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Alopecia associated with underlying nerve sheath myxoma
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Alopecia associated with underlying nerve sheath myxoma John M. Burket, M.D. Portland, OR This report describes a 16-year-old girl who was referred for a 2.5-cm macular area of alopecia of the posterior portion of the scalp. A subcutaneous nerve sheath myxoma was found underlying the area of alopecia. Hair growth resumed within 4 weeks after removal of the nerve sheath tumor. (J AM ACAD DERMATOL 1987; 16:209-11.)
A localized area of alopecia in a child can be a challenge in diagnosis. The differential diagnosis may include a number of possibilities, including tinea, alopecia areata, trichotillomania, and morphea. The case discussed here was associated with an underlying uncommon tumor of the skin. In 1969 Harkin and Reed ~ described a dermal myxoid tumor that they believed to be of nerve sheath origin. They separated this tumor from myxoid variants of neurofibroma and named it "nerve sheath myxoma." Gallager and Helwig, 2 in 1980, reported fifty-three similar cases under the title "neurothekeoma." Although they admitted some nosologic overlap of the tumor that they were reporting and nerve sheath myxoma, they believed that the latter term should be reserved for lesions that more commonly occur in soft tissue, with lobules surrounded by a thickened perineurium and possessing a more abundant mucinous matrix. However, in 1983 Enzinger and Weiss, ~ from the same institution, considered these two entities to be the same and termed them "benign myxoid tumor of nerve sheath." The writers cited in the previous paragraphs point out that the tumors usually arise during childhood and early adult life and tend to occur on the upper portion of the body, especially the head, neck, and shoulders. There is a marked fe-
From the Department of Dermatology, Oregon Health Sciences University. Reprint requests to: Dr. John M. Burket, 1000 East Main St., Medford, OR 97504.
male preponderance, with a female/male ratio of 4.3:1. The tumors are located in the dermis and subcutis and have not been identified in deep soft tissue. Histologically the tumor is divided into distinct lobules by fibrous connective tissue that has been compared to perineurium. The cells making up the lobules are spindled, rounded, or stellate, lying singly or in small groups within a myxoid matrix. Benign giant ceils are sometimes present, and neurites can occasionally be identified among the tumor cells. The tumors were invariably benign. Of the fifty-three cases of Gallager and Helwig, 2 only two recurred, and they had been inadequately excised. CASE REPORT
A 16-year-old girl complained of an area of alopecia on the midposterior portion of the scalp of 2 months' duration (Fig. 1). For the previous 3 to 4 years she had been able, with deep palpation, to feel a lump in the area. Although there was some tenderness, the patient denied any history of trauma, rubbing, pulling, or other manipulation of the area. The patient had a 2.5-cm macular alopecic area in the midline of the posterior part of the scalp. The skin was under no increased tension, nor was any lichenification, stubble, or exclamation-point hairs found. A potassium hydroxide examination and fungal culture had negative findings for fungus. One could discern a mildly tender subcutaneous lesion that was clinically suggestive of a lymph node. Card au lait spots, hypopigmented macules, neurofibromas, and other skin lesions were not found. Touch and temperature sensations were normal. The skin was incised, and a subcutaneous structure, 209
210
Journal of the American Academy of Dermatology
Burket
Fig. 1. Macular alopecia, 2.5 cm in area, on the posterior portion of the occiput. Fig. 2. Lobule of spindled and stellate cells. A fibrous capsule is seen at upper left. (Hematoxylin-eosin stain; x 100.) Fig. 3. Multinucleated giant cell "ingesting" mucosubstance (mucosubstance, blue; nuclei, red). (Colloidal iron stain; × 1,000.)
2 cm in diameter, was carefully dissected from the surrounding fat. The wound was closed primarily. Within 4 weeks after removal of the tumor, hair was regrowing in the area of alopecia. Study of hematoxylin-and-eosin-stained sections revealed a lobulated cystic tumor surrounded by a thick, fibrous capsule and subdivided by similar connective tissue-appearing septae into lobules. The lobules were either composed of, or rimmed by, spindled, rounded, and stellate cells lying within a myxoid matrix (Fig. 2). The mucinous material stained positively with colloidal iron stain. Careful examination of the septae failed to reveal cross striations, and they stained with Gomori's trichrome stain, as one would expect for con-
nective tissue. Occasional groups of foreign body giant ceils, some of which appeared to be engulfing mucin, were seen (Fig. 3). Occasional neurites could be identified among the tumor cells with the Hirano-Zimmerman method for nerve cells and fibers. DISCUSSION Although the tumor's subcutaneous location, prominent cystic-mucinous component, and thickened perineural-like connective tissue fit well with the concept of Gallager and Helwig 2 concerning nerve sheath myxoma, other entities were considered in the microscopic differential diagnosis of this lesion. The lack of muscle ,tissue ruled out an
Volume 16 Number 1, Part 2 January 1987
intramuscular myxoma, and the absence of lipocytes led one away from a diagnosis of myxolipoma. Juxta-articular myxoma usually occurs around the knee joints of older men and would be expected to contain synovium and adipose tissue. Although myxoid change may occur in a neurofibroma, one would not expect to see the nests of cells and mucoid matrix separated by fibrous septae that this tumor demonstrated. The subcutaneous location, presence of mucopolysaccharide matrix, and lack of melanin made a neuronevus unlikely. A mucin-filled ganglion is sometimes found within the perineurium of peripheral nerves; however, the reported cases have usually involved the peroneal and radial nerves. Further, the lobules in
Alopecia with nerve sheath myxoma
this patient's tumor contained a large cellular component not seen in neural mucin ganglion cysts. The depth and extent of the lesion, along with the fibrous capsule and septae, argued against focal mucinosis. Finally, since the well-differentiated cells lacked atypicality, a diagnosis of malignant myxomatous process was not entertained. REFERENCES 1. Harkin JC, Reed RJ: Tumors of the peripheral nervous system. Washington, DC, 1969, Armed Forces Institute of Pathology, pp. 60-64. (Atlas of tumor pathology, Second Series, Fasiele 3.) 2. Gallager RL, He/wig EG: Neurothekeoma: A benign cutaneous tumor of neural origin. Am J C/in Pathol 74:759764, 1980. 3. Enzinger FM, Weiss SW: Soft tissue tumors. St. Louis, 1983, The C.V. Mosby Co., pp. 615-617.
Argyria: The intradermal "photograph," manifestation of passive photosensitivity
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Walter B. Shelley, M.D. ,* E. Dorinda Shelley, M.D. ,* and Veronika Burmeister** Toledo, OH, and Peoria, IL X-ray microprobe and electron microscopic study was made of the remarkable blue-black pigmentation that sunlight elicits in patients with argyria. The patient under study had developed argyria following injection of silver nitrate as a sclerosant into his varicose veins 41 years ago. Similarities are demonstrated between the darkening of the skin and the darkening of a photographic film following light exposure. In both instances, colorless silver salts and compounds present in an inert matrix (collagen versus gelatin) are reduced by incident light to black metallic silver. This passive photosensitivity reaction leads to silver tattooing of the light-exposed skin and to photographic imaging in the film. (J AM ACADDERMATOL1987;16:211-7.)
Because of the widespread use of the protoantibiotic silver protein (Argyrol), argyria became
From the Division of Dermatology,Departmentof Medicine, Medical College of Ohio,* and the Departmentof Dermatology,University of Illinois College of Medicine at Peoria.** Support provided by Neutrogena Corporation, Los Angeles, CA. Reprint requests to: Dr, Walter B. Shelley, Division of Dermatology, Medical College of Ohio, C,S. 10008, Toledo, OH 43699.
one of the most common iatrogenic diseases following the turn of this century. The permanence and irreversibility of its blue-black color ensured an ever-expanding pool of patients as long as Argyrol was available. ~ Today, in the absence of Argyrol, argyria is mainly a curiosity seen in older workers in silver refinery plants 2 or in older individuals who had been treated for syphilis with silver preparations such as arsphenamine (Salvar211
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II I
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Alopecia associated with underlying nerve sheath myxoma John M. Burket, M.D. Portland, OR This report describes a 16-year-old girl who was referred for a 2.5-cm macular area of alopecia of the posterior portion of the scalp. A subcutaneous nerve sheath myxoma was found underlying the area of alopecia. Hair growth resumed within 4 weeks after removal of the nerve sheath tumor. (J AM ACAD DERMATOL 1987; 16:209-11.)
A localized area of alopecia in a child can be a challenge in diagnosis. The differential diagnosis may include a number of possibilities, including tinea, alopecia areata, trichotillomania, and morphea. The case discussed here was associated with an underlying uncommon tumor of the skin. In 1969 Harkin and Reed ~ described a dermal myxoid tumor that they believed to be of nerve sheath origin. They separated this tumor from myxoid variants of neurofibroma and named it "nerve sheath myxoma." Gallager and Helwig, 2 in 1980, reported fifty-three similar cases under the title "neurothekeoma." Although they admitted some nosologic overlap of the tumor that they were reporting and nerve sheath myxoma, they believed that the latter term should be reserved for lesions that more commonly occur in soft tissue, with lobules surrounded by a thickened perineurium and possessing a more abundant mucinous matrix. However, in 1983 Enzinger and Weiss, ~ from the same institution, considered these two entities to be the same and termed them "benign myxoid tumor of nerve sheath." The writers cited in the previous paragraphs point out that the tumors usually arise during childhood and early adult life and tend to occur on the upper portion of the body, especially the head, neck, and shoulders. There is a marked fe-
From the Department of Dermatology, Oregon Health Sciences University. Reprint requests to: Dr. John M. Burket, 1000 East Main St., Medford, OR 97504.
male preponderance, with a female/male ratio of 4.3:1. The tumors are located in the dermis and subcutis and have not been identified in deep soft tissue. Histologically the tumor is divided into distinct lobules by fibrous connective tissue that has been compared to perineurium. The cells making up the lobules are spindled, rounded, or stellate, lying singly or in small groups within a myxoid matrix. Benign giant ceils are sometimes present, and neurites can occasionally be identified among the tumor cells. The tumors were invariably benign. Of the fifty-three cases of Gallager and Helwig, 2 only two recurred, and they had been inadequately excised. CASE REPORT
A 16-year-old girl complained of an area of alopecia on the midposterior portion of the scalp of 2 months' duration (Fig. 1). For the previous 3 to 4 years she had been able, with deep palpation, to feel a lump in the area. Although there was some tenderness, the patient denied any history of trauma, rubbing, pulling, or other manipulation of the area. The patient had a 2.5-cm macular alopecic area in the midline of the posterior part of the scalp. The skin was under no increased tension, nor was any lichenification, stubble, or exclamation-point hairs found. A potassium hydroxide examination and fungal culture had negative findings for fungus. One could discern a mildly tender subcutaneous lesion that was clinically suggestive of a lymph node. Card au lait spots, hypopigmented macules, neurofibromas, and other skin lesions were not found. Touch and temperature sensations were normal. The skin was incised, and a subcutaneous structure, 209
210
Journal of the American Academy of Dermatology
Burket
Fig. 1. Macular alopecia, 2.5 cm in area, on the posterior portion of the occiput. Fig. 2. Lobule of spindled and stellate cells. A fibrous capsule is seen at upper left. (Hematoxylin-eosin stain; x 100.) Fig. 3. Multinucleated giant cell "ingesting" mucosubstance (mucosubstance, blue; nuclei, red). (Colloidal iron stain; × 1,000.)
2 cm in diameter, was carefully dissected from the surrounding fat. The wound was closed primarily. Within 4 weeks after removal of the tumor, hair was regrowing in the area of alopecia. Study of hematoxylin-and-eosin-stained sections revealed a lobulated cystic tumor surrounded by a thick, fibrous capsule and subdivided by similar connective tissue-appearing septae into lobules. The lobules were either composed of, or rimmed by, spindled, rounded, and stellate cells lying within a myxoid matrix (Fig. 2). The mucinous material stained positively with colloidal iron stain. Careful examination of the septae failed to reveal cross striations, and they stained with Gomori's trichrome stain, as one would expect for con-
nective tissue. Occasional groups of foreign body giant ceils, some of which appeared to be engulfing mucin, were seen (Fig. 3). Occasional neurites could be identified among the tumor cells with the Hirano-Zimmerman method for nerve cells and fibers. DISCUSSION Although the tumor's subcutaneous location, prominent cystic-mucinous component, and thickened perineural-like connective tissue fit well with the concept of Gallager and Helwig 2 concerning nerve sheath myxoma, other entities were considered in the microscopic differential diagnosis of this lesion. The lack of muscle ,tissue ruled out an
Volume 16 Number 1, Part 2 January 1987
intramuscular myxoma, and the absence of lipocytes led one away from a diagnosis of myxolipoma. Juxta-articular myxoma usually occurs around the knee joints of older men and would be expected to contain synovium and adipose tissue. Although myxoid change may occur in a neurofibroma, one would not expect to see the nests of cells and mucoid matrix separated by fibrous septae that this tumor demonstrated. The subcutaneous location, presence of mucopolysaccharide matrix, and lack of melanin made a neuronevus unlikely. A mucin-filled ganglion is sometimes found within the perineurium of peripheral nerves; however, the reported cases have usually involved the peroneal and radial nerves. Further, the lobules in
Alopecia with nerve sheath myxoma
this patient's tumor contained a large cellular component not seen in neural mucin ganglion cysts. The depth and extent of the lesion, along with the fibrous capsule and septae, argued against focal mucinosis. Finally, since the well-differentiated cells lacked atypicality, a diagnosis of malignant myxomatous process was not entertained. REFERENCES 1. Harkin JC, Reed RJ: Tumors of the peripheral nervous system. Washington, DC, 1969, Armed Forces Institute of Pathology, pp. 60-64. (Atlas of tumor pathology, Second Series, Fasiele 3.) 2. Gallager RL, He/wig EG: Neurothekeoma: A benign cutaneous tumor of neural origin. Am J C/in Pathol 74:759764, 1980. 3. Enzinger FM, Weiss SW: Soft tissue tumors. St. Louis, 1983, The C.V. Mosby Co., pp. 615-617.
Argyria: The intradermal "photograph," manifestation of passive photosensitivity
a
Walter B. Shelley, M.D. ,* E. Dorinda Shelley, M.D. ,* and Veronika Burmeister** Toledo, OH, and Peoria, IL X-ray microprobe and electron microscopic study was made of the remarkable blue-black pigmentation that sunlight elicits in patients with argyria. The patient under study had developed argyria following injection of silver nitrate as a sclerosant into his varicose veins 41 years ago. Similarities are demonstrated between the darkening of the skin and the darkening of a photographic film following light exposure. In both instances, colorless silver salts and compounds present in an inert matrix (collagen versus gelatin) are reduced by incident light to black metallic silver. This passive photosensitivity reaction leads to silver tattooing of the light-exposed skin and to photographic imaging in the film. (J AM ACADDERMATOL1987;16:211-7.)
Because of the widespread use of the protoantibiotic silver protein (Argyrol), argyria became
From the Division of Dermatology,Departmentof Medicine, Medical College of Ohio,* and the Departmentof Dermatology,University of Illinois College of Medicine at Peoria.** Support provided by Neutrogena Corporation, Los Angeles, CA. Reprint requests to: Dr, Walter B. Shelley, Division of Dermatology, Medical College of Ohio, C,S. 10008, Toledo, OH 43699.
one of the most common iatrogenic diseases following the turn of this century. The permanence and irreversibility of its blue-black color ensured an ever-expanding pool of patients as long as Argyrol was available. ~ Today, in the absence of Argyrol, argyria is mainly a curiosity seen in older workers in silver refinery plants 2 or in older individuals who had been treated for syphilis with silver preparations such as arsphenamine (Salvar211