Original Paper Received: January 5, 2015 Accepted after revision: March 4, 2015 Published online: April 14, 2015
Dermatology 2015;231:63–69 DOI: 10.1159/000381421
Eccrine Angiomatous Hamartoma: A Clinicopathological Study of 26 Cases Tutyana Sanusi a Yanqiu Li b Lan Sun c Chunsen Wang a Youwen Zhou d Changzheng Huang a
a
Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, b Department of Dermatology, Zhongshan Hospital, and c Department of Dermatology, The First Hospital of Wuhan City, Wuhan, P.R. China; d Department of Dermatology and Skin Science, University of British Columbia, Vancouver, B.C., Canada
Abstract Background: Eccrine angiomatous hamartoma (EAH) is a rare benign cutaneous tumor characterized by the proliferation of eccrine glands and capillaries. Objective: The aim of this study was to summarize the clinicopathological characteristics of EAH. Methods: A retrospective chart review was performed on all patients diagnosed with EAH from 1977 to 2012 in the Union Hospital, Wuhan, P.R. China, and the clinicopathological features were compared with the cases reported in the literature. Results: A total of 26 patients with EAH were identified. The male:female ratio was 1.2: 1. EAH most commonly presents as a solitary (80.8%) plaque (50.0%) on the lower extremities (61.5%). Most patients presented with hyperhidrosis localizing to the lesion. Although most patients did not have major pain or anatomic deformity, one patient had severe pain as well as difficulty walking and moving, necessitating leg amputation. The histopathological findings showed typical features of EAH. Conclusion: EAH is a rare but
T.S. and Y.L. contributed equally to this work. C.H. and Y.Z. are equal corresponding authors.
© 2015 S. Karger AG, Basel 1018–8665/15/2311–0063$39.50/0 E-Mail [email protected] www.karger.com/drm
characteristically benign skin hamartomatous condition. In rare occasions it can be associated with severe structural and functional impairment. © 2015 S. Karger AG, Basel
Introduction
Eccrine angiomatous hamartoma (EAH) is a rare benign cutaneous tumor which is characterized histopathologically by an intradermal proliferation of eccrine sweat glands and vascular structures in the dermis. It usually presents at birth or during early infancy and childhood, without gender predominance. Most often it manifests as a solitary lesion on the lower extremities. The lesions are often associated with painful sensation on pressure and exhibit hyperhidrosis. The presence of pain and hyperhidrosis can help to raise suspicion of EAH, but histologic confirmation is required for a definitive diagnosis. Microscopically, the lesions are well circumscribed but not encapsulated. The lesions are located in the deep dermis and in subcutaneous tissue.
Changzheng Huang Department of Dermatology, Union Hospital, Tongji Medical College Huazhong University of Science and Technology Wuhan 430022, Hubei (P.R. China) E-Mail hcz0501 @ 126.com Youwen Zhou Department of Dermatology and Skin Science, University of British Columbia 835 West 10th Ave, Vancouver, BC V5Z 4E8 (Canada) E-Mail ywzhou @ interchange.ubc.ca
Downloaded by: Emory University 198.143.47.65 - 1/27/2016 9:36:42 AM
Key Words Eccrine angiomatous hamartoma · Eccrine tumor · Eccrine sweat gland
Materials and Methods A retrospective chart review of all cases of EAH diagnosed from 1977 to 2012 was conducted, revealing 26 cases in the Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, P.R. China. The clinical details were recorded, including the patient’s age and sex, morphology and distribution of lesions, duration, signs and symptoms, histopathological characteristics and treatments. Ultrasound and MRI were performed in one patient. Excisional or incisional biopsies were performed in all cases. The histopathological sections were fixed in buffered 10% formaldehyde for at least 24 h, dehydrated in graded alcohol and then embedded in paraffin. The tissue blocks were cut in serial sections of 4 μm, which were stained with hematoxylin and eosin and examined by light microscopy. Diagnosis was made based on microscopic features typical of EAH, including (1) hyperplasia of normal or dilated eccrine glands, (2) close association of the eccrine structures with capillary angiomatous foci, and (3) the variable presence of pilar, lipomatous, mucinous and/or lymphatic structures.
Results
Clinical Findings The patients’ clinical features are listed in table 1. A total of 26 cases were identified. There were 14 males (53.8%) and 12 females (46.2%). The male:female ratio was 1.2:1. The mean age at diagnosis was 16.4 years (birth to 46 years). 24 cases had onset at 18 years Number of lesions Single Multiple Clinical structure Plaque Nodule Patch Nodule and plaque Plaque and deformity Location Head and neck Trunk Extremities Upper extremities Lower extremities Signs and symptoms (localized) Hyperhidrosis only Hyperhidrosis, pain and hypertrichosis Hyperhidrosis and pain Hyperhidrosis and hypertrichosis Pain only Asymptomatic
14 (53.8%) 12 (46.2%) 24 (92.3%) 2 (7.7%) 21 (80.8%) 5 (19.2%) 13 (50.0%) 6 (23.1%) 3 (11.5%) 3 (11.5%) 1 (3.8%) 2 (7.7%) 6 (23.1%) 18 (69.2%) 2 (7.7%) 16 (61.5%) 1 (3.8%) 4 (15.4%) 12 (46.2%) 1 (3.8%) 3 (11.5%) 5 (19.2%)
ing of hyperhidrosis only was found in 1 patient (3.8%). Hyperhidrosis with other features was found in 17 cases, e.g. coexistence of hyperhidrosis and pain (12/46.2%), hyperhidrosis, pain and hypertrichosis (4/15.4%) or both hyperhidrosis and hypertrichosis (1/3.8%). Ultrasound was performed in 1 patient (case 26) who had a large plaque and anatomic deformity, which showed diffuse lesion with increased blood flow. MRI revealed a contrast-enhanced subcutaneous lesion with diffuse signal intensities in T1and T2-weighted sequences (fig. 1). Histopathology In general, the histopathological findings were similar in all patients and showed typical features of EAH (fig. 2). The lesions showed a proliferation of hyperplastic and an increased number of mature eccrine glands of normal appearance with duct formation and vascular structures. The blood vessels were mainly capillaries, some of which showed slightly dilatation. Mild hyperkeratosis of the epidermis were seen in 7 patients; 5 cases showed hyperpigmentation of the stratum basale. In addition, evidence of Sanusi/Li/Sun/Wang/Zhou/Huang
Downloaded by: Emory University 198.143.47.65 - 1/27/2016 9:36:42 AM
Treatment is required for cases with symptoms such as severe pain, hyperhidrosis, progressive growth or cosmetic considerations, but several authors recommend a watchful waiting approach if the surgery required is aggressive. Ultrasound and MRI may be valuable tools for preoperative study. Due to the rare nature of EAH, there have been only 47 cases reported in the literature. Here we report 26 cases of EAH, summarizing clinical pathological features that showed both similarities and unique features compared to what has been reported in the literature.
e
d
f
c
b
g
h
Fig. 1. Clinical pictures of EAH. a–c A faint blue hairy plaque on the sacral region (a) and a red to brown plaque on the trunk (b) and right thigh (c), accompanied by hyperhidrosis and hypertri-
chosis. d–f Multiple violaceous plaques on the medial side of the right leg with deformity, pain, hyperhidrosis and hypertrichosis are seen. h MRI of the right leg.
Clinicopathological Study of Eccrine Angiomatous Hamartoma
Dermatology 2015;231:63–69 DOI: 10.1159/000381421
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a
a
b
c
d
Fig. 2. Increased number of mature eccrine
hyperkeratosis with follicular plugs, acanthosis and papillomatous hyperplasia was observed occasionally. Five cases showed a few lymphocytes scattered in the dermis. Neither mitotic figures nor cytological atypia were observed. Treatment Twenty-two patients received surgical excision of the lesions under local anesthesia; two of them received embolization before excision. Case 16 had a 20 × 19 cm lesion and was treated with surgical excision followed by skin grafting. Three patients did not receive surgery due to lack of symptoms. One patient (case 26) had surgical amputation of her leg under general anesthesia because of deformity and severe pain of her lesions. There were no spontaneous remissions in any patients who did not have treatment. No complication or recurrence was observed at 1-year follow-up.
Discussion
EAH is a rare benign cutaneous tumor characterized histologically by the proliferation of eccrine glands and capillaries. The term ‘eccrine angiomatous hamartoma’ 66
Dermatology 2015;231:63–69 DOI: 10.1159/000381421
was coined by Heyman et al. [1] in 1968, but the condition was probably first described by Lotzbeck in 1859 as an angioma-like tumor on the cheek of a child, characterized by numerous clusters of eccrine glands within a stroma containing prominent blood vessels [2]. In our case series, the male:female ratio was 1.2:1, similar to literature reports, which claim no gender predominance [3]. The vast majority of our patients had onset at
Dermatology 2015;231:63–69 DOI: 10.1159/000381421
Eccrine Angiomatous Hamartoma: A Clinicopathological Study of 26 Cases Tutyana Sanusi a Yanqiu Li b Lan Sun c Chunsen Wang a Youwen Zhou d Changzheng Huang a
a
Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, b Department of Dermatology, Zhongshan Hospital, and c Department of Dermatology, The First Hospital of Wuhan City, Wuhan, P.R. China; d Department of Dermatology and Skin Science, University of British Columbia, Vancouver, B.C., Canada
Abstract Background: Eccrine angiomatous hamartoma (EAH) is a rare benign cutaneous tumor characterized by the proliferation of eccrine glands and capillaries. Objective: The aim of this study was to summarize the clinicopathological characteristics of EAH. Methods: A retrospective chart review was performed on all patients diagnosed with EAH from 1977 to 2012 in the Union Hospital, Wuhan, P.R. China, and the clinicopathological features were compared with the cases reported in the literature. Results: A total of 26 patients with EAH were identified. The male:female ratio was 1.2: 1. EAH most commonly presents as a solitary (80.8%) plaque (50.0%) on the lower extremities (61.5%). Most patients presented with hyperhidrosis localizing to the lesion. Although most patients did not have major pain or anatomic deformity, one patient had severe pain as well as difficulty walking and moving, necessitating leg amputation. The histopathological findings showed typical features of EAH. Conclusion: EAH is a rare but
T.S. and Y.L. contributed equally to this work. C.H. and Y.Z. are equal corresponding authors.
© 2015 S. Karger AG, Basel 1018–8665/15/2311–0063$39.50/0 E-Mail [email protected] www.karger.com/drm
characteristically benign skin hamartomatous condition. In rare occasions it can be associated with severe structural and functional impairment. © 2015 S. Karger AG, Basel
Introduction
Eccrine angiomatous hamartoma (EAH) is a rare benign cutaneous tumor which is characterized histopathologically by an intradermal proliferation of eccrine sweat glands and vascular structures in the dermis. It usually presents at birth or during early infancy and childhood, without gender predominance. Most often it manifests as a solitary lesion on the lower extremities. The lesions are often associated with painful sensation on pressure and exhibit hyperhidrosis. The presence of pain and hyperhidrosis can help to raise suspicion of EAH, but histologic confirmation is required for a definitive diagnosis. Microscopically, the lesions are well circumscribed but not encapsulated. The lesions are located in the deep dermis and in subcutaneous tissue.
Changzheng Huang Department of Dermatology, Union Hospital, Tongji Medical College Huazhong University of Science and Technology Wuhan 430022, Hubei (P.R. China) E-Mail hcz0501 @ 126.com Youwen Zhou Department of Dermatology and Skin Science, University of British Columbia 835 West 10th Ave, Vancouver, BC V5Z 4E8 (Canada) E-Mail ywzhou @ interchange.ubc.ca
Downloaded by: Emory University 198.143.47.65 - 1/27/2016 9:36:42 AM
Key Words Eccrine angiomatous hamartoma · Eccrine tumor · Eccrine sweat gland
Materials and Methods A retrospective chart review of all cases of EAH diagnosed from 1977 to 2012 was conducted, revealing 26 cases in the Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, P.R. China. The clinical details were recorded, including the patient’s age and sex, morphology and distribution of lesions, duration, signs and symptoms, histopathological characteristics and treatments. Ultrasound and MRI were performed in one patient. Excisional or incisional biopsies were performed in all cases. The histopathological sections were fixed in buffered 10% formaldehyde for at least 24 h, dehydrated in graded alcohol and then embedded in paraffin. The tissue blocks were cut in serial sections of 4 μm, which were stained with hematoxylin and eosin and examined by light microscopy. Diagnosis was made based on microscopic features typical of EAH, including (1) hyperplasia of normal or dilated eccrine glands, (2) close association of the eccrine structures with capillary angiomatous foci, and (3) the variable presence of pilar, lipomatous, mucinous and/or lymphatic structures.
Results
Clinical Findings The patients’ clinical features are listed in table 1. A total of 26 cases were identified. There were 14 males (53.8%) and 12 females (46.2%). The male:female ratio was 1.2:1. The mean age at diagnosis was 16.4 years (birth to 46 years). 24 cases had onset at 18 years Number of lesions Single Multiple Clinical structure Plaque Nodule Patch Nodule and plaque Plaque and deformity Location Head and neck Trunk Extremities Upper extremities Lower extremities Signs and symptoms (localized) Hyperhidrosis only Hyperhidrosis, pain and hypertrichosis Hyperhidrosis and pain Hyperhidrosis and hypertrichosis Pain only Asymptomatic
14 (53.8%) 12 (46.2%) 24 (92.3%) 2 (7.7%) 21 (80.8%) 5 (19.2%) 13 (50.0%) 6 (23.1%) 3 (11.5%) 3 (11.5%) 1 (3.8%) 2 (7.7%) 6 (23.1%) 18 (69.2%) 2 (7.7%) 16 (61.5%) 1 (3.8%) 4 (15.4%) 12 (46.2%) 1 (3.8%) 3 (11.5%) 5 (19.2%)
ing of hyperhidrosis only was found in 1 patient (3.8%). Hyperhidrosis with other features was found in 17 cases, e.g. coexistence of hyperhidrosis and pain (12/46.2%), hyperhidrosis, pain and hypertrichosis (4/15.4%) or both hyperhidrosis and hypertrichosis (1/3.8%). Ultrasound was performed in 1 patient (case 26) who had a large plaque and anatomic deformity, which showed diffuse lesion with increased blood flow. MRI revealed a contrast-enhanced subcutaneous lesion with diffuse signal intensities in T1and T2-weighted sequences (fig. 1). Histopathology In general, the histopathological findings were similar in all patients and showed typical features of EAH (fig. 2). The lesions showed a proliferation of hyperplastic and an increased number of mature eccrine glands of normal appearance with duct formation and vascular structures. The blood vessels were mainly capillaries, some of which showed slightly dilatation. Mild hyperkeratosis of the epidermis were seen in 7 patients; 5 cases showed hyperpigmentation of the stratum basale. In addition, evidence of Sanusi/Li/Sun/Wang/Zhou/Huang
Downloaded by: Emory University 198.143.47.65 - 1/27/2016 9:36:42 AM
Treatment is required for cases with symptoms such as severe pain, hyperhidrosis, progressive growth or cosmetic considerations, but several authors recommend a watchful waiting approach if the surgery required is aggressive. Ultrasound and MRI may be valuable tools for preoperative study. Due to the rare nature of EAH, there have been only 47 cases reported in the literature. Here we report 26 cases of EAH, summarizing clinical pathological features that showed both similarities and unique features compared to what has been reported in the literature.
e
d
f
c
b
g
h
Fig. 1. Clinical pictures of EAH. a–c A faint blue hairy plaque on the sacral region (a) and a red to brown plaque on the trunk (b) and right thigh (c), accompanied by hyperhidrosis and hypertri-
chosis. d–f Multiple violaceous plaques on the medial side of the right leg with deformity, pain, hyperhidrosis and hypertrichosis are seen. h MRI of the right leg.
Clinicopathological Study of Eccrine Angiomatous Hamartoma
Dermatology 2015;231:63–69 DOI: 10.1159/000381421
65
Downloaded by: Emory University 198.143.47.65 - 1/27/2016 9:36:42 AM
a
a
b
c
d
Fig. 2. Increased number of mature eccrine
hyperkeratosis with follicular plugs, acanthosis and papillomatous hyperplasia was observed occasionally. Five cases showed a few lymphocytes scattered in the dermis. Neither mitotic figures nor cytological atypia were observed. Treatment Twenty-two patients received surgical excision of the lesions under local anesthesia; two of them received embolization before excision. Case 16 had a 20 × 19 cm lesion and was treated with surgical excision followed by skin grafting. Three patients did not receive surgery due to lack of symptoms. One patient (case 26) had surgical amputation of her leg under general anesthesia because of deformity and severe pain of her lesions. There were no spontaneous remissions in any patients who did not have treatment. No complication or recurrence was observed at 1-year follow-up.
Discussion
EAH is a rare benign cutaneous tumor characterized histologically by the proliferation of eccrine glands and capillaries. The term ‘eccrine angiomatous hamartoma’ 66
Dermatology 2015;231:63–69 DOI: 10.1159/000381421
was coined by Heyman et al. [1] in 1968, but the condition was probably first described by Lotzbeck in 1859 as an angioma-like tumor on the cheek of a child, characterized by numerous clusters of eccrine glands within a stroma containing prominent blood vessels [2]. In our case series, the male:female ratio was 1.2:1, similar to literature reports, which claim no gender predominance [3]. The vast majority of our patients had onset at
