Ophthal Plast Reconstr Surg, Vol. 32, No. 4, 2016
Case Reports
REFERENCES 1. Comi AM. Pathophysiology of Sturge–Weber syndrome. J Child Neurol 2003;18:509–16. 2. Ch’ng S, Tan ST. Facial port-wine stains—clinical stratification and risks of neuro-ocular involvement. J Plast Reconstr Aesthet Surg 2008;61:889–93. 3. Sharan S, Swamy B, Taranath DA, et al. Port-wine vascular malformations and glaucoma risk in Sturge–Weber syndrome. J AAPOS 2009;13:374–8. 4. Lindsey PS, Shields JA, Goldberg RE, et al. Bilateral choroidal hemangiomas and facial nevus flammeus. Retina 1981;1:88–95. 5. Shields CL, Kligman BE, Suriano M, et al. Phacomatosis pigmentovascularis of cesioflammea type in 7 patients: combination of ocular pigmentation (melanocytosis or melanosis) and nevus flammeus with risk for melanoma. Arch Ophthalmol 2011;129:746–50. 6. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111:997–1008. 7. Shields CL, Shields JA, Eagle RC, et al. Clinicopathologic review of 142 cases of lacrimal gland lesions. Ophthalmology 1989;96:431–5. 8. Teo L, Seah LL, Choo CT, et al. A survey of the histopathology of lacrimal gland lesions in a tertiary referral centre. Orbit 2013;32:1–7.
Acantholytic Acanthoma of the Eyelid: Unusual Presentation Ruchi Mittal, M.D.*, and Samir Mahapatra, M.S† Abstract: Acantholytic acanthoma is a benign epidermal tumor with a truncal predisposition and is not previously reported as an eyelid mass. A 63-year-old man, presented with a history of nodular masses of both his eyelid margin and eyelid surface of 3 years duration. Excision biopsy of lesion of eyelid margin was consistent with dermal nevus. Lesions of the eyelid surface displayed prominent acanthosis, acantholysis, and spongiosis. Variable hyperkeratosis and papillomatosis was also noted. There was absence of cytological atypia or mitoses. The microscopic picture was consistent with acantholytic acanthoma. The presence of extensive acantholysis in the present case excluded incidental focal epidermolytic hyperkeratosis and focal acantholytic dyskeratosis. Acantholytic vesiculobullous disorders and numerous acanthotic disorders, which are more common in the eyelid, need exclusion. To the authors’ best knowledge, as acantholytic acanthoma of the eyelid is not previously reported, they recommend that it should be included as differential diagnoses of benign epidermal lesions of eyelid.
FIG. 1. Clinical picture of OD, showing pigmented lesion on the eyelid margin (arrow) and raised polypoidal lesion on lateral aspect of eyelid of OD (arrowhead).
acantholytic acanthomas in 1988.2 The term “acantholysis” refers to loss of keratinocytic cell-to-cell contact, and acanthoma refers to a benign tumor of epidermal keratinocytes. Acantholytic acanthoma is present as a solitary, commonly asymptomatic keratotic papule or nodule, shows male preponderance, and has truncal predisposition.2,3 A review of English literature search revealed no case of acantholytic acanthoma of the eyelid. Brownstein’s series of 31 acantholytic acanthomas did not reveal any case with facial involvement, except for a single case of aural involvement. The present case is not only unique in its presentation as eyelid lesion but also unique in its associated presence of dermal marginal nevus in the same eyelid.
CASE REPORT A 63-year-old man presented with history of 2 nodular masses on his left upper eyelid and nodular masses on his right eyelid (Fig. 1) of 3 years duration. All the lesions appeared almost at the same time, gradually increased in size over a period of 1 year, and then became stationary. The lesions at the eyelid margin (Fig. 1, arrow) were pigmented and slightly
A
cantholytic acanthoma is a distinctive and rare benign solitary cutaneous tumor, first described by Brownstein1 in 1985, who also published his largest case series of 31
*Dalmia Ophthalmic Pathology Service, LV Prasad Eye Institute, Bhubaneswar, Odisha, India; and †Department of Ophthalmic Plastic Surgery, Orbit and Ocular Oncology, L V Prasad Eye Institute, Visakhapatnam, Andhra Pradesh, India Accepted for publication June 20, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Ruchi Mittal, M.D., LV Prasad Eye Institute, Patia, Bhubaneswar 751 024, Odisha, India. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000276
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FIG. 2. There is variable hyperkeratosis, irregular acanthosis, and papillomatosis. Note that there is absence of squamous eddies (hematoxylin-eosin stain, original magnification: ×100).
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 4, 2016
FIG. 3. There is prominent acantholysis associated with suprabasilar clefts. There is no evidence of dyskeratosis (hematoxylin-eosin stain, original magnification: ×400).
elevated. On eversion of the eyelid, the palpebral conjunctiva was normal. The lid margins were regular without loss of eyelashes. The lesions of the eyelid surface were pigmented, slightly elevated, and present at the junction of middle and lateral one-third of the left upper lid and close to the lateral aspect of the right lid (Fig. 1, arrowhead). The remaining ocular and systemic examination was normal. Regional lymph nodes were not enlarged. Based on the above clinical picture, diagnosis of upper lid nevus for the eyelid margin lesions and benign papilloma for the surface lesion was made, and excision biopsy of both the lesions of the OD was done under local anesthesia for cosmetic reasons. Microscopic examination of the lesion of the right eyelid margin (Fig. 1, arrow) was consistent with intradermal nevus. The surface lesion (Fig. 1, arrowhead) was polypoidal, showed variable but compact hyperkeratosis (Fig. 2). There was hypergranulosis, irregular acanthosis, and papillomatosis (Fig. 2). Acantholysis was the most prominent finding through the fullthickness stratum spinosum, predominantly being suprabasal, associated with suprabasilar clefts (Figs. 3 and 4). Acantholytic cells appeared rounded with condensed cytoplasm, large nucleus with prominent nucleoli, however, there was absence of squamous whorls, dyskeratosis, or cytological atypia. Spongiosis was also noted throughout the stratum spinosum (Fig. 4). Stroma was fibrovascular with absence of inflammation. A diagnosis of acantholytic acanthoma was made. This was subsequently followed by excision of left eyelid margin and surface lesions, which were also diagnosed as intradermal nevus and acantholytic acanthoma, respectively. At 6 months follow-up postexcision, there had been no local recurrence of the lesion.
DISCUSSION Wide gamut of benign and malignant lesions can involve the eyelid. Given the rarity of acantholytic acanthoma in literature, especially in the periocular region, it may pose a diagnostic challenge both clinically and histologically. Clinically acantholytic acanthoma shows no specific appearance, can be present as a keratotic papule or nodule, and can sometimes mimic squamous papilloma, molluscum contagiosum, and hemorrhagic bulla.4,5 They are commonly solitary lesions, although multiple lesions have been reported in the genital region. Histologically, acantholytic acanthoma needs to be differentiated from other
Case Reports
FIG. 4. There is marked spongiosis; prominent acantholysis is also noted with suprabasilar clefts (arrow; hematoxylin-eosin stain, original magnification: ×400).
common lesions displaying acantholysis and acanthosis. Acantholytic vesiculobullous disorders include pemphigus, Hailey–Hailey disease, spongiotic dermatitis, and Grover disease. Darier disease although presents hyperkeratotic papule, shows classically corp ronds and grains. Acanthotic disorders such as clear cell acanthoma, epidermolytic acanthoma, warty dykeratoma, actinic keratosis, and irritated seborrheic keratosis need exclusion. Acantholysis as the prominent finding helps exclusion of all acanthotic disorders except warty dyskeratoma with presence of papillomatosis and supra basilar clefts; however, the presence of keratotic umbilicated center and dyskeratosis in warty dyskeratoma is distinctive.3 Acantholysis may be observed in irritated seborrheic keratosis; however, the acantholysis of irritated seborrheic keratosis is not predominantly suprabasal, and seborrheic keratosis of irritated type shows numerous characteristic “squamous eddies” or whorls.3 Simple surgical excision is the mainstay of treatment of acanthotic acanthoma; however, Minakawa et al.5 have suggested therapy with retinoic acid as a nonsurgical alternate for patients unwilling for surgery. The presence of marginal intradermal nevus in the eyelid margin appears to be incidental. Incidental focal epidermolytic hyperkeratosis and focal acantholytic dyskeratosis has been described adjacent or within unrelated pathological benign and malignant lesions such as seborrheic keratosis, actinic keratosis, carcinoma, melanocytic lesions, and melanoma. Although the changes in incidental forms are less extensive, the authors feel that it would be prudent to exclude associated presence of more common and clinically more important pathological conditions (mentioned above) when epidermolysis, dyskeratosis, and/or acantholysis is noted in a eyelid biopsy.
REFERENCES 1. Brownstein MH. The benign acanthomas. J Cutan Pathol 1985;12:172–88. 2. Brownstein MH. Acantholytic acanthoma. J Am Acad Dermatol 1988;19(5 pt 1):783–6. 3. Kikham N. Tumors and cysts of the epidermis. In: David E. Elder, ed. Histopathology of the Skin. 10th ed. New Delhi: Wolters Kluwer, 2009:791–849. 4. Cho S, Lee HK, Song KY. Acantholytic acanthoma clinically resembling a molluscum contagiosum. J Eur Acad Dermatol Venereol 2007;21:119–20. 5. Minakawa S, Matsuzaki Y, Nakano H, et al. Acantholysis caused repeated hemorrhagic bullae in a case of acantholytic acanthoma. J Dermatol 2012;39:1107–8.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Case Reports
REFERENCES 1. Comi AM. Pathophysiology of Sturge–Weber syndrome. J Child Neurol 2003;18:509–16. 2. Ch’ng S, Tan ST. Facial port-wine stains—clinical stratification and risks of neuro-ocular involvement. J Plast Reconstr Aesthet Surg 2008;61:889–93. 3. Sharan S, Swamy B, Taranath DA, et al. Port-wine vascular malformations and glaucoma risk in Sturge–Weber syndrome. J AAPOS 2009;13:374–8. 4. Lindsey PS, Shields JA, Goldberg RE, et al. Bilateral choroidal hemangiomas and facial nevus flammeus. Retina 1981;1:88–95. 5. Shields CL, Kligman BE, Suriano M, et al. Phacomatosis pigmentovascularis of cesioflammea type in 7 patients: combination of ocular pigmentation (melanocytosis or melanosis) and nevus flammeus with risk for melanoma. Arch Ophthalmol 2011;129:746–50. 6. Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111:997–1008. 7. Shields CL, Shields JA, Eagle RC, et al. Clinicopathologic review of 142 cases of lacrimal gland lesions. Ophthalmology 1989;96:431–5. 8. Teo L, Seah LL, Choo CT, et al. A survey of the histopathology of lacrimal gland lesions in a tertiary referral centre. Orbit 2013;32:1–7.
Acantholytic Acanthoma of the Eyelid: Unusual Presentation Ruchi Mittal, M.D.*, and Samir Mahapatra, M.S† Abstract: Acantholytic acanthoma is a benign epidermal tumor with a truncal predisposition and is not previously reported as an eyelid mass. A 63-year-old man, presented with a history of nodular masses of both his eyelid margin and eyelid surface of 3 years duration. Excision biopsy of lesion of eyelid margin was consistent with dermal nevus. Lesions of the eyelid surface displayed prominent acanthosis, acantholysis, and spongiosis. Variable hyperkeratosis and papillomatosis was also noted. There was absence of cytological atypia or mitoses. The microscopic picture was consistent with acantholytic acanthoma. The presence of extensive acantholysis in the present case excluded incidental focal epidermolytic hyperkeratosis and focal acantholytic dyskeratosis. Acantholytic vesiculobullous disorders and numerous acanthotic disorders, which are more common in the eyelid, need exclusion. To the authors’ best knowledge, as acantholytic acanthoma of the eyelid is not previously reported, they recommend that it should be included as differential diagnoses of benign epidermal lesions of eyelid.
FIG. 1. Clinical picture of OD, showing pigmented lesion on the eyelid margin (arrow) and raised polypoidal lesion on lateral aspect of eyelid of OD (arrowhead).
acantholytic acanthomas in 1988.2 The term “acantholysis” refers to loss of keratinocytic cell-to-cell contact, and acanthoma refers to a benign tumor of epidermal keratinocytes. Acantholytic acanthoma is present as a solitary, commonly asymptomatic keratotic papule or nodule, shows male preponderance, and has truncal predisposition.2,3 A review of English literature search revealed no case of acantholytic acanthoma of the eyelid. Brownstein’s series of 31 acantholytic acanthomas did not reveal any case with facial involvement, except for a single case of aural involvement. The present case is not only unique in its presentation as eyelid lesion but also unique in its associated presence of dermal marginal nevus in the same eyelid.
CASE REPORT A 63-year-old man presented with history of 2 nodular masses on his left upper eyelid and nodular masses on his right eyelid (Fig. 1) of 3 years duration. All the lesions appeared almost at the same time, gradually increased in size over a period of 1 year, and then became stationary. The lesions at the eyelid margin (Fig. 1, arrow) were pigmented and slightly
A
cantholytic acanthoma is a distinctive and rare benign solitary cutaneous tumor, first described by Brownstein1 in 1985, who also published his largest case series of 31
*Dalmia Ophthalmic Pathology Service, LV Prasad Eye Institute, Bhubaneswar, Odisha, India; and †Department of Ophthalmic Plastic Surgery, Orbit and Ocular Oncology, L V Prasad Eye Institute, Visakhapatnam, Andhra Pradesh, India Accepted for publication June 20, 2014. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Ruchi Mittal, M.D., LV Prasad Eye Institute, Patia, Bhubaneswar 751 024, Odisha, India. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000276
e94
FIG. 2. There is variable hyperkeratosis, irregular acanthosis, and papillomatosis. Note that there is absence of squamous eddies (hematoxylin-eosin stain, original magnification: ×100).
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 32, No. 4, 2016
FIG. 3. There is prominent acantholysis associated with suprabasilar clefts. There is no evidence of dyskeratosis (hematoxylin-eosin stain, original magnification: ×400).
elevated. On eversion of the eyelid, the palpebral conjunctiva was normal. The lid margins were regular without loss of eyelashes. The lesions of the eyelid surface were pigmented, slightly elevated, and present at the junction of middle and lateral one-third of the left upper lid and close to the lateral aspect of the right lid (Fig. 1, arrowhead). The remaining ocular and systemic examination was normal. Regional lymph nodes were not enlarged. Based on the above clinical picture, diagnosis of upper lid nevus for the eyelid margin lesions and benign papilloma for the surface lesion was made, and excision biopsy of both the lesions of the OD was done under local anesthesia for cosmetic reasons. Microscopic examination of the lesion of the right eyelid margin (Fig. 1, arrow) was consistent with intradermal nevus. The surface lesion (Fig. 1, arrowhead) was polypoidal, showed variable but compact hyperkeratosis (Fig. 2). There was hypergranulosis, irregular acanthosis, and papillomatosis (Fig. 2). Acantholysis was the most prominent finding through the fullthickness stratum spinosum, predominantly being suprabasal, associated with suprabasilar clefts (Figs. 3 and 4). Acantholytic cells appeared rounded with condensed cytoplasm, large nucleus with prominent nucleoli, however, there was absence of squamous whorls, dyskeratosis, or cytological atypia. Spongiosis was also noted throughout the stratum spinosum (Fig. 4). Stroma was fibrovascular with absence of inflammation. A diagnosis of acantholytic acanthoma was made. This was subsequently followed by excision of left eyelid margin and surface lesions, which were also diagnosed as intradermal nevus and acantholytic acanthoma, respectively. At 6 months follow-up postexcision, there had been no local recurrence of the lesion.
DISCUSSION Wide gamut of benign and malignant lesions can involve the eyelid. Given the rarity of acantholytic acanthoma in literature, especially in the periocular region, it may pose a diagnostic challenge both clinically and histologically. Clinically acantholytic acanthoma shows no specific appearance, can be present as a keratotic papule or nodule, and can sometimes mimic squamous papilloma, molluscum contagiosum, and hemorrhagic bulla.4,5 They are commonly solitary lesions, although multiple lesions have been reported in the genital region. Histologically, acantholytic acanthoma needs to be differentiated from other
Case Reports
FIG. 4. There is marked spongiosis; prominent acantholysis is also noted with suprabasilar clefts (arrow; hematoxylin-eosin stain, original magnification: ×400).
common lesions displaying acantholysis and acanthosis. Acantholytic vesiculobullous disorders include pemphigus, Hailey–Hailey disease, spongiotic dermatitis, and Grover disease. Darier disease although presents hyperkeratotic papule, shows classically corp ronds and grains. Acanthotic disorders such as clear cell acanthoma, epidermolytic acanthoma, warty dykeratoma, actinic keratosis, and irritated seborrheic keratosis need exclusion. Acantholysis as the prominent finding helps exclusion of all acanthotic disorders except warty dyskeratoma with presence of papillomatosis and supra basilar clefts; however, the presence of keratotic umbilicated center and dyskeratosis in warty dyskeratoma is distinctive.3 Acantholysis may be observed in irritated seborrheic keratosis; however, the acantholysis of irritated seborrheic keratosis is not predominantly suprabasal, and seborrheic keratosis of irritated type shows numerous characteristic “squamous eddies” or whorls.3 Simple surgical excision is the mainstay of treatment of acanthotic acanthoma; however, Minakawa et al.5 have suggested therapy with retinoic acid as a nonsurgical alternate for patients unwilling for surgery. The presence of marginal intradermal nevus in the eyelid margin appears to be incidental. Incidental focal epidermolytic hyperkeratosis and focal acantholytic dyskeratosis has been described adjacent or within unrelated pathological benign and malignant lesions such as seborrheic keratosis, actinic keratosis, carcinoma, melanocytic lesions, and melanoma. Although the changes in incidental forms are less extensive, the authors feel that it would be prudent to exclude associated presence of more common and clinically more important pathological conditions (mentioned above) when epidermolysis, dyskeratosis, and/or acantholysis is noted in a eyelid biopsy.
REFERENCES 1. Brownstein MH. The benign acanthomas. J Cutan Pathol 1985;12:172–88. 2. Brownstein MH. Acantholytic acanthoma. J Am Acad Dermatol 1988;19(5 pt 1):783–6. 3. Kikham N. Tumors and cysts of the epidermis. In: David E. Elder, ed. Histopathology of the Skin. 10th ed. New Delhi: Wolters Kluwer, 2009:791–849. 4. Cho S, Lee HK, Song KY. Acantholytic acanthoma clinically resembling a molluscum contagiosum. J Eur Acad Dermatol Venereol 2007;21:119–20. 5. Minakawa S, Matsuzaki Y, Nakano H, et al. Acantholysis caused repeated hemorrhagic bullae in a case of acantholytic acanthoma. J Dermatol 2012;39:1107–8.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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