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Multiple vascular fibromas and myxoid fibromas of the fingers
Gross and microscopic svrnpOSiUf'11 PM&&'
Multiple vascular fibromas and myxoid fibromas of the fingers A histologic and ultrastructural study Ralph J. Coskey, M.D., Amir H. Meh;-egan, M.D., and AJrel P. Lupu1escu, M.D. Detroit, MI A father and his son were seen be.:ause of multiple wartlike lesions cn their fingers and palms. On histologic ex:::mination, these lesions showed changes compatible with vas.:ular fibromas, myxoid fibromas. and. in a few instances. verruca vulgaris. We are not aware of reports of similar cases in the dermatologic literature. (J AM ACAD DERMATOL 2:425-431, 1980.)
CASE REPORTS
Case 1 A 30-year-old white man was seen originally on Aug. 24, 1978. because of multiple verrucous papules on tis fingers and palms. He claimed that he had had these lesions for 16 years. He also stated that his father had had similar lesions for 25 years. Physical examination revealed approximately one hundred verrucous skin-colored to brown-red, warty papules, measuring 1 to 3 mm in diameter, on the dorsal and palmar a~pects of the fingers as well as the palms (Figs. 1 and 2). Over a period of many months, approximately fifty lesions were excised with either a curet or iris scissors, and the base of each of these sites was electrodesiccated. However, many of the lesions tended to recur.
Fig. 1. Multiple warty lesions are
~een
on the fingers.
Case 2 A 58-year-old white man, the father of Patient 1, was seen on March 12, 1979. because of multiple verrucous 1- to 3-mm papules on the palms as well as on the palrr:ar and dorsal aspects of the fingers. These lesions were clinically similar to those of his son. Biopsies of two lesions were obtained. Histopathology. Approximately twenty-two lesions excised from the fingers or plilms of Patient 1 were From the Department of Dermatology. Wayne Slate Cniversi:y. School of Medicine. Reprint requests ro: Dr. Ralph J. Caskey. 23! 33 Orchard Lake Rd .• Suite 20]. Farmington. MI 48024/313-476-4851.
:.1190-9622/80'050425+07500.70/0 © 1980 Am Acad Dermatol
Fig. 2. There are many verrucous papules in the area of :he distal interphalangeal joints.
425
426
CosKey et 0/
.loumal of the American Academy of OerlTla~ology
Fig. 3. A, Acanthosis, vascular proliferation, and myxomatous connective tissue are seen in the section. (Hematoxylin-eosin stain; x 125.) B, Multiple fibroblasts and large amounts of acid mucopolysaccharide are seen in the dermis. (Alcian blue-PAS stain; X400.)
examined by light microscopy and two lesions by electron microscopy. Two lesions excised from Patient 2 were also examined by light microscopy. Sections examined by light microscopy were stained with hematoxylin and eosin, acid orcein-Giemsa, and periodic acid-Schiff (PAS}-alcian blue stain. Electron microscopic sections were stained with the uranyl acetate and lead citrate stain. The histopathologic findings on papules excised from the fingers and the palms were the same. Eight of the lesions from Patient 1 and one from Patient 2 showed changes of myxoid fibroma. The epidermis showed hyperkeratosis, acanthosis, and papillomatosis. The dermis exhibited capillary proliferation and markedly myxomatous tissue. The myxomatous connective tissue did not show elastic fibers and contained large amounts of acid mucopolysaccharides, demonstrated in sections stained by alcian blue and
PAS combination (Fig. 3). Biopsies of twelve of the lesions from Patient 1 and one from Patient 2 showed changes of vascular fibromas. Thus, fibrovascular proliferation was seen in the dermis, with or without acanthosis or hyperkeratosis of the overlying epidermis (Fig. 4). A few lesions from Patient 1 showed mainly verrucous hyperplasia of the epidermis with hypergranulosis and hyperkeratosis. There also were columnar areas of parakeratosis in connection with the tips of elongated papillae showing changes compatible with verruca VUlgaris. Electron microscopy. Electron microscopic examination of the epidermis revealed keratinocytes containing clumped tonofilaments and intracytoplasmic desmosomes. The tonofilaments were frequently associated with dense granules. Mitoses could also be seen. No evidence of wart virus was present (Figs. 5 and 6). Proliferating fibroblasts were seen in the dermis.
Vobmc 2 Number 5 ~1ay, 1980
Vascular and myxoid fibromas of fin[?('I"s
427
Fig. 4. A. Papillomatous, hyperkeratotic, acanthotic vascular fibroma. (Hematoxylin-eosin stain; X 12.) B. Higher-power view of A shows fibrovascular proliferation. (Hematoxylin-eosin stain; X 125.) These cells contained abundant ergastoplasm and an enlarged nucleus. Sometimes medium-sized granules of different size were visible in these cells. They probably represented colloid bodies which originate within the endoplasmic reticulum cisternae. Amorphous homogeneous rr.ucoid material was seen between the :ollagen fibrils (Figs. 7 ar:d 8). DISCUSSION
When the clinical and microscopic features are considered, the differential diagnosis of these two patients must include verruca vulgaris, epidermodysplasia verruciforrnis, acrokeratosis verruciform is , acquired digital fibrokeratoma, cutaneous myxoid cyst, and cutaneous focal mucinosis. Although these lesions resembled verrucae clinically, the histology of the majority of lesions from which biopsies were taken was not compati'ale with this diagnosis. Also, virus particles were not seen with electron microscopy. Epidermodysplasia verruciforrnis1-:l is a rare
familial disease that most often starts in childhood. 1t is characterized by cutaneous lesions that most often resemble confluent or discrete verruca plana or, in some instances, tinea versicolor-like lesions. Lesions tend to occur on the face, neck, hands, elbows, and knees but may also involve the trunk. The mode of inheritance is not clear. About 25% to 30% of patients, especially those with tinea versicolor-like lesions, may develop solar keratoses, Bowen's disease, or squamous cell carcinoma in some of the lesions. The histologic sections most often resemble flat warts. Electron microscopic examination of a lesion will show intranuclear viral particles compatible with wart virus. Our cases were familial, but the histologic changes of verruca plana were not present. Acrokeratosis verruciformis,j·5 is characterized by verrucous papules on the dorSum of the hands, on the feet, at times on the elb:Jws, and other areas. It is inheri:ed as an autosomal-dominant trait and may be associated with Darier's disease.
428
Coskey et (lJ
Journal of the American Academy of Dci'matolDgy
Fig. S. Acanthotic cell with dividing nucleus (N), several intracytoplasmic ctesmosomes (D). clumped tonofilaments (T), and high electron-dense granules (keratohyaline granules?) (Dgj, (Uranyl acetate and lead citrate stain; x8,400,)
Histologically, it does show hyperkeratosis, thickening of the granular layer, acanthosis, papillomatosis, and c:1Ufch spire-like elevations of the epidermis, The latter finding was not seen in our patients, Acquired digital fibrokeratorna 6 " has to be considered. This lesion usually is a solitary. hyperkeratotic, dome-shaped, elongated, peduncu-
lated growth involving the area of either the proximal or distal interphalangeal joint of the finger or rarely the toc, Histologic examination of the lesions shows hyperkeratosis and acanthosis surrounding centrally located areas of fibrous proliferation, Cutaneous rnyxoid cysts or synovial lesions of the fingers H,8 are usually solitary, They favor the
f'ig. 6. Numerous intracytoplasmic desmosomes (D). endoplasmic reticulum cisternae (Er). and intercellular spaces (Is), (Uranyl acetate and lead citrate stain; X22.200,) Fig. 7. Fibroblast with enlarged nucleus (N). extensive endoplasmic reticulum cisternae (Er). colloid bodies (eB). and elongated mitochondria (M), (Uranyl acetat.:: and ~ead citrate stain; x 19,530,)
Volume 2
Number 5 May. 1980
Vascular and my_wid /ibromas o.ffingers
Figs. 6 and 7. For legends. see opposite page_
429
Journal of the American Acade:-ny 0:
430 Coskey et o!
DenTIatology
Fig. 8. Several hyperplastic fibroblasts (F) In the dermal area, with a productive ergastoplasm (Er); collagen fibers (C) and mucoid material (Me) can be seen between them. (Uranyl acetate and lead citrate stain; x 15,500.)
periungual area of the fingers. They are soft, fluctuant, and smooth-surfaced. However, they may have a verrucous surface and be fairly firm. Their average size is approximately 6 mm. Histopathologic examination of these lesions shows replacement of the dennis by mucinous material, with an increase in the number of fibroblasts. Multiple c1eftlike spaces appear, coalescing to form macinfilled cysts. The mucin is hyaluronic acid. Our patients did have lesions that hist010gically had t..'le appearance of myxoid cysts. There also were many other lesions that showed only fibrous proliferation. Cutaneous focal mucinosis lO is usually characterized by a solitary, smooth-surfaced, white to skin-colored papular nodule which may be on the face, trunk, or extremities, and certainly does not resemble findings in our cases.
It appears that the cases we have described are unique and different from other acral papular lesions described in the dermatologic literature. We can best describe them as mUltiple familial vascular fibromas and myxoid fibromas of the hands. Since fibroblasts manufacture both co Hagen and ground substance, it is possible that this dermatosis is due to a local ized disturbance in fibroblast function. REFEREI\"CES ,. Jablonska S, Orth G. larzabec-Chorzelska M. et al: Epidermodyspbsia ver:-Llcifurmis versus disseminated verrucae pJanae. Is epid;.:rr:lOdysplasia verrucifonnis a generalized infection with ',vart virus? J Invest Dcrmatoi 72:114-119, 1979. 2. Rajagopalan K, BahrLl J, Loo DSC. et al: Familia! epidennodysplasia ven'ucifonnis of Lewandowsky and Lutz. Arch IXnnatol 105:73-78, 1972.
Volume 2 Number 5 May, 1980
3. Prawer SE, Pass F, Vance lC, et al: Depressed immune function in epidcrmodysplasia verruciformis. Arch Der· matol Jl3:495.499, 1977. 4. Schueller WA: Acrokeratosis verruciform is of Hopf. Arch Dermatol 106:81·83. 1972. 5. Herndon JH Jr, Wilson JD: Acrokeratosis verrucifor· mis (HopI) and Darier's disease. Arch Dermatol 93:
305·310. 1966.
6. Bart RS, Andrade R, Kopf A W, et al: Acquired digital fibrokeratoma. Arch Dermatol 97:120·129, 1968.
V{[scu/ar {/nd l1l)'xoid fibromas of fingers
431
7. Hare Pl. Smith PAl: Acquired (digital) fibrokeratoma. Br J Dermatol 81:667·670, 1969. 8. Johnson WC. Graham JH, Helwig FB: Cutaneous rnyxoid cyst. lAMA 191:15·20, 1979. 9. Bourns HK, Sanerkin WG: Mucoid lesions (mucoid cysts) of fingers and toes. Br J Surg 226:860-866. 1963. 10. Johnson WC, Helwig EB: Cutaneous focal mucinosis. Arch Dermatol 93:13·20, 1966.
Multiple vascular fibromas and myxoid fibromas of the fingers A histologic and ultrastructural study Ralph J. Coskey, M.D., Amir H. Meh;-egan, M.D., and AJrel P. Lupu1escu, M.D. Detroit, MI A father and his son were seen be.:ause of multiple wartlike lesions cn their fingers and palms. On histologic ex:::mination, these lesions showed changes compatible with vas.:ular fibromas, myxoid fibromas. and. in a few instances. verruca vulgaris. We are not aware of reports of similar cases in the dermatologic literature. (J AM ACAD DERMATOL 2:425-431, 1980.)
CASE REPORTS
Case 1 A 30-year-old white man was seen originally on Aug. 24, 1978. because of multiple verrucous papules on tis fingers and palms. He claimed that he had had these lesions for 16 years. He also stated that his father had had similar lesions for 25 years. Physical examination revealed approximately one hundred verrucous skin-colored to brown-red, warty papules, measuring 1 to 3 mm in diameter, on the dorsal and palmar a~pects of the fingers as well as the palms (Figs. 1 and 2). Over a period of many months, approximately fifty lesions were excised with either a curet or iris scissors, and the base of each of these sites was electrodesiccated. However, many of the lesions tended to recur.
Fig. 1. Multiple warty lesions are
~een
on the fingers.
Case 2 A 58-year-old white man, the father of Patient 1, was seen on March 12, 1979. because of multiple verrucous 1- to 3-mm papules on the palms as well as on the palrr:ar and dorsal aspects of the fingers. These lesions were clinically similar to those of his son. Biopsies of two lesions were obtained. Histopathology. Approximately twenty-two lesions excised from the fingers or plilms of Patient 1 were From the Department of Dermatology. Wayne Slate Cniversi:y. School of Medicine. Reprint requests ro: Dr. Ralph J. Caskey. 23! 33 Orchard Lake Rd .• Suite 20]. Farmington. MI 48024/313-476-4851.
:.1190-9622/80'050425+07500.70/0 © 1980 Am Acad Dermatol
Fig. 2. There are many verrucous papules in the area of :he distal interphalangeal joints.
425
426
CosKey et 0/
.loumal of the American Academy of OerlTla~ology
Fig. 3. A, Acanthosis, vascular proliferation, and myxomatous connective tissue are seen in the section. (Hematoxylin-eosin stain; x 125.) B, Multiple fibroblasts and large amounts of acid mucopolysaccharide are seen in the dermis. (Alcian blue-PAS stain; X400.)
examined by light microscopy and two lesions by electron microscopy. Two lesions excised from Patient 2 were also examined by light microscopy. Sections examined by light microscopy were stained with hematoxylin and eosin, acid orcein-Giemsa, and periodic acid-Schiff (PAS}-alcian blue stain. Electron microscopic sections were stained with the uranyl acetate and lead citrate stain. The histopathologic findings on papules excised from the fingers and the palms were the same. Eight of the lesions from Patient 1 and one from Patient 2 showed changes of myxoid fibroma. The epidermis showed hyperkeratosis, acanthosis, and papillomatosis. The dermis exhibited capillary proliferation and markedly myxomatous tissue. The myxomatous connective tissue did not show elastic fibers and contained large amounts of acid mucopolysaccharides, demonstrated in sections stained by alcian blue and
PAS combination (Fig. 3). Biopsies of twelve of the lesions from Patient 1 and one from Patient 2 showed changes of vascular fibromas. Thus, fibrovascular proliferation was seen in the dermis, with or without acanthosis or hyperkeratosis of the overlying epidermis (Fig. 4). A few lesions from Patient 1 showed mainly verrucous hyperplasia of the epidermis with hypergranulosis and hyperkeratosis. There also were columnar areas of parakeratosis in connection with the tips of elongated papillae showing changes compatible with verruca VUlgaris. Electron microscopy. Electron microscopic examination of the epidermis revealed keratinocytes containing clumped tonofilaments and intracytoplasmic desmosomes. The tonofilaments were frequently associated with dense granules. Mitoses could also be seen. No evidence of wart virus was present (Figs. 5 and 6). Proliferating fibroblasts were seen in the dermis.
Vobmc 2 Number 5 ~1ay, 1980
Vascular and myxoid fibromas of fin[?('I"s
427
Fig. 4. A. Papillomatous, hyperkeratotic, acanthotic vascular fibroma. (Hematoxylin-eosin stain; X 12.) B. Higher-power view of A shows fibrovascular proliferation. (Hematoxylin-eosin stain; X 125.) These cells contained abundant ergastoplasm and an enlarged nucleus. Sometimes medium-sized granules of different size were visible in these cells. They probably represented colloid bodies which originate within the endoplasmic reticulum cisternae. Amorphous homogeneous rr.ucoid material was seen between the :ollagen fibrils (Figs. 7 ar:d 8). DISCUSSION
When the clinical and microscopic features are considered, the differential diagnosis of these two patients must include verruca vulgaris, epidermodysplasia verruciforrnis, acrokeratosis verruciform is , acquired digital fibrokeratoma, cutaneous myxoid cyst, and cutaneous focal mucinosis. Although these lesions resembled verrucae clinically, the histology of the majority of lesions from which biopsies were taken was not compati'ale with this diagnosis. Also, virus particles were not seen with electron microscopy. Epidermodysplasia verruciforrnis1-:l is a rare
familial disease that most often starts in childhood. 1t is characterized by cutaneous lesions that most often resemble confluent or discrete verruca plana or, in some instances, tinea versicolor-like lesions. Lesions tend to occur on the face, neck, hands, elbows, and knees but may also involve the trunk. The mode of inheritance is not clear. About 25% to 30% of patients, especially those with tinea versicolor-like lesions, may develop solar keratoses, Bowen's disease, or squamous cell carcinoma in some of the lesions. The histologic sections most often resemble flat warts. Electron microscopic examination of a lesion will show intranuclear viral particles compatible with wart virus. Our cases were familial, but the histologic changes of verruca plana were not present. Acrokeratosis verruciformis,j·5 is characterized by verrucous papules on the dorSum of the hands, on the feet, at times on the elb:Jws, and other areas. It is inheri:ed as an autosomal-dominant trait and may be associated with Darier's disease.
428
Coskey et (lJ
Journal of the American Academy of Dci'matolDgy
Fig. S. Acanthotic cell with dividing nucleus (N), several intracytoplasmic ctesmosomes (D). clumped tonofilaments (T), and high electron-dense granules (keratohyaline granules?) (Dgj, (Uranyl acetate and lead citrate stain; x8,400,)
Histologically, it does show hyperkeratosis, thickening of the granular layer, acanthosis, papillomatosis, and c:1Ufch spire-like elevations of the epidermis, The latter finding was not seen in our patients, Acquired digital fibrokeratorna 6 " has to be considered. This lesion usually is a solitary. hyperkeratotic, dome-shaped, elongated, peduncu-
lated growth involving the area of either the proximal or distal interphalangeal joint of the finger or rarely the toc, Histologic examination of the lesions shows hyperkeratosis and acanthosis surrounding centrally located areas of fibrous proliferation, Cutaneous rnyxoid cysts or synovial lesions of the fingers H,8 are usually solitary, They favor the
f'ig. 6. Numerous intracytoplasmic desmosomes (D). endoplasmic reticulum cisternae (Er). and intercellular spaces (Is), (Uranyl acetate and lead citrate stain; X22.200,) Fig. 7. Fibroblast with enlarged nucleus (N). extensive endoplasmic reticulum cisternae (Er). colloid bodies (eB). and elongated mitochondria (M), (Uranyl acetat.:: and ~ead citrate stain; x 19,530,)
Volume 2
Number 5 May. 1980
Vascular and my_wid /ibromas o.ffingers
Figs. 6 and 7. For legends. see opposite page_
429
Journal of the American Acade:-ny 0:
430 Coskey et o!
DenTIatology
Fig. 8. Several hyperplastic fibroblasts (F) In the dermal area, with a productive ergastoplasm (Er); collagen fibers (C) and mucoid material (Me) can be seen between them. (Uranyl acetate and lead citrate stain; x 15,500.)
periungual area of the fingers. They are soft, fluctuant, and smooth-surfaced. However, they may have a verrucous surface and be fairly firm. Their average size is approximately 6 mm. Histopathologic examination of these lesions shows replacement of the dennis by mucinous material, with an increase in the number of fibroblasts. Multiple c1eftlike spaces appear, coalescing to form macinfilled cysts. The mucin is hyaluronic acid. Our patients did have lesions that hist010gically had t..'le appearance of myxoid cysts. There also were many other lesions that showed only fibrous proliferation. Cutaneous focal mucinosis lO is usually characterized by a solitary, smooth-surfaced, white to skin-colored papular nodule which may be on the face, trunk, or extremities, and certainly does not resemble findings in our cases.
It appears that the cases we have described are unique and different from other acral papular lesions described in the dermatologic literature. We can best describe them as mUltiple familial vascular fibromas and myxoid fibromas of the hands. Since fibroblasts manufacture both co Hagen and ground substance, it is possible that this dermatosis is due to a local ized disturbance in fibroblast function. REFEREI\"CES ,. Jablonska S, Orth G. larzabec-Chorzelska M. et al: Epidermodyspbsia ver:-Llcifurmis versus disseminated verrucae pJanae. Is epid;.:rr:lOdysplasia verrucifonnis a generalized infection with ',vart virus? J Invest Dcrmatoi 72:114-119, 1979. 2. Rajagopalan K, BahrLl J, Loo DSC. et al: Familia! epidennodysplasia ven'ucifonnis of Lewandowsky and Lutz. Arch IXnnatol 105:73-78, 1972.
Volume 2 Number 5 May, 1980
3. Prawer SE, Pass F, Vance lC, et al: Depressed immune function in epidcrmodysplasia verruciformis. Arch Der· matol Jl3:495.499, 1977. 4. Schueller WA: Acrokeratosis verruciform is of Hopf. Arch Dermatol 106:81·83. 1972. 5. Herndon JH Jr, Wilson JD: Acrokeratosis verrucifor· mis (HopI) and Darier's disease. Arch Dermatol 93:
305·310. 1966.
6. Bart RS, Andrade R, Kopf A W, et al: Acquired digital fibrokeratoma. Arch Dermatol 97:120·129, 1968.
V{[scu/ar {/nd l1l)'xoid fibromas of fingers
431
7. Hare Pl. Smith PAl: Acquired (digital) fibrokeratoma. Br J Dermatol 81:667·670, 1969. 8. Johnson WC. Graham JH, Helwig FB: Cutaneous rnyxoid cyst. lAMA 191:15·20, 1979. 9. Bourns HK, Sanerkin WG: Mucoid lesions (mucoid cysts) of fingers and toes. Br J Surg 226:860-866. 1963. 10. Johnson WC, Helwig EB: Cutaneous focal mucinosis. Arch Dermatol 93:13·20, 1966.