. described previously.^' Since the first description, An excisional biopsy was taken from the nodule. Two further examples of ALHE have been reported under a components, vascular and inflammatory, were observed variety of names.^ ' The relationship between ALHE and throughout the dermis under a slightly acanthotic epiderthe so-called Kimura's disease^ is a matter of controversy. mis. The degree of involvement of these two components Nevertheless, recent reports''"' have suggested that these varied from one area to another within the lesion. entities are two different diseases. Clinical and histopathological features of ALHE have been reviewed extensively in recent years'* " and a critical appraisal of the literature does not provide well-documented cases in which the lesions were located on the vulva. We describe herein a unique case with clinical and histopathological changes characteristic of ALHE with vulvar involvement only. Summary
Case Report A 27-year-old woman consulted our clinic with a 4month history of severe, continuous vulvar pruritus.
Correspondence: Antonio Aguilar Martinez, Sesena 13, 5 2X024-Madrid, Spain.
Figure I. Three well-defined, smooth-surfaced, raised lesions located on the lahia majora.
65
66
A.AGUILAR fA a/.
Figure 2. Phoiomicrograph dcmon.strating a vascular .space lined hy plump endothelial cells which show intracytnplasniic vacuoles. Collagen fibres are disposed in concentric layers around the vascular space (H&E x 290).
The vascular component was composed of a proliferation of endothelial cells arranged in solid nests and cords or forming vascular spaces of different randomly distributed thickness. These vascular spaces showed irregular and branching lumina and were lined by plump endothelial cells which protruded into the lumen. Some of these cells showed intracytoplasmic vacuoles. Usually the collagen fibres tended to form concentric layers around the lumina {Fig. 2). No elastic fibres were found in these areas. Abnormal mitoses or cellular atypia were not observed. The second component was a mild, diffuse inflammatory infihrate composed of lymphocytes, eosinophils and plasma cells in varying numbers which also surrounded the vascular structures. Germinal centres were not observed. After the excisional biopsy of the largest lesion, pruritis almost completely subsided and the patient refused further biopsy or treatment. Discussion Clinically, ALHE appears as solitary or multiple papules or nodules commonly situated on the head and neck region. Occasionally, trunk and extremities have been involved and there are isolated reports in which the lesions could be observed in the oral mucosa,'^ '^ the orbit and ocular area,'"* inguinal folds and penis^ as well as widespread lesions over the skin.'^ Nevertheless, to our knowledge, no vulvar lesions have been described previously. The lesions vary in size from a few millimetres to several centimetres and there is no correlation between size and location. Young adults are most frequently affected'' " with a slight predominance in females. From a histopathological viewpoint, a recent survey" has confirmed that ALHE shows a distinctive histopathology: vascular proliferation of thick- and thin-walled
blood vessels lined by prominent endothelial cells with vacuolated cytoplasm protruding into the vascular lumen. These endothelial cells appear to be arranged in solid nests and cords. The vascular component is associated to a varying degree with a diffuse inflammatory infiltrate composed oflymphocytes, histiocytes and eosinophils. In some isolated cases lymphoid follicles with germinal centres are formed. Other prominent features are cutaneous artero-venous (A-V) shunt formation and intravascular endothelial proliferation in some large vessels.'""' The degree of involvement of both vascular and inflammatory components varies with the depth of the lesion. Those lesions that are situated in subcutaneous tissue showed more intravascular endothelial proliferation and A-V shunting that dermal lesions, whereas in the dermal lesions, as in our case, the vascular proliferation is the most obvious feature with less inflammatory infiltrate. The origin of ALHE is not clearly elucidated. Nevertheless, several reports have shown that different events such as infection, trauma or pregnancy precede the onset of the lesions.'"'^ Moreover, in a great number of these cases, a common histological feature was the formation of an A-V shunt and intravascular proliferation. These findings suggest that ALHE is a benign reactive process and the inflammatory infiltrate appears secondary to the va.scular proliferation.'' Different treatment modalities such as surgical excision, intralesional steroids, superficial X-ray, etretinate,'^ intravenous vinblastine sulphate'^ or carbon-dioxide laser vaporization''' have been employed with a variable degree of success. Nevertheless, in a great number of cases, the disease is episodic although spontaneous remissions arc known to occur. References 1. Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. British Journat of Dermatology 1969; 81: 1-15. 2. Winer LH, Levin GH. Acquired vascular tumors of the skin in the adult: a report of five unusual cases. Archives of Dermatotogy 1959; 79: 17 31. 3. Peterson WC Jr, Fusaro RM, Goltz RW. Atypical pyogenic granuloma. A case of benign hemangioendotheliosis. Archives of Dermatotogy 1964; 90: 197-201. 4. Wilson-Jones K, Marks R. Papular angioplasia. \ ascular papules of face and scalp simulating malignant vascular tumors. Archives of Dermatology 1970; 102: 422-427. 5. Mehregan AH, Shapiro L. Angiolymphoid hyperplasia with eosinophilia. .-trchives of Dermatotogy 1971; 103: 50-57. 6. Rt)sai J, Ackerman LR. Intravenous atypical vascular proliferation. .\ cutaneous lesion simulating a malignant blood vessel tumor. Archives of Dermatotogy 1974; 109: 714 717. 7. Gnmez-Orhaneja J, Sanchez Yus K. Hyperplasie angiolymphoide avec eosinophilie. Annales de Dermatotogie et de Syphitigraphie 1974; 101:408-409. 8. Kawada A, Takahashi H, Anzai T. Eosinophilic lymphofolliculosisofthe skin (Kimura's dmtzse). Japanese Journat of Dermatotogy 1966; 76: 61-72.
VULVAL ANGIOLYMPHOID HYPERPLASIA 9. Atsumichi U, .Masazumi T, Mutenomo E. Epithelioid Hemangioma versus Kimura's disease. A comparative clinicopathologic study. American Journat of Surgical Pathotogy 1987; II: 758-766. UI. Googe PB, Harris HL, Mihm MC Jr. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities. Journat of Cutaneous Pathology 1987; 14: 263 271. 11. Olsen TG, Helwig EB. Angiolymphoid hyperplasia with cnsinophilia. A clinicopathologic study of 116 patients. Journat of the .-imerican Academy of Dermatutogy 1985; 12: 781 796. 12. Biichner .'\, Silverman S, Wara WM, I lansen LS. Angiolymphoid hyperplasia with eosinophilia (Kitnura's disease). Oral Surgery 1980; 49: 309 313. 13. Boisnic S, Frances C, Bletry O, Franceschini P, Chomette G. Un cas d'hyperplasic angiolymphoide avec eosinophilie (HALE) a dehut muqueux. .innates de Dermatologie et de I enereotogie 1987;
114: 561-566. 14. 1 lidayat AA, Cameron JD, Zimmerman LE. Angiolymphoid hyperplasia with eosinophilia (Kimura's disease) of the orhit and
15. 16. 17. 18.
19.
67
ocular anexa. American fournat of Ophthatmotogy 1983; 96: 176189. Sarnoff DS, SchifT GM. Widespread cutaneous angiolymphoid hyperplasia with eosinophilia. Journat of Dermatotogie Surgery and Oncotogy 1983; 9: 905 909." Reed RJ, Terazakis N. Subcutaneous angiohlastic lymphoid hyperplasia with eosinophilia (Kimura's disease). Cancer 1972; 29:489-497. Menz J, Su WDP. Angiolymphoid hyperplasia with eosinophilia: .Monoclonal leukocyte antihody studies in two cases. Archives of Dermatotogy 1987; 123: 866 867. Massa MC, Fretzin DF, Chowhury L, Sweet DL Angiolymphoid hyperplasia demonstrating extensive skin and mucosal lesions controlled with vinblastine therapy. Journat of the American Academy of Dermatology 1984; 11: 3'33-339. Hohhs ER, Bailin PL, Ratz J[., Varhrough CL. Treatment of angiolymphoid hyperplasia of the external ear with carbon dioxide laser. Journal of the American Academy oJ Dermatology 1988; 19: 345 349.
Angiolymphoid hyperplasia with eosinophilia limited to the vulva A.AGUILAR, P.AMBROJO, L.REQUENA, L.OLMOS AND E . S A N C H E Z Y\JS Department of Dermatology, Hospital Clinico de San Carlos, Universidad Complutense de Madrid, Spain Accepted for publication 2% June 1989
There was no evidence of previous injury to the site and the family and personal history were not contributory. A patient with angiolymphoid hyperplasia with eosinoThe patient was started on topical applications of philia is described. This is, to our knowledge, the first corticosteroid cream which produced no response. case report in which the lesions were located on the vulva. On physical examination, there were three red-purple vascular, painless lesions located on the external aspect of Angiolymphoid hyperplasia with eosinophilia (ALHE) is the labia majora (Fig. 1). The lesions ranged in size from a a benign, uncommon skin disease characterized clinically 2-mm slightly raised papule to 8-mm nodular domeby single or multiple intraderma! or subcutaneous shaped lesions which were compressible and emptied nodules located mainly on the head or neck region of partially on diascopy. There was no regional lymphadeyoung adults. Histologically, the striking features are nopathy and no relevant lesions were seen in other areas. proliferation of anomalous vessels with plump endothelial The remainder of the general medical examination was cells associated with a variable degree of inflammatory normal. infiltrate containing lymphocytes and eosinophils. The following laboratory studies were normal: full In 1969, Wells and Whimstcr' published a series of blood counts, erythrocyte sedimentation rates, scrum nine patients which they referred to under the title calcium, glucose, cholesterol, creatinine, uric acid, alka'subcutaneous angiolymphoid hyperplasia with eosino- line phosphatase, lactic dehydrogcnase, SGOT, SGPT, philia' but some isolated cases nf atypical cutaneous serum protein electrophorcsis and urinalysis. Chest Xvascular disorders with endothelial proliferation had been ray was within normal limits. described previously.^' Since the first description, An excisional biopsy was taken from the nodule. Two further examples of ALHE have been reported under a components, vascular and inflammatory, were observed variety of names.^ ' The relationship between ALHE and throughout the dermis under a slightly acanthotic epiderthe so-called Kimura's disease^ is a matter of controversy. mis. The degree of involvement of these two components Nevertheless, recent reports''"' have suggested that these varied from one area to another within the lesion. entities are two different diseases. Clinical and histopathological features of ALHE have been reviewed extensively in recent years'* " and a critical appraisal of the literature does not provide well-documented cases in which the lesions were located on the vulva. We describe herein a unique case with clinical and histopathological changes characteristic of ALHE with vulvar involvement only. Summary
Case Report A 27-year-old woman consulted our clinic with a 4month history of severe, continuous vulvar pruritus.
Correspondence: Antonio Aguilar Martinez, Sesena 13, 5 2X024-Madrid, Spain.
Figure I. Three well-defined, smooth-surfaced, raised lesions located on the lahia majora.
65
66
A.AGUILAR fA a/.
Figure 2. Phoiomicrograph dcmon.strating a vascular .space lined hy plump endothelial cells which show intracytnplasniic vacuoles. Collagen fibres are disposed in concentric layers around the vascular space (H&E x 290).
The vascular component was composed of a proliferation of endothelial cells arranged in solid nests and cords or forming vascular spaces of different randomly distributed thickness. These vascular spaces showed irregular and branching lumina and were lined by plump endothelial cells which protruded into the lumen. Some of these cells showed intracytoplasmic vacuoles. Usually the collagen fibres tended to form concentric layers around the lumina {Fig. 2). No elastic fibres were found in these areas. Abnormal mitoses or cellular atypia were not observed. The second component was a mild, diffuse inflammatory infihrate composed of lymphocytes, eosinophils and plasma cells in varying numbers which also surrounded the vascular structures. Germinal centres were not observed. After the excisional biopsy of the largest lesion, pruritis almost completely subsided and the patient refused further biopsy or treatment. Discussion Clinically, ALHE appears as solitary or multiple papules or nodules commonly situated on the head and neck region. Occasionally, trunk and extremities have been involved and there are isolated reports in which the lesions could be observed in the oral mucosa,'^ '^ the orbit and ocular area,'"* inguinal folds and penis^ as well as widespread lesions over the skin.'^ Nevertheless, to our knowledge, no vulvar lesions have been described previously. The lesions vary in size from a few millimetres to several centimetres and there is no correlation between size and location. Young adults are most frequently affected'' " with a slight predominance in females. From a histopathological viewpoint, a recent survey" has confirmed that ALHE shows a distinctive histopathology: vascular proliferation of thick- and thin-walled
blood vessels lined by prominent endothelial cells with vacuolated cytoplasm protruding into the vascular lumen. These endothelial cells appear to be arranged in solid nests and cords. The vascular component is associated to a varying degree with a diffuse inflammatory infiltrate composed oflymphocytes, histiocytes and eosinophils. In some isolated cases lymphoid follicles with germinal centres are formed. Other prominent features are cutaneous artero-venous (A-V) shunt formation and intravascular endothelial proliferation in some large vessels.'""' The degree of involvement of both vascular and inflammatory components varies with the depth of the lesion. Those lesions that are situated in subcutaneous tissue showed more intravascular endothelial proliferation and A-V shunting that dermal lesions, whereas in the dermal lesions, as in our case, the vascular proliferation is the most obvious feature with less inflammatory infiltrate. The origin of ALHE is not clearly elucidated. Nevertheless, several reports have shown that different events such as infection, trauma or pregnancy precede the onset of the lesions.'"'^ Moreover, in a great number of these cases, a common histological feature was the formation of an A-V shunt and intravascular proliferation. These findings suggest that ALHE is a benign reactive process and the inflammatory infiltrate appears secondary to the va.scular proliferation.'' Different treatment modalities such as surgical excision, intralesional steroids, superficial X-ray, etretinate,'^ intravenous vinblastine sulphate'^ or carbon-dioxide laser vaporization''' have been employed with a variable degree of success. Nevertheless, in a great number of cases, the disease is episodic although spontaneous remissions arc known to occur. References 1. Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. British Journat of Dermatology 1969; 81: 1-15. 2. Winer LH, Levin GH. Acquired vascular tumors of the skin in the adult: a report of five unusual cases. Archives of Dermatotogy 1959; 79: 17 31. 3. Peterson WC Jr, Fusaro RM, Goltz RW. Atypical pyogenic granuloma. A case of benign hemangioendotheliosis. Archives of Dermatotogy 1964; 90: 197-201. 4. Wilson-Jones K, Marks R. Papular angioplasia. \ ascular papules of face and scalp simulating malignant vascular tumors. Archives of Dermatology 1970; 102: 422-427. 5. Mehregan AH, Shapiro L. Angiolymphoid hyperplasia with eosinophilia. .-trchives of Dermatotogy 1971; 103: 50-57. 6. Rt)sai J, Ackerman LR. Intravenous atypical vascular proliferation. .\ cutaneous lesion simulating a malignant blood vessel tumor. Archives of Dermatotogy 1974; 109: 714 717. 7. Gnmez-Orhaneja J, Sanchez Yus K. Hyperplasie angiolymphoide avec eosinophilie. Annales de Dermatotogie et de Syphitigraphie 1974; 101:408-409. 8. Kawada A, Takahashi H, Anzai T. Eosinophilic lymphofolliculosisofthe skin (Kimura's dmtzse). Japanese Journat of Dermatotogy 1966; 76: 61-72.
VULVAL ANGIOLYMPHOID HYPERPLASIA 9. Atsumichi U, .Masazumi T, Mutenomo E. Epithelioid Hemangioma versus Kimura's disease. A comparative clinicopathologic study. American Journat of Surgical Pathotogy 1987; II: 758-766. UI. Googe PB, Harris HL, Mihm MC Jr. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two distinct histopathological entities. Journat of Cutaneous Pathology 1987; 14: 263 271. 11. Olsen TG, Helwig EB. Angiolymphoid hyperplasia with cnsinophilia. A clinicopathologic study of 116 patients. Journat of the .-imerican Academy of Dermatutogy 1985; 12: 781 796. 12. Biichner .'\, Silverman S, Wara WM, I lansen LS. Angiolymphoid hyperplasia with eosinophilia (Kitnura's disease). Oral Surgery 1980; 49: 309 313. 13. Boisnic S, Frances C, Bletry O, Franceschini P, Chomette G. Un cas d'hyperplasic angiolymphoide avec eosinophilie (HALE) a dehut muqueux. .innates de Dermatologie et de I enereotogie 1987;
114: 561-566. 14. 1 lidayat AA, Cameron JD, Zimmerman LE. Angiolymphoid hyperplasia with eosinophilia (Kimura's disease) of the orhit and
15. 16. 17. 18.
19.
67
ocular anexa. American fournat of Ophthatmotogy 1983; 96: 176189. Sarnoff DS, SchifT GM. Widespread cutaneous angiolymphoid hyperplasia with eosinophilia. Journat of Dermatotogie Surgery and Oncotogy 1983; 9: 905 909." Reed RJ, Terazakis N. Subcutaneous angiohlastic lymphoid hyperplasia with eosinophilia (Kimura's disease). Cancer 1972; 29:489-497. Menz J, Su WDP. Angiolymphoid hyperplasia with eosinophilia: .Monoclonal leukocyte antihody studies in two cases. Archives of Dermatotogy 1987; 123: 866 867. Massa MC, Fretzin DF, Chowhury L, Sweet DL Angiolymphoid hyperplasia demonstrating extensive skin and mucosal lesions controlled with vinblastine therapy. Journat of the American Academy of Dermatology 1984; 11: 3'33-339. Hohhs ER, Bailin PL, Ratz J[., Varhrough CL. Treatment of angiolymphoid hyperplasia of the external ear with carbon dioxide laser. Journal of the American Academy oJ Dermatology 1988; 19: 345 349.
