Eccrine Angiomatous Hamartoma

Background

This is a rare lesion that usuallyt occurs on the lower extremities of children. It is usually solitary and may be painful. It has a variable coloration and may present with a nodule or plaque.

OUTLINE

Epidemiology

Pathogenesis

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Special Stains/Immunohistochemistry/Electron Microscopy

Prognosis and Treatment

Commonly Used Terms

EPIDEMIOLOGY CHARACTERIZATION

AGE RANGE-MEDIAN Children or birth

PATHOGENESIS CHARACTERIZATION

HAMARTOMA

GROSS APPEARANCE/
CLINICAL VARIANTS CHARACTERIZATION

GENERAL

Eccrine angiomatous hamartoma: report of three patients.

Smith C, Montesinos E, Revert A, Ramon D, Molina I, Jorda E.

Servicio de Dermatologia, Hospital Clinico Universitario, Valencia, Spain.
Pediatr Dermatol 1996 Mar-Apr;13(2):139-42 Abstract quote
Eccrine angiomatous hamartoma is a rare, benign entity. The histologic features include proliferation of eccrine sweat glands and angiomatous capillary channels. We report three children who each had a single lesion on a limb.

These lesions were clinically angiomatous, and we obtained the diagnosis by histologic and immunohistochemical studies.

Eccrine angiomatous hamartoma: report of seven cases.

Cebreiro C, Sanchez-Aguilar D, Gomez Centeno P, Fernandez-Redondo V, Toribio J.

Department of Dermatology, General Hospital of Galicia, Faculty of Medicine, Santiago de Compostela, Spain.
Clin Exp Dermatol 1998 Nov;23(6):267-70 Abstract quote
Eccrine angiomatous hamartoma is a rare cutaneous lesion. Histologically it is characterized by the proliferation of eccrine glands, vascular structures and occasionally other elements in the middle and deep dermis; we now report seven further cases of this condition, two with lipomatous involvement and also discuss its nosology.

Eccrine angiomatous hamartoma: report of a case and literature review.

Nakatsui TC, Schloss E, Krol A, Lin AN.

Division of Dermatology and Cutaneous Sciences, University of Alberta, Edmonton.

J Am Acad Dermatol 1999 Jul;41(1):109-11 Abstract quote
Eccrine angiomatous hamartoma is a rare condition characterized histologically by increased numbers of eccrine structures and numerous capillary channels. Patients characteristically have a solitary, congenital nodule that may be painful and that may show hyperhidrosis.

It is important to recognize this condition because it is a benign lesion for which aggressive treatment is not indicated. We report the case of a congenital eccrine angiomatous hamartoma that had a firm nodule studded with blue papules.

Eccrine angiomatous hamartoma.

Pelle MT, Pride HB, Tyler WB.

Departments of Dermatology and Pathology, Geisinger Medical Center.
J Am Acad Dermatol 2002 Sep;47(3):429-35 Abstract quote
Four patients with eccrine angiomatous hamartoma are described. Blue-purple color, enlarging size, location on an extremity, pain, and hypertrichosis were common features and aided differentiation from other vascular anomalies and hamartomas of childhood.

None of our patients experienced lesional hyperhidrosis, and simple excision alleviated pain.

VARIANTS

ADULT

Late-onset eccrine angiomatous hamartoma: clinical, histological and imaging findings.

Laeng RH, Heilbrunner J, Itin PH.

Department of Pathology, Kantonsspital Aarau, Switzerland.
Dermatology 2001;203(1):70-4 Abstract quote
Eccrine angiomatous hamartoma (EAH) is an exceedingly rare benign tumor-like lesion prevalent in childhood which may produce pain and marked sweating. Although an aggressive treatment is not generally indicated, surgery may be considered in severe cases.

In this report we present novel morphological findings by immunophenotyping, document the first MRI findings in EAH and emphasize the importance of preoperative imaging of such lesions.

FINGER

Eccrine angiomatous hamartoma of the finger leading to amputation.

Gabrielsen TO, Elgjo K, Sommerschild H.

Department of Dermatology, National Hospital (Rikshospitalet), Oslo, Norway
Clin Exp Dermatol 1991 Jan;16(1):44-5 Abstract quote
A case of eccrine angiomatous naevus in a 34-year-old pregnant woman is described. The tumour was located on the dorsal aspect of the distal phalanx of the left little finger causing severe pain. Partial resection provided no improvement and finally amputation was necessary.

Histological examination revealed the typical appearance of an eccrine angiomatous hamartoma with a large number of eccrine ducts combined with vascular structures.

MULTIPLE

Eccrine angiomatous hamartoma: a multiple variant.

Aloi F, Tomasini C, Pippione M.

Department of Dermatology, University of Turin, Italy.
Dermatology 1992;184(3):219-22 Abstract quote
A case of multiple eccrine angiomatous hamartoma present in a boy since birth is reported. Clinically, this condition must be differentiated from other neonatal angiomatoses. Sometimes the clinical findings are nonspecific, whereas histologic examination may exclude angiomatoses with visceral involvement.

In our case the hamartomatous nature of this tumor is documented also by the presence of pilar structures intimately related to the eccrine-angiomatous complex in one of two lesions histologically examined. Therefore, the histologic classification of eccrine angiomatous hamartoma into subgroups seems to be excessive.

WRISTS

Eccrine angiomatous hamartoma: a report of symmetric and painful lesions of the wrists.

Morrell DS, Ghali FE, Stahr BJ, McCauliffe DP.

Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599-7287, USA.
Pediatr Dermatol 2001 Mar-Apr;18(2):117-9 Abstract quote
Eccrine angiomatous hamartoma (EAH) is a rare, benign cutaneous lesion histologically defined as a proliferation of eccrine glands within a closely associated vascular stroma.

Typically EAH presents as a solitary flesh-colored, hyperhidrotic, painful papule or plaque appearing at birth or during childhood. Only two previously reported cases have been described involving multiple, symmetrically located lesions. The occurrence of knuckle pads in patients with EAH has not been reported.

We present an instance of multiple painful EAH lesions occurring symmetrically on the extensor wrists in a 14-year-old girl with knuckle pads.

HISTOLOGICAL TYPES CHARACTERIZATION

GENERAL

Eccrine angiomatous hamartoma: a rare skin lesion with diverse histological features.

Challa VR, Jona J.
Dermatologica 1977;155(4):206-9 Abstract quote
A case of painful eccrine angiomatous hamartoma of the wrist in a 10-year-old girl is presented. It was located in the reticular dermis and contained abnormal tubules resembling eccrine secretory coils and thin vascular channels dispersed in the collagenous tissue of the dermis. Some eccrine ducts showed dilatation.

The noteworthy features of this case were the absence of hyperhidrosis over the lesion, and the presence of focal neural infiltration by the tubular structures. Electron microscopy showed mostly clear cells and occasional dark cells lining the tubular structures.

This case supports the theory of abnormal induction of heterotypic dependency with resultant malformation of adnexal as well as mesenchymal elements.

Congenital eccrine angiomatous hamartoma.

Sanmartin O, Botella R, Alegre V, Martinez A, Aliaga A.

Department of Dermatology, University General Hospital, Valencia, Spain.
Am J Dermatopathol 1992 Apr;14(2):161-4 Abstract quote
A case of congenital eccrine angiomatous hamartoma of the foot in a three-month-old girl is presented. Eccrine angiomatous hamartoma is a benign tumoral lesion that combines the proliferation of multiple benign eccrine structures and angiomatous channels. We review the literature on the subject.

VARIANTS

LIPOMATOUS

Eccrine angiomatous hamartoma: a lipomatous variant.

Donati P, Amantea A, Balus L.

Laboratory for Cutaneous Histopathology, S. Gallicano Institute, Rome.
J Cutan Pathol 1989 Aug;16(4):227-9 Abstract quote
The eccrine angiomatous hamartoma is a rare cutaneous lesion histologically characterized by the presence in the intradermal lobules of mature eccrine sweat glands and angiomatous capillary channels.

We report a case of eccrine angiomatous hamartoma with unusual lipomatous involvement.

SPECIAL STAINS/IMMUNOPEROXIDASE/
OTHER CHARACTERIZATION

Eccrine angiomatous hamartoma (nevus): immunohistochemical findings and review of the literature.

Sulica RL, Kao GF, Sulica VI, Penneys NS.

Division of Dermatology, Georgetown University Medical School, Washington, D.C.
J Cutan Pathol 1994 Feb;21(1):71-5 Abstract quote
Eccrine angiomatous hamartoma (nevus) is a rare form of congenital tumorous malformation with proliferation of eccrine secretory coils and ducts, surrounding capillary angiomatous channels and occasionally other minor elements. To date, there have been only about 24 cases reported in the literature.

We report an additional case with more detailed description of the microscopic findings, including immunohistochemical observations. The patient was a 28-year-old female who presented with painless, flesh- to reddish brown-colored, violaceous or bluish subcutaneous nodules on the extremities and trunk. The tumors did not show sweating following exertion. The histologic features were comparable to the previously reported cases. The hamartomatous eccrine sweat glands and ducts and a few apocrine glands demonstrated qualitatively diminished antigens commonly found in the eccrine sweat apparatuses, such as carcinoembryonic antigen (CEA) and S-100 protein.

The findings of CD34, CD44, human nerve growth factor receptor and Ulex europaeus antigens have not been previously reported. The histologic features suggested a "hamartomatous" growth rather than a true neoplastic process.

PROGNOSIS AND TREATMENT CHARACTERIZATION

PROGNOSTIC FACTORS

TREATMENT

Eccrine angiomatous hamartoma.

Calderone DC, Glass LF, Seleznick M, Fenske NA.

Division of Dermatology and Cutaneous Surgery, H. Lee Moffitt Cancer Center and Research Institute, James A. Haley Veterans Administration Hospital, University of South Florida College of Medicine, Tampa 33612.
J Dermatol Surg Oncol 1994 Dec;20(12):837-8 Abstract quote
BACKGROUND. Eccrine angiomatous hamartoma (EAH) is a rare benign lesion that mimics a capillary ("strawberry") hemangioma (CH) in appearance. EAH slowly grows and becomes symptomatic whereas CH typically regresses over time.

OBJECTIVE. To increase awareness of this rare hamartoma and emphasize the need for histopathologic evaluation of similar appearing lesions and thus prompt excision versus observation.

METHODS. Literature review and report of an illustrative case.

RESULTS. Histopathologic confirmation of EAH in a lesion clinically indistinguishable from CH and successful surgical extirpation.

CONCLUSION. EAH is a rare hamartomatous neoplasma clinically similar to CH in appearance. Histopathologic examination of suspected lesions is recommended for delineation as CH generally involutes spontaneously whereas EAH may require surgical excision due to pain and hyperhidrosis.

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Eight Edition. Mosby 1996.
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Weedon D. Weedon's Skin Pathology. Second Edition. Churchill Livingstone. 2002.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

Commonly Used Terms

Soft Tissue Tumors

Last Updated 9/18/2002

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GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL
Eccrine angiomatous hamartoma: report of three patients. Smith C, Montesinos E, Revert A, Ramon D, Molina I, Jorda E. Servicio de Dermatologia, Hospital Clinico Universitario, Valencia, Spain.	Pediatr Dermatol 1996 Mar-Apr;13(2):139-42 Abstract quote Eccrine angiomatous hamartoma is a rare, benign entity. The histologic features include proliferation of eccrine sweat glands and angiomatous capillary channels. We report three children who each had a single lesion on a limb. These lesions were clinically angiomatous, and we obtained the diagnosis by histologic and immunohistochemical studies.
Eccrine angiomatous hamartoma: report of seven cases. Cebreiro C, Sanchez-Aguilar D, Gomez Centeno P, Fernandez-Redondo V, Toribio J. Department of Dermatology, General Hospital of Galicia, Faculty of Medicine, Santiago de Compostela, Spain.	Clin Exp Dermatol 1998 Nov;23(6):267-70 Abstract quote Eccrine angiomatous hamartoma is a rare cutaneous lesion. Histologically it is characterized by the proliferation of eccrine glands, vascular structures and occasionally other elements in the middle and deep dermis; we now report seven further cases of this condition, two with lipomatous involvement and also discuss its nosology.
Eccrine angiomatous hamartoma: report of a case and literature review. Nakatsui TC, Schloss E, Krol A, Lin AN. Division of Dermatology and Cutaneous Sciences, University of Alberta, Edmonton.	J Am Acad Dermatol 1999 Jul;41(1):109-11 Abstract quote Eccrine angiomatous hamartoma is a rare condition characterized histologically by increased numbers of eccrine structures and numerous capillary channels. Patients characteristically have a solitary, congenital nodule that may be painful and that may show hyperhidrosis. It is important to recognize this condition because it is a benign lesion for which aggressive treatment is not indicated. We report the case of a congenital eccrine angiomatous hamartoma that had a firm nodule studded with blue papules.
Eccrine angiomatous hamartoma. Pelle MT, Pride HB, Tyler WB. Departments of Dermatology and Pathology, Geisinger Medical Center.	J Am Acad Dermatol 2002 Sep;47(3):429-35 Abstract quote Four patients with eccrine angiomatous hamartoma are described. Blue-purple color, enlarging size, location on an extremity, pain, and hypertrichosis were common features and aided differentiation from other vascular anomalies and hamartomas of childhood. None of our patients experienced lesional hyperhidrosis, and simple excision alleviated pain.
VARIANTS
ADULT
Late-onset eccrine angiomatous hamartoma: clinical, histological and imaging findings. Laeng RH, Heilbrunner J, Itin PH. Department of Pathology, Kantonsspital Aarau, Switzerland.	Dermatology 2001;203(1):70-4 Abstract quote Eccrine angiomatous hamartoma (EAH) is an exceedingly rare benign tumor-like lesion prevalent in childhood which may produce pain and marked sweating. Although an aggressive treatment is not generally indicated, surgery may be considered in severe cases. In this report we present novel morphological findings by immunophenotyping, document the first MRI findings in EAH and emphasize the importance of preoperative imaging of such lesions.
FINGER
Eccrine angiomatous hamartoma of the finger leading to amputation. Gabrielsen TO, Elgjo K, Sommerschild H. Department of Dermatology, National Hospital (Rikshospitalet), Oslo, Norway	Clin Exp Dermatol 1991 Jan;16(1):44-5 Abstract quote A case of eccrine angiomatous naevus in a 34-year-old pregnant woman is described. The tumour was located on the dorsal aspect of the distal phalanx of the left little finger causing severe pain. Partial resection provided no improvement and finally amputation was necessary. Histological examination revealed the typical appearance of an eccrine angiomatous hamartoma with a large number of eccrine ducts combined with vascular structures.
MULTIPLE
Eccrine angiomatous hamartoma: a multiple variant. Aloi F, Tomasini C, Pippione M. Department of Dermatology, University of Turin, Italy.	Dermatology 1992;184(3):219-22 Abstract quote A case of multiple eccrine angiomatous hamartoma present in a boy since birth is reported. Clinically, this condition must be differentiated from other neonatal angiomatoses. Sometimes the clinical findings are nonspecific, whereas histologic examination may exclude angiomatoses with visceral involvement. In our case the hamartomatous nature of this tumor is documented also by the presence of pilar structures intimately related to the eccrine-angiomatous complex in one of two lesions histologically examined. Therefore, the histologic classification of eccrine angiomatous hamartoma into subgroups seems to be excessive.
WRISTS
Eccrine angiomatous hamartoma: a report of symmetric and painful lesions of the wrists. Morrell DS, Ghali FE, Stahr BJ, McCauliffe DP. Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599-7287, USA.	Pediatr Dermatol 2001 Mar-Apr;18(2):117-9 Abstract quote Eccrine angiomatous hamartoma (EAH) is a rare, benign cutaneous lesion histologically defined as a proliferation of eccrine glands within a closely associated vascular stroma. Typically EAH presents as a solitary flesh-colored, hyperhidrotic, painful papule or plaque appearing at birth or during childhood. Only two previously reported cases have been described involving multiple, symmetrically located lesions. The occurrence of knuckle pads in patients with EAH has not been reported. We present an instance of multiple painful EAH lesions occurring symmetrically on the extensor wrists in a 14-year-old girl with knuckle pads.

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
Eccrine angiomatous hamartoma: a rare skin lesion with diverse histological features. Challa VR, Jona J.	Dermatologica 1977;155(4):206-9 Abstract quote A case of painful eccrine angiomatous hamartoma of the wrist in a 10-year-old girl is presented. It was located in the reticular dermis and contained abnormal tubules resembling eccrine secretory coils and thin vascular channels dispersed in the collagenous tissue of the dermis. Some eccrine ducts showed dilatation. The noteworthy features of this case were the absence of hyperhidrosis over the lesion, and the presence of focal neural infiltration by the tubular structures. Electron microscopy showed mostly clear cells and occasional dark cells lining the tubular structures. This case supports the theory of abnormal induction of heterotypic dependency with resultant malformation of adnexal as well as mesenchymal elements.
Congenital eccrine angiomatous hamartoma. Sanmartin O, Botella R, Alegre V, Martinez A, Aliaga A. Department of Dermatology, University General Hospital, Valencia, Spain.	Am J Dermatopathol 1992 Apr;14(2):161-4 Abstract quote A case of congenital eccrine angiomatous hamartoma of the foot in a three-month-old girl is presented. Eccrine angiomatous hamartoma is a benign tumoral lesion that combines the proliferation of multiple benign eccrine structures and angiomatous channels. We review the literature on the subject.
VARIANTS
LIPOMATOUS
Eccrine angiomatous hamartoma: a lipomatous variant. Donati P, Amantea A, Balus L. Laboratory for Cutaneous Histopathology, S. Gallicano Institute, Rome.	J Cutan Pathol 1989 Aug;16(4):227-9 Abstract quote The eccrine angiomatous hamartoma is a rare cutaneous lesion histologically characterized by the presence in the intradermal lobules of mature eccrine sweat glands and angiomatous capillary channels. We report a case of eccrine angiomatous hamartoma with unusual lipomatous involvement.

Epidemiology
Pathogenesis
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/Immunohistochemistry/Electron Microscopy
Prognosis and Treatment
Commonly Used Terms

EPIDEMIOLOGY	CHARACTERIZATION
AGE RANGE-MEDIAN	Children or birth

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSTIC FACTORS
TREATMENT
Eccrine angiomatous hamartoma. Calderone DC, Glass LF, Seleznick M, Fenske NA. Division of Dermatology and Cutaneous Surgery, H. Lee Moffitt Cancer Center and Research Institute, James A. Haley Veterans Administration Hospital, University of South Florida College of Medicine, Tampa 33612.	J Dermatol Surg Oncol 1994 Dec;20(12):837-8 Abstract quote BACKGROUND. Eccrine angiomatous hamartoma (EAH) is a rare benign lesion that mimics a capillary ("strawberry") hemangioma (CH) in appearance. EAH slowly grows and becomes symptomatic whereas CH typically regresses over time. OBJECTIVE. To increase awareness of this rare hamartoma and emphasize the need for histopathologic evaluation of similar appearing lesions and thus prompt excision versus observation. METHODS. Literature review and report of an illustrative case. RESULTS. Histopathologic confirmation of EAH in a lesion clinically indistinguishable from CH and successful surgical extirpation. CONCLUSION. EAH is a rare hamartomatous neoplasma clinically similar to CH in appearance. Histopathologic examination of suspected lesions is recommended for delineation as CH generally involutes spontaneously whereas EAH may require surgical excision due to pain and hyperhidrosis.