- Email: [email protected]
Eccrine poroma in an unusual site: A clinical and dermoscopic simulator of amelanotic melanoma
Letters 539
J AM ACAD DERMATOL VOLUME 53, NUMBER 3
nerves suggests the hypothesis that abnormal neuromodulation may play a role in the vascular component of PPV and may explain the frequent coexistence of nevus flammeus and nevus anemicus.14 In conclusion, the lack of precise knowledge about the pathogenesis and the genetics of this group of diseases does not yet allow us to develop a diagnostic algorithm and a precise scheme for the follow-up of these patients. Andrea Diociaiuti, PhD Beatrice Guidi, PhD Jose` Angel Aguilar Sanchez, MD Claudio Feliciani, MD Rodolfo Capizzi, MD Pierluigi Amerio, MD Department of Dermatology Catholic University of Sacred Hearth Rome, Italy Correspondence to: Beatrice Guidi, MD Institute of Dermatology Policlinico A. Gemelli Universita` Cattolica del Sacro Cuore Largo A. Gemelli 1 00168, Rome, Italy E-mail: [email protected] REFERENCES 1. Toda K. A new type of phakomatosis pigmentovascularis Ota. Jpn J Dermatol 1966;76:47-51. 2. Hasegawa Y, Yasuhara M. A case of phakomatosis pigmentovascularis type IIIb. Skin Res 1990;32:71-81. 3. Uysal G, Guven A, Ozhan B, Ozturk H, Mutluay AH, Tulunay O. Phakomatosis pigmentovascularis with Sturge-Weber syndrome: a case report. J Dermatol 2000;27:467-70. 4. Di Landro A, Tadini GL, Marcgesi L, Cainelli T. Phakomatosis pigmentovascularis: a new case with renal angiomas and some consideration about classification. Pediatr Dermatol 1999;16:25-30. 5. Teekhasaenee C, Ritch R. Glaucoma in phakomatosis pigmentovascularis. Ophthalmology 1997;104:150-7. 6. Dilber C, Tasdemir HA, Dagdemir A, Incesu L, Odaci E. SturgeWeber syndrome involved frontoparietal region without facial nevus. Pediatr Neurol 2002;26:387-90. 7. Daisuke T, Kazuyoshi F, Masumi S, Ken F, Kisa S, Toshio H, et al. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease. Pediatr Dermatol 1999;16:35-8. 8. Sigg C, Pelloni F. Oligosymptomatic form of Klippel-Tre´naunay-Weber syndrome associated with giant nevus spilus. Arch Dermatol 1989;125:1284-5. 9. Suzuki K, Ishizaki H, Takahashi H. Phakomatosis pigmentovascularis IIIb associated with porokeratosis, acanthosis nigricans, and endocrinopathy in brother and sister. Skin Res 1990;32:6. 10. Mahroughan M, Mehregan AM, Mehregan DA. Phakomatosis pigmentovascularis: report of a case. Pediatr Dermatol 1996;13: 36-8. 11. Libow LF. Phakomatosis pigmentovascularis type IIIb. J Am Acad Dermatol 1993;29:305-7.
12. Tadini G, Restano L, Gonzalez-Perez R, et al. Phacomatosis pigmentokeratotica: report of new cases and further delineation of the syndrome. Arch Dermatol 1998;134:333-7. 13. Happle R. Allelic somatic mutations may explain vascular twin nevi. Hum Genet 1991;86:321-2. 14. Happle R. Mosaicism in human skin. Arch Dermatol 1993; 129:1460-70. doi:10.1016/j.jaad.2005.01.091
Eccrine poroma in an unusual site: A clinical and dermoscopic simulator of amelanotic melanoma To the Editor: Eccrine poroma is an uncommon benign cutaneous neoplasm that usually appears in middle-aged or elderly individuals as a solitary, reddish-pink papule or nodule on the palms or soles.1 We report a case of an eccrine poroma with an unusual location on the pubic region, which simulated amelanotic melanoma both clinically and dermoscopically. A 78-year-old white male was examined for an asymptomatic, well-circumscribed, firm, superficially ulcerated, 1.6 cm 3 1.3 cm, reddish-pink nodule in juxtaposition to a papillomatous plaque with a light brown coloration, located on the pubic region. The plaque had been present for approximately 40 years and had rapidly increased in size during the last year (Fig 1, A). Physical examination did not show any similar lesions or regional lymphadenopathy. Dermoscopic analysis revealed a polymorphous vascular pattern composed mainly of pink to reddish, irregularly shaped and sized structures reminiscent of milky-red areas or red lagoons. Hairpin vessels, dotted vessels, and some linear irregular vessels were also present. In addition, a small ulceration was observed at the periphery of the nodule (Fig 1, B). On the basis of clinical and dermoscopic features, a diagnosis of amelanotic melanoma was made, and the lesion was biopsied. Histopathologic examination revealed a wellcircumscribed, eroded nodule composed of small, monomorphous cuboidal poroid cells with eosinophilic cytoplasm and monomorphic nuclei (Fig 2). Tubular structures and cystic spaces were present within the neoplasm. The background stroma contained eosinophilic hyalinized collagen, and several large and small vessels were seen, especially under the epidermis. In addition, at the edge of the lesion there was a slight pigmentation of basal keratinocytes that correlates to the brownish pigmentation of the papillomatous plaque. Histopathologic findings allowed us to establish the diagnosis of eccrine poroma.
540 Letters
J AM ACAD DERMATOL SEPTEMBER 2005
Fig 1. (A) A reddish-pink nodule, superficially ulcerated, overlying a papillomatous plaque with a light brown coloration, located on the pubic region. (B) Dermoscopic findings of the reddish-pink nodule shows structures similar to milky-red areas or red lagoons (*), linear irregular (^) and dotted (") vessels, hairpin vessels ( ), and ulceration (*).
Fig 2. Histopathologic examination of the eccrine poroma shows a benign tumor composed of aggregates of small cuboidal cells with eosinophilic cytoplasm and monomorphic nuclei. (Hematoxylin-eosin stain; original magnification: 32.)
Clinically, eccrine poroma may exhibit polymorphic features that can make the diagnosis difficult. Clinical differential diagnoses of eccrine poromas described in previous case reports include pyogenic granuloma, hemangioma, seborrheic keratosis, verruca, fibroma, melanoma, nevus, cyst, and basal and squamous cell carcinoma.1-4 In the case described here, the clinical aspect and recent changes in the lesion were highly suggestive of amelanotic melanoma. Moreover, dermoscopic analysis revealed a polymorphous vascular pattern, which is a well-known feature of hypomelanotic and amelanotic melanoma.5-6 In our case, the dermoscopic finding of a polymorphous vascular pattern correlates histopathologically with the presence of several large and small vessels between the aggregates of poroid cells in the reticular dermis. To our knowledge, only one study7 has reported the dermoscopic aspects of two cases of eccrine poroma clinically characterized by brown to black nodules. Dermoscopic analysis of those two cases showed features similar to features observed in pigmented
basal cell carcinoma, such as blue-gray ovoid nests, blue-gray dots, and arborizing telangiectasia in the absence of pigment network. However, a careful evaluation of the dermoscopic image of one of the lesions seems to reveal the presence of dotted and globular vessels, similar to the pattern observed in our patient, which was not described by the authors. In addition, a polymorphous vascular pattern characterized by pinpoint and hairpin-like vessels has recently been reported by Blum et al8 in one case of porocarcinoma and one case of amelanotic melanoma. In conclusion, the similarity of dermoscopic features observed in our case with those reported in amelanotic melanoma underscores the importance of further dermoscopic characterization of non-melanocytic amelanotic lesions, such as eccrine poroma. Davide Altamura, MD Domenico Piccolo, MD Gian Piero Lozzi, MD Ketty Peris, MD Department of Dermatology University of L’Aquila L’Aquila, Italy Correspondence to: Ketty Peris, MD Department of Dermatology University of L’Aquila Via Vetoio-Coppito 2 67100 L’Aquila, Italy E-mail: [email protected] REFERENCES 1. Abenoza P, Ackerman AB. Neoplasm with eccrine differentiation. In: Ackerman’s histologic diagnosis of neoplastic skin diseases. Philadelphia: Lea and Febiger; 1990. pp. 113-85. 2. Moore TO, Orman HL, Orman SK, Helm KF. Poromas of the head and neck. J Am Acad Dermatol 2001;44:48-52. 3. Kircik L, Armus S, Kipping H, Pincus SH. Eccrine poroma in an unusual location. Cutis 1994;54:183-4.
Letters 541
J AM ACAD DERMATOL VOLUME 53, NUMBER 3
4. Roaf V, Chin N, Lynfield Y. Pigmented sweat gland tumor mimicking melanoma. Cutis 1997;59:43-6. 5. Pizzichetta MA, Talamini R, Stanganelli I, Puddu P, Bono R, Argenziano G, et al. Amelanotic/hypomelanotic melanoma: clinical and dermoscopic features. Br J Dermatol 2004;150:1117-24. 6. Argenziano G, Zalaudek I, Corona R, Sera F, Cicale L, Petrillo G, et al. Vascular structures in skin tumors: a dermoscopy study. Arch Dermatol 2004;140:1485-9. 7. Kuo H-W, Ohara K. Pigmented eccrine poroma: A report of two cases and study with dermatoscopy. Dermatol Surg 2003; 29:1076-9. 8. Blum A, Metzler G, Bauer J. Polymorphous vascular patterns in dermoscopy as a sign of malignant skin tumors. A case of an amelanotic melanoma and a porocarcinoma. Dermatology 2005;210:58-9. doi:10.1016/j.jaad.2005.02.057
Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil To the Editor: An otherwise healthy 41-year-old man presented with recurring crops of pruritic, serpiginous, erythematous papules and plaques on his trunk and proximal extremities. The lesions began as papules, became vesicles, scaled, crusted, and resolved with post-inflammatory hyperpigmentation. Biopsy showed a subcorneal blister containing neutrophils with epidermal acanthosis and spongiosis (Figs 1 and 2). Direct immunofluorescence testing showed intercellular staining for IgA in the upper epidermis (Fig 1). The patient was diagnosed with the subcorneal pustular dermatosis subtype of IgA pemphigus. The patient failed multiple treatments, including: acitretin, broadband ultraviolet B therapy, dapsone, methotrexate, and topical and oral steroids. He showed improvement on alefacept, but treatment was discontinued secondary to low CD41 count. Cyclosporine was also effective, but was discontinued secondary to elevated creatinine levels. Adalimumab 40 mg biweekly was started, and after the third dose his skin completely cleared (Fig 3, A and B). Mycophenolate mofetil 1 gm daily was used in conjunction with adalimumab. It had been started 2 weeks prior with no significant improvement upon initiation as monotherapy. Other than occasional, small numbers of new pustules, he has been symptom-free for 5 months.
DISCUSSION IgA pemphigus is an autoimmune intraepidermal blistering disease that presents with a vesiculopustular eruption secondary to circulating IgA autoantibodies that target cell surface components of the epidermis.1 Histologically, IgA pemphigus is
Fig 1. Histiologic sections of affected skin show a subcorneal blister filled with neutrophils forming a subcorneal vesiculopustule. The epidermis shows minimal acantholysis. The superficial dermis shows a mixed inflammatory infiltrate consisting of neutrophils, lymphocytes and histiocytes. (Hematoxylin-eosin stain; original magnification: 340.)
Fig 2. Direct immunofluorescence testing shows IgA deposition in the epidermal squamous intercellular substance. There is increased intensity in the upper layers of the epidermis, a feature which may be seen in the subcorneal pustular variant of IgA pemphigus. (Original magnification: 320.)
classically characterized by intraepidermal bullae with abundant neutrophils, some eosinophils, and acantholysis. Direct immunofluorescence testing shows intraepidermal IgA deposition throughout the epidermis.1 Unlike IgG pemphigus, IgA pemphigus is not often controlled by steroids alone.2,3 Dapsone, the drug of choice for IgA pemphigus treatment,3-6 provided no therapeutic value in our patient. Other successful IgA pemphigus treatments include:
J AM ACAD DERMATOL VOLUME 53, NUMBER 3
nerves suggests the hypothesis that abnormal neuromodulation may play a role in the vascular component of PPV and may explain the frequent coexistence of nevus flammeus and nevus anemicus.14 In conclusion, the lack of precise knowledge about the pathogenesis and the genetics of this group of diseases does not yet allow us to develop a diagnostic algorithm and a precise scheme for the follow-up of these patients. Andrea Diociaiuti, PhD Beatrice Guidi, PhD Jose` Angel Aguilar Sanchez, MD Claudio Feliciani, MD Rodolfo Capizzi, MD Pierluigi Amerio, MD Department of Dermatology Catholic University of Sacred Hearth Rome, Italy Correspondence to: Beatrice Guidi, MD Institute of Dermatology Policlinico A. Gemelli Universita` Cattolica del Sacro Cuore Largo A. Gemelli 1 00168, Rome, Italy E-mail: [email protected] REFERENCES 1. Toda K. A new type of phakomatosis pigmentovascularis Ota. Jpn J Dermatol 1966;76:47-51. 2. Hasegawa Y, Yasuhara M. A case of phakomatosis pigmentovascularis type IIIb. Skin Res 1990;32:71-81. 3. Uysal G, Guven A, Ozhan B, Ozturk H, Mutluay AH, Tulunay O. Phakomatosis pigmentovascularis with Sturge-Weber syndrome: a case report. J Dermatol 2000;27:467-70. 4. Di Landro A, Tadini GL, Marcgesi L, Cainelli T. Phakomatosis pigmentovascularis: a new case with renal angiomas and some consideration about classification. Pediatr Dermatol 1999;16:25-30. 5. Teekhasaenee C, Ritch R. Glaucoma in phakomatosis pigmentovascularis. Ophthalmology 1997;104:150-7. 6. Dilber C, Tasdemir HA, Dagdemir A, Incesu L, Odaci E. SturgeWeber syndrome involved frontoparietal region without facial nevus. Pediatr Neurol 2002;26:387-90. 7. Daisuke T, Kazuyoshi F, Masumi S, Ken F, Kisa S, Toshio H, et al. Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease. Pediatr Dermatol 1999;16:35-8. 8. Sigg C, Pelloni F. Oligosymptomatic form of Klippel-Tre´naunay-Weber syndrome associated with giant nevus spilus. Arch Dermatol 1989;125:1284-5. 9. Suzuki K, Ishizaki H, Takahashi H. Phakomatosis pigmentovascularis IIIb associated with porokeratosis, acanthosis nigricans, and endocrinopathy in brother and sister. Skin Res 1990;32:6. 10. Mahroughan M, Mehregan AM, Mehregan DA. Phakomatosis pigmentovascularis: report of a case. Pediatr Dermatol 1996;13: 36-8. 11. Libow LF. Phakomatosis pigmentovascularis type IIIb. J Am Acad Dermatol 1993;29:305-7.
12. Tadini G, Restano L, Gonzalez-Perez R, et al. Phacomatosis pigmentokeratotica: report of new cases and further delineation of the syndrome. Arch Dermatol 1998;134:333-7. 13. Happle R. Allelic somatic mutations may explain vascular twin nevi. Hum Genet 1991;86:321-2. 14. Happle R. Mosaicism in human skin. Arch Dermatol 1993; 129:1460-70. doi:10.1016/j.jaad.2005.01.091
Eccrine poroma in an unusual site: A clinical and dermoscopic simulator of amelanotic melanoma To the Editor: Eccrine poroma is an uncommon benign cutaneous neoplasm that usually appears in middle-aged or elderly individuals as a solitary, reddish-pink papule or nodule on the palms or soles.1 We report a case of an eccrine poroma with an unusual location on the pubic region, which simulated amelanotic melanoma both clinically and dermoscopically. A 78-year-old white male was examined for an asymptomatic, well-circumscribed, firm, superficially ulcerated, 1.6 cm 3 1.3 cm, reddish-pink nodule in juxtaposition to a papillomatous plaque with a light brown coloration, located on the pubic region. The plaque had been present for approximately 40 years and had rapidly increased in size during the last year (Fig 1, A). Physical examination did not show any similar lesions or regional lymphadenopathy. Dermoscopic analysis revealed a polymorphous vascular pattern composed mainly of pink to reddish, irregularly shaped and sized structures reminiscent of milky-red areas or red lagoons. Hairpin vessels, dotted vessels, and some linear irregular vessels were also present. In addition, a small ulceration was observed at the periphery of the nodule (Fig 1, B). On the basis of clinical and dermoscopic features, a diagnosis of amelanotic melanoma was made, and the lesion was biopsied. Histopathologic examination revealed a wellcircumscribed, eroded nodule composed of small, monomorphous cuboidal poroid cells with eosinophilic cytoplasm and monomorphic nuclei (Fig 2). Tubular structures and cystic spaces were present within the neoplasm. The background stroma contained eosinophilic hyalinized collagen, and several large and small vessels were seen, especially under the epidermis. In addition, at the edge of the lesion there was a slight pigmentation of basal keratinocytes that correlates to the brownish pigmentation of the papillomatous plaque. Histopathologic findings allowed us to establish the diagnosis of eccrine poroma.
540 Letters
J AM ACAD DERMATOL SEPTEMBER 2005
Fig 1. (A) A reddish-pink nodule, superficially ulcerated, overlying a papillomatous plaque with a light brown coloration, located on the pubic region. (B) Dermoscopic findings of the reddish-pink nodule shows structures similar to milky-red areas or red lagoons (*), linear irregular (^) and dotted (") vessels, hairpin vessels ( ), and ulceration (*).
Fig 2. Histopathologic examination of the eccrine poroma shows a benign tumor composed of aggregates of small cuboidal cells with eosinophilic cytoplasm and monomorphic nuclei. (Hematoxylin-eosin stain; original magnification: 32.)
Clinically, eccrine poroma may exhibit polymorphic features that can make the diagnosis difficult. Clinical differential diagnoses of eccrine poromas described in previous case reports include pyogenic granuloma, hemangioma, seborrheic keratosis, verruca, fibroma, melanoma, nevus, cyst, and basal and squamous cell carcinoma.1-4 In the case described here, the clinical aspect and recent changes in the lesion were highly suggestive of amelanotic melanoma. Moreover, dermoscopic analysis revealed a polymorphous vascular pattern, which is a well-known feature of hypomelanotic and amelanotic melanoma.5-6 In our case, the dermoscopic finding of a polymorphous vascular pattern correlates histopathologically with the presence of several large and small vessels between the aggregates of poroid cells in the reticular dermis. To our knowledge, only one study7 has reported the dermoscopic aspects of two cases of eccrine poroma clinically characterized by brown to black nodules. Dermoscopic analysis of those two cases showed features similar to features observed in pigmented
basal cell carcinoma, such as blue-gray ovoid nests, blue-gray dots, and arborizing telangiectasia in the absence of pigment network. However, a careful evaluation of the dermoscopic image of one of the lesions seems to reveal the presence of dotted and globular vessels, similar to the pattern observed in our patient, which was not described by the authors. In addition, a polymorphous vascular pattern characterized by pinpoint and hairpin-like vessels has recently been reported by Blum et al8 in one case of porocarcinoma and one case of amelanotic melanoma. In conclusion, the similarity of dermoscopic features observed in our case with those reported in amelanotic melanoma underscores the importance of further dermoscopic characterization of non-melanocytic amelanotic lesions, such as eccrine poroma. Davide Altamura, MD Domenico Piccolo, MD Gian Piero Lozzi, MD Ketty Peris, MD Department of Dermatology University of L’Aquila L’Aquila, Italy Correspondence to: Ketty Peris, MD Department of Dermatology University of L’Aquila Via Vetoio-Coppito 2 67100 L’Aquila, Italy E-mail: [email protected] REFERENCES 1. Abenoza P, Ackerman AB. Neoplasm with eccrine differentiation. In: Ackerman’s histologic diagnosis of neoplastic skin diseases. Philadelphia: Lea and Febiger; 1990. pp. 113-85. 2. Moore TO, Orman HL, Orman SK, Helm KF. Poromas of the head and neck. J Am Acad Dermatol 2001;44:48-52. 3. Kircik L, Armus S, Kipping H, Pincus SH. Eccrine poroma in an unusual location. Cutis 1994;54:183-4.
Letters 541
J AM ACAD DERMATOL VOLUME 53, NUMBER 3
4. Roaf V, Chin N, Lynfield Y. Pigmented sweat gland tumor mimicking melanoma. Cutis 1997;59:43-6. 5. Pizzichetta MA, Talamini R, Stanganelli I, Puddu P, Bono R, Argenziano G, et al. Amelanotic/hypomelanotic melanoma: clinical and dermoscopic features. Br J Dermatol 2004;150:1117-24. 6. Argenziano G, Zalaudek I, Corona R, Sera F, Cicale L, Petrillo G, et al. Vascular structures in skin tumors: a dermoscopy study. Arch Dermatol 2004;140:1485-9. 7. Kuo H-W, Ohara K. Pigmented eccrine poroma: A report of two cases and study with dermatoscopy. Dermatol Surg 2003; 29:1076-9. 8. Blum A, Metzler G, Bauer J. Polymorphous vascular patterns in dermoscopy as a sign of malignant skin tumors. A case of an amelanotic melanoma and a porocarcinoma. Dermatology 2005;210:58-9. doi:10.1016/j.jaad.2005.02.057
Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil To the Editor: An otherwise healthy 41-year-old man presented with recurring crops of pruritic, serpiginous, erythematous papules and plaques on his trunk and proximal extremities. The lesions began as papules, became vesicles, scaled, crusted, and resolved with post-inflammatory hyperpigmentation. Biopsy showed a subcorneal blister containing neutrophils with epidermal acanthosis and spongiosis (Figs 1 and 2). Direct immunofluorescence testing showed intercellular staining for IgA in the upper epidermis (Fig 1). The patient was diagnosed with the subcorneal pustular dermatosis subtype of IgA pemphigus. The patient failed multiple treatments, including: acitretin, broadband ultraviolet B therapy, dapsone, methotrexate, and topical and oral steroids. He showed improvement on alefacept, but treatment was discontinued secondary to low CD41 count. Cyclosporine was also effective, but was discontinued secondary to elevated creatinine levels. Adalimumab 40 mg biweekly was started, and after the third dose his skin completely cleared (Fig 3, A and B). Mycophenolate mofetil 1 gm daily was used in conjunction with adalimumab. It had been started 2 weeks prior with no significant improvement upon initiation as monotherapy. Other than occasional, small numbers of new pustules, he has been symptom-free for 5 months.
DISCUSSION IgA pemphigus is an autoimmune intraepidermal blistering disease that presents with a vesiculopustular eruption secondary to circulating IgA autoantibodies that target cell surface components of the epidermis.1 Histologically, IgA pemphigus is
Fig 1. Histiologic sections of affected skin show a subcorneal blister filled with neutrophils forming a subcorneal vesiculopustule. The epidermis shows minimal acantholysis. The superficial dermis shows a mixed inflammatory infiltrate consisting of neutrophils, lymphocytes and histiocytes. (Hematoxylin-eosin stain; original magnification: 340.)
Fig 2. Direct immunofluorescence testing shows IgA deposition in the epidermal squamous intercellular substance. There is increased intensity in the upper layers of the epidermis, a feature which may be seen in the subcorneal pustular variant of IgA pemphigus. (Original magnification: 320.)
classically characterized by intraepidermal bullae with abundant neutrophils, some eosinophils, and acantholysis. Direct immunofluorescence testing shows intraepidermal IgA deposition throughout the epidermis.1 Unlike IgG pemphigus, IgA pemphigus is not often controlled by steroids alone.2,3 Dapsone, the drug of choice for IgA pemphigus treatment,3-6 provided no therapeutic value in our patient. Other successful IgA pemphigus treatments include: