I
III
I
ll II
II
I
Vulvar melanosis Robert I. Rudolph, M D *
Wyomissing, Pennsylvania
Vulvar melanosis is a condition in which intensely pigmented irregular macules, clinically mimicking malignant melanoma, appear on the vulva. A biopsy specimen shows only marked lower epidermal pigmentation without any melanocytic atypia or nesting. The process is benign, and reassurance is the only treatment. (J AM ACAD DERMATOL1990;23:982-4.) Black, irregular lesions anywhere on the skin or mucosal surfaces engender significant concern in both patient and physician, yet in some cases the anxiety caused by such spots is found to be unjustified. The patient described herein had multiple black spots on the vulva mimicking malignant melanoma that on biopsy proved simply to be areas of intense epidermal hyperpigmentation, a condition given the name "vulvar melanosis."
~-
CASE REPORT
A 34-year-old white woman was referred by her gynecologist because he had noted many intensely hyperpigmerited spots on the vulva. They had developed after the birth of the patient's last child a few years before. The patient had not taken any oral contraceptives or applied anything to the area. She had no family history of such spots, and her general health was excellent. Examination revealed multiple black, somewhat irregular, variously sized (2 to 15 mm) macules on the labia minora but none in the vagina or on the cervix (Fig. 1). A few small black macules were present on the perineal skin, but the conjuctival, anal, and oral mucosal surfaces contained no pigmented spots. Several biopsies were performed. The only significant pathologic finding was intense pigment deposition in keratinocytes of the lower portion of the epidermis (Figs. 2 and 3). Melanocytes were somewhat increased in number but looked normal; no nests of melanocytes or abnormal nuclei were evident. Melanin was also noted in adnexal structures. The epidermis showed some elongation of the rete but appeared to be normal otherwise. No dermal inflammatory infiltrate was present, but some melanophages were seen. From the Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia. Reprints not available. *In private practice at 1134 Penn Ave., Wyomissing, Pa. 16/4/17821
982
Fig. 1. Multiple black, irregular macules on labia minora.
DISCUSSION The concept of vulvar melanosis appears to be well described and recognized by dermatopathologists.l-4 A perusal of five major dermatology textbooks, however, reveals no mention of this condition. A few case reports have appeared in the literature, but in general the condition seems rather u n recognized. 5, 6 Sison-Torre and Ackerman I described eight cases in white women, who ranged in age from 26 to 45 years. The lesions had been noted from 1 to 2 months to up to 26 years. Sites of involvement included the labia minora, labia majora, vaginal introitus, and perineum. In all patients the lesions consisted of hy-
Volume Number November
23 5, Part
2
Vulvar melanosis 983
1990
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Fig. 2. Marked epidermal pigmentation, especially in lower portion. Note involvement of entire specimen. (Hematoxylin-eosin stain; x 102.)
Fig. 3. Close-up view showing intense lower epidermal pigmentation and normal-appearing melanocytes in basal layer. No nesting is seen. (Hematoxylin-eosin stain; x406.)
perpigmented (brownish black), purely macular areas, often with irregular borders and with some mottling of coloration, ranging in size from 5 to 45 mm. One patient noted some vulvar pruritus, but the others were asymptomatic. Two patients had taken oral contraceptives in the past, but none had a history of heavy metal exposure, topical hormonal therapies, or local irradiation, or a family history of pigmentary conditions9 Malignant melanoma or an atypical melanocytic proliferation was the main clinical impression in six of the eight patients.
The pathologic picture was the same in all specimens: intense pigmentation of the epidermis was present, most prominently in the basal cell layer. Melanocytes seemed to be somewhat increased in number, were arranged singly along the dermoepidermal junction, and occasionally showed markedly elongated dendrites extending up into the epidermis but exhibited no consistent atypical histologic features such as abnormal nuclei. No nests of melanocytes were noted. The dermis appeared normal except for many melanophages.
984
Journal of the American Academy of Dermatology
Rudolph
Table I. Histologic differentiation of melanosis of vulva from malignant melanoma in situ of vulva Melanosis of vulva
Slight increase in number of typical melanocytes arranged as solitary units of derrnoepidermal junction
No nests of melanocytes
Typical melanoeytes arranged as solitary units in basal layer of epithelial structures of adnexa Some typical melanocytes with prominent dendrites at dermoepidermal junction; few extend to middle half of epidermis Uniform hyperpigmentation of the epidermis, most prominent in basal layer No melanocytes in mitosis
Malignant melanoma in situ of vulva
Marked increase in number of atypical melanocytes arranged as solitary units (and usually in nests) at all levels of epidermis, sometimes including cornified layer Nests of atypical melanocytes that vary in size and shape and tend to confluence Atypical melanocytes arranged as solitary units and/or in nests within epithelial structures of adnexa Many atypical melanocytes with striking dendrites at dermoepidermal junction and above it; some extend to granular layer Variable pigmentation at all levels of epidermis
and pigmented Bowen's disease. Systemic conditions that can have associated mucosal pigmentary changes include the Peutz-Jeghers syndrome (gastrointestinal polyps and mucocutaneous pigmentation), and the N A M E (nevi, atrial myxomas, myxold neurofibromas, ephelides) and L A M B (lentigines, atrial myxomas, rnucocutaneous myxomas, blue nevi) syndromes, as well as a few others.12 The associated systemic findings and pathologic features clearly distinguish these diseases from the condition described herein. The real concern of the differential diagnosis in vulvar melanosis, of course, is whether there is malignancy. It has been pointed out emphatically that it is impossible to differentiate clinically between this benign pigmentary process and early or evolving melanoma on the vulva. 1 Adequate and prompt biopsy is essential. The histopathologic differention between benign vulvar melanosis and malignant melanoma of the vulva is, however, so clear cut (Table I) that the diagnosis can be made quickly. Vulvar melanosis is probably a completely benign condition and has no potential for malignant transformation. I-4 Extensive or mutilating surgical extirpations are therefore unwarranted; the only treatment necessary is reassurance. REFERENCES
Modified from Sison-TorreEQ, Ackerman AB. Am J Dermatopathol 1985;7(suppl):51-60.
1, Sison-Torre EQ, Ackerman AB. Melanosis of the vulva. Am J Dermatopathol 1985;7(suppl):51-60. 2. Maize JC, Ackerman AB. Pigmented lesions of the skin. Philadelphia: Lea & Febiger, 1987:318-20. 3. Ackerman AB, Troy JL, Rosen LB, et al. Differential diagnosis in dermatopathology II. Philadelphia: Lea & Febiger, 1988:102-5. 4. Maize JC. Mucosal melanosis. Dermatol Clin 1988;6:28393.
Vulvar melanosis fits into the group of pigmentary lesions categorized as mucosal melanoses. 4 Included in this classification are oral lesions such as the labial melanotie macule (idiopathic or reactive) and the melanoacanthoma, and genital lesions such as the penile lentigo and the atypical pigmented penile macule. 7"11 The literature is somewhat confusing about the precise nosology of these pigmented lesions, but essentially they all seem to share the same pathologic features as described previously. The differential diagnosis includes junctional nevi
5. JacksonR. Melanosisof the vulva.J DermatolSurg Oncol 1984;10:119-21. 6. TsukadaY" Benignmelan~ ~ the vaginaand cervix"Am J Obstet Gynecol 1976;124:211. 7. Weathers DR, Corio R_L,Crawford BE, et al. The labial melanotic macule.Oral Surg 1977;44:219-26. 8. Sexton FM, Maize JC. Melanoticmaculesand melanoacanthomasof the lip:a comparativestudywithcensusof the basal melanoeyticpopulation.Am J Dermatopathol 1987; 9:438-44. 9. KopfAW, Bart RS. Tumor conference43: penile lentigo. J Dermatol Surg Oneol 1982;8:637-9. 10. RevuzJ, ClericiT. Penile melanosis.J AM ACADDERMATOL 1989;20:567-70. 11. Leicht S, Youngberg G, Diaz-Miranda C. Atypical pigmentedpenilemacules.Arch Dermatol 1988;124:1267-70. 12. LuckyAW. Pigmentaryabnormalitiesin geneticdisorders. Dermatol Clin 1988;6:193-7.
Some melanocytes may be in mitosis
III
I
ll II
II
I
Vulvar melanosis Robert I. Rudolph, M D *
Wyomissing, Pennsylvania
Vulvar melanosis is a condition in which intensely pigmented irregular macules, clinically mimicking malignant melanoma, appear on the vulva. A biopsy specimen shows only marked lower epidermal pigmentation without any melanocytic atypia or nesting. The process is benign, and reassurance is the only treatment. (J AM ACAD DERMATOL1990;23:982-4.) Black, irregular lesions anywhere on the skin or mucosal surfaces engender significant concern in both patient and physician, yet in some cases the anxiety caused by such spots is found to be unjustified. The patient described herein had multiple black spots on the vulva mimicking malignant melanoma that on biopsy proved simply to be areas of intense epidermal hyperpigmentation, a condition given the name "vulvar melanosis."
~-
CASE REPORT
A 34-year-old white woman was referred by her gynecologist because he had noted many intensely hyperpigmerited spots on the vulva. They had developed after the birth of the patient's last child a few years before. The patient had not taken any oral contraceptives or applied anything to the area. She had no family history of such spots, and her general health was excellent. Examination revealed multiple black, somewhat irregular, variously sized (2 to 15 mm) macules on the labia minora but none in the vagina or on the cervix (Fig. 1). A few small black macules were present on the perineal skin, but the conjuctival, anal, and oral mucosal surfaces contained no pigmented spots. Several biopsies were performed. The only significant pathologic finding was intense pigment deposition in keratinocytes of the lower portion of the epidermis (Figs. 2 and 3). Melanocytes were somewhat increased in number but looked normal; no nests of melanocytes or abnormal nuclei were evident. Melanin was also noted in adnexal structures. The epidermis showed some elongation of the rete but appeared to be normal otherwise. No dermal inflammatory infiltrate was present, but some melanophages were seen. From the Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia. Reprints not available. *In private practice at 1134 Penn Ave., Wyomissing, Pa. 16/4/17821
982
Fig. 1. Multiple black, irregular macules on labia minora.
DISCUSSION The concept of vulvar melanosis appears to be well described and recognized by dermatopathologists.l-4 A perusal of five major dermatology textbooks, however, reveals no mention of this condition. A few case reports have appeared in the literature, but in general the condition seems rather u n recognized. 5, 6 Sison-Torre and Ackerman I described eight cases in white women, who ranged in age from 26 to 45 years. The lesions had been noted from 1 to 2 months to up to 26 years. Sites of involvement included the labia minora, labia majora, vaginal introitus, and perineum. In all patients the lesions consisted of hy-
Volume Number November
23 5, Part
2
Vulvar melanosis 983
1990
. ,! 9
~:
,
~- ; r ,
.,-" 9'
.
-,:
.
.
.
.
.
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.
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.-. ~),!!~,:,
.
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,
.
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,
. .
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., 9
Fig. 2. Marked epidermal pigmentation, especially in lower portion. Note involvement of entire specimen. (Hematoxylin-eosin stain; x 102.)
Fig. 3. Close-up view showing intense lower epidermal pigmentation and normal-appearing melanocytes in basal layer. No nesting is seen. (Hematoxylin-eosin stain; x406.)
perpigmented (brownish black), purely macular areas, often with irregular borders and with some mottling of coloration, ranging in size from 5 to 45 mm. One patient noted some vulvar pruritus, but the others were asymptomatic. Two patients had taken oral contraceptives in the past, but none had a history of heavy metal exposure, topical hormonal therapies, or local irradiation, or a family history of pigmentary conditions9 Malignant melanoma or an atypical melanocytic proliferation was the main clinical impression in six of the eight patients.
The pathologic picture was the same in all specimens: intense pigmentation of the epidermis was present, most prominently in the basal cell layer. Melanocytes seemed to be somewhat increased in number, were arranged singly along the dermoepidermal junction, and occasionally showed markedly elongated dendrites extending up into the epidermis but exhibited no consistent atypical histologic features such as abnormal nuclei. No nests of melanocytes were noted. The dermis appeared normal except for many melanophages.
984
Journal of the American Academy of Dermatology
Rudolph
Table I. Histologic differentiation of melanosis of vulva from malignant melanoma in situ of vulva Melanosis of vulva
Slight increase in number of typical melanocytes arranged as solitary units of derrnoepidermal junction
No nests of melanocytes
Typical melanoeytes arranged as solitary units in basal layer of epithelial structures of adnexa Some typical melanocytes with prominent dendrites at dermoepidermal junction; few extend to middle half of epidermis Uniform hyperpigmentation of the epidermis, most prominent in basal layer No melanocytes in mitosis
Malignant melanoma in situ of vulva
Marked increase in number of atypical melanocytes arranged as solitary units (and usually in nests) at all levels of epidermis, sometimes including cornified layer Nests of atypical melanocytes that vary in size and shape and tend to confluence Atypical melanocytes arranged as solitary units and/or in nests within epithelial structures of adnexa Many atypical melanocytes with striking dendrites at dermoepidermal junction and above it; some extend to granular layer Variable pigmentation at all levels of epidermis
and pigmented Bowen's disease. Systemic conditions that can have associated mucosal pigmentary changes include the Peutz-Jeghers syndrome (gastrointestinal polyps and mucocutaneous pigmentation), and the N A M E (nevi, atrial myxomas, myxold neurofibromas, ephelides) and L A M B (lentigines, atrial myxomas, rnucocutaneous myxomas, blue nevi) syndromes, as well as a few others.12 The associated systemic findings and pathologic features clearly distinguish these diseases from the condition described herein. The real concern of the differential diagnosis in vulvar melanosis, of course, is whether there is malignancy. It has been pointed out emphatically that it is impossible to differentiate clinically between this benign pigmentary process and early or evolving melanoma on the vulva. 1 Adequate and prompt biopsy is essential. The histopathologic differention between benign vulvar melanosis and malignant melanoma of the vulva is, however, so clear cut (Table I) that the diagnosis can be made quickly. Vulvar melanosis is probably a completely benign condition and has no potential for malignant transformation. I-4 Extensive or mutilating surgical extirpations are therefore unwarranted; the only treatment necessary is reassurance. REFERENCES
Modified from Sison-TorreEQ, Ackerman AB. Am J Dermatopathol 1985;7(suppl):51-60.
1, Sison-Torre EQ, Ackerman AB. Melanosis of the vulva. Am J Dermatopathol 1985;7(suppl):51-60. 2. Maize JC, Ackerman AB. Pigmented lesions of the skin. Philadelphia: Lea & Febiger, 1987:318-20. 3. Ackerman AB, Troy JL, Rosen LB, et al. Differential diagnosis in dermatopathology II. Philadelphia: Lea & Febiger, 1988:102-5. 4. Maize JC. Mucosal melanosis. Dermatol Clin 1988;6:28393.
Vulvar melanosis fits into the group of pigmentary lesions categorized as mucosal melanoses. 4 Included in this classification are oral lesions such as the labial melanotie macule (idiopathic or reactive) and the melanoacanthoma, and genital lesions such as the penile lentigo and the atypical pigmented penile macule. 7"11 The literature is somewhat confusing about the precise nosology of these pigmented lesions, but essentially they all seem to share the same pathologic features as described previously. The differential diagnosis includes junctional nevi
5. JacksonR. Melanosisof the vulva.J DermatolSurg Oncol 1984;10:119-21. 6. TsukadaY" Benignmelan~ ~ the vaginaand cervix"Am J Obstet Gynecol 1976;124:211. 7. Weathers DR, Corio R_L,Crawford BE, et al. The labial melanotic macule.Oral Surg 1977;44:219-26. 8. Sexton FM, Maize JC. Melanoticmaculesand melanoacanthomasof the lip:a comparativestudywithcensusof the basal melanoeyticpopulation.Am J Dermatopathol 1987; 9:438-44. 9. KopfAW, Bart RS. Tumor conference43: penile lentigo. J Dermatol Surg Oneol 1982;8:637-9. 10. RevuzJ, ClericiT. Penile melanosis.J AM ACADDERMATOL 1989;20:567-70. 11. Leicht S, Youngberg G, Diaz-Miranda C. Atypical pigmentedpenilemacules.Arch Dermatol 1988;124:1267-70. 12. LuckyAW. Pigmentaryabnormalitiesin geneticdisorders. Dermatol Clin 1988;6:193-7.
Some melanocytes may be in mitosis
