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Discussion of questions 20–29
Journal of the American Academy of Dermatology Volume 35, Number 5, Part 1
Longley J. Is mastocytosis a mast cell neoplasia or a reactive hyperplasia? Clues from the study of mast cell growth factor. Ann Med 1994;26:115-6. Oku T, Hashizume H, Yokote R, et al. The familial occurrence of bullous mastocytosis (diffuse cutaneous mastocytosis). Arch Dermatol 1990;126:1478-84.
Self-Assessment examination answers
799
Orkine M, Good RA, Clawson CC, et al. Bullous mastocytosis. Arch Dermatol 1970; 101:547-64. Soter NA. The skin in mastocytosis. J Invest Dermatol 1991; 96(suppl):32s-39s.
DISCUSSION OF QUESTIONS 11-19 Acute febrile neutrophilic dermatosis, or Sweet's syndrome, was described in 1964 by British dermatologist R. D. Sweet. Painful and tender deep-red plaques are most common on the face, neck, and extremities. Skin lesions are accompanied by fever, malaise, and peripheral neutrophilic leukocytosis. The distinctive histologic feature is a dense dermal infiltrate composed of mature neutrophils without frank vasculitis. Eighty percent of patients are women. Systemic corticosteroids offer rapid alleviation of symptoms. Relapse occurs in 25% to 50% of treated patients. The cause of Sweet's syndrome is unknown, although evidence suggests an immunologic-hypersensitivity mechanism. In support of this hypothesis, typical lesions have been induced at the sites of intradermal injection of streptococcal and candidal antigens. Extracutaneous manifestations, including conjunctivitis or episcleritis (30% to 70% of patients), migratory polyarthralgia or arthritis (20% to 50%), and hepatic or renal dysfunction (10% to 15%), may occur before, at the same time as, or after the onset of the skin lesions. Signs and symptoms of inflammatory bowel disease may occur. Sweet's syndrome has been reported to occur after upper respiratory or gastrointestinal tract infection, connective tissue diseases, thyroiditis, vaccinations, and pregnancy. Sweet's syndrome has also been associated with malignancy. Seven percent of reported cases are associated with solid minors, and more than 10% are
associated with hematologic malignancy, most commonly acute myeloid and myetomonocytic leukemias. The cutaneous lesions may occur before or during the course of the leukemia, during remission, or as an indication of relapse. Vesicobullous, necrotic, and ulcerative lesions are more common in association with leukemia. Atypical lesions are more likely to arise on mucous membranes and at sites of trauma. The cutaneous infiltrate consists primarily of mature neutrophils. In the patient with leukemia, treatment other than corticosteroids may avoid infectious complications. Indomethacin, naproxen, potassium iodide, dapsone, and colchicine may be effective. For this series, the recommended choices are as follows: 11, c; 12, a; 13, d; 14, d; 15, e; 16, a; 17, e; 18, e; 19, e. Laura J. Haygood, MD Ted Rosen, MD Houston, Texas
DISCUSSION OF QUESTIONS 20-29
BIBLIOGRAPHY
Case 20. Chondrodermatitis nodularis helicis (c) Main histologic features • Localized acanthosis • Fibrinoid necrosis of papillary dermis • Granulation tissue • Perichondritis
BIBLIOGRAPHY
Chan HL, Lee YS, Kuo TT. Sweet's syndrome: clinicopathologic study of eleven cases. Int J Dermatol 1994;33:425-32. Cohen PR, Kurzrock R. Sweet's syndrome and cancer. Clin Dermatol 1993; 11:149-57. Kemmett D, Hunter JA. Sweet's syndrome: a clinicopathologic review of twenty-nine cases. J Am Acad Dermatol 1990; 23503-7. Su WPD, Liu HN. Diagnostic criteria for Sweet's syndrome. Curls 1986;37:167-74. von den Dfiesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994;31:535-56.
Bottomley WW, Goodfield MD. Chondrodermatitis nodularis helicis occurring with systemic sclerosis: An under-reported association? Clin Exp Dermatol 1994;19:219-20. Graham JH, Johnson WC, Helwig EB. Dermal pathology. Hagerstown, MD: Harper and Row, 1972:773-4.
Case 21. TrichoepitheUoma
(d)
Main histologic features • Epithelial islands of basaloid cells in a trabecular pattern
Journal of the American Academy of Dermatology
800
Self-Assessment examination answers
November 1996
• Fibrotic stroma surrounding islands • Keratinaceous cysts
• Increased dermal hyaluronic acid • Orthohyperkeratosis
BIBLIOGRAPHY
BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Linage, 1990:596-9. Smith KJ, Skelton HG, Holland TT. Recent advances and controversies concerning adnexal neoplasms. Dermatol Clin 1992; 10:117-60.
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:113-8. George R, Kurian S, Jacob M, et al. Diagnostic evaluation of the lupus band test in discoid and systemic lupus erythematosus. Int J Dermatol 1995;34:170-3.
Case 22. Lichen planus (a)
Case 26. Hidrocystoma (b)
Main histologic features • Bandlike lymphocyte infiltrate in papillary dermis • Basal layer vacuolization • Orthohyperkeratosis
Main histologic features • Variably shaped dermal cyst lined by one ortwo rows of cuboidal epithelial cells • In areas with apocrine differentiation, an outer layer of myoepithelial cells may be present.
BIBLIOGRAPHY
BIBLIOGRAPHY
Akasu R, From L, Kahn HJ. Lymphocyte and macrophage subsets in active and inactive lesions of lichen planus. Am J Dermatopathol 1993; 15:217-23. Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990;98-100.
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:624-5. Thorisdottir K, Maloney ME. Stump the experts: apocrine hidrocystoma. Dermatol Surg 1995;21:122, 188.
Case 23. Pilomatrixoma (b)
Case 27. Transient acantholytic dermatosis
(c)
Main histologic features • Circumscribed cystlike amaor of uniform basaloid cells • Anucleate shadow cells • Dystrophic calcification
Main histologic features • Focal area of basilar to upper epidermal acantholysis • Variable parakeratosis • Variable acanthosis
BIBLIOGRAPHY
BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk: Appleton-Lange, 1990:605-7. Kaddu S, Soyer HP, Cerroni L, et al. Clinical and histopathologic specmma of pilomatricomas in adults. Int J Dermatol 1994;33:705-8.
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:624-5. Quarterman MJ, Davis LS. Transient acantholytic dermatosis in a postoperative febrile patient. Int J Dermatol 1995;34:113.
Case 24. Porokeratosis (c) Main histologic features • Cornoid lamella • Variable epidermal apoptosis beneath comoid lamella BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:403-5. Kanitakis J, Misery L, Nicolas JF, et al. Disseminated superficial porokeratosis in a patient with AIDS. Br J Derrnatol 1994; 131:284-9.
Case 25. Lupus erythematosus (c) Main histologic features • Variable epidermal atrophy • Basal cell layer vacuolization • Variable epidermal layer apoptosis • Superficial and deep perivascular and periadnexal lymphocytic infiltrate
Case 28. Scabies (a) Main histologic feature • Female mite in the space between the stratum c o m e u m and granular cell layer BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:392-3. Schlesinger I, Oelrich DM, Tyring SK. Crested (Norwegian) scabies in patients with AIDS: the range of clinical presentations. South Med J 1994;87:352-6.
Case 29. Keloid (d) Main histologic feature • Dermal whorls and bundles of hyalinized collagen BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:414. Sahl WJ Jr, Clever H. Cutaneous scars: part II. Int J Dermatol 1994;33:763-9.
Longley J. Is mastocytosis a mast cell neoplasia or a reactive hyperplasia? Clues from the study of mast cell growth factor. Ann Med 1994;26:115-6. Oku T, Hashizume H, Yokote R, et al. The familial occurrence of bullous mastocytosis (diffuse cutaneous mastocytosis). Arch Dermatol 1990;126:1478-84.
Self-Assessment examination answers
799
Orkine M, Good RA, Clawson CC, et al. Bullous mastocytosis. Arch Dermatol 1970; 101:547-64. Soter NA. The skin in mastocytosis. J Invest Dermatol 1991; 96(suppl):32s-39s.
DISCUSSION OF QUESTIONS 11-19 Acute febrile neutrophilic dermatosis, or Sweet's syndrome, was described in 1964 by British dermatologist R. D. Sweet. Painful and tender deep-red plaques are most common on the face, neck, and extremities. Skin lesions are accompanied by fever, malaise, and peripheral neutrophilic leukocytosis. The distinctive histologic feature is a dense dermal infiltrate composed of mature neutrophils without frank vasculitis. Eighty percent of patients are women. Systemic corticosteroids offer rapid alleviation of symptoms. Relapse occurs in 25% to 50% of treated patients. The cause of Sweet's syndrome is unknown, although evidence suggests an immunologic-hypersensitivity mechanism. In support of this hypothesis, typical lesions have been induced at the sites of intradermal injection of streptococcal and candidal antigens. Extracutaneous manifestations, including conjunctivitis or episcleritis (30% to 70% of patients), migratory polyarthralgia or arthritis (20% to 50%), and hepatic or renal dysfunction (10% to 15%), may occur before, at the same time as, or after the onset of the skin lesions. Signs and symptoms of inflammatory bowel disease may occur. Sweet's syndrome has been reported to occur after upper respiratory or gastrointestinal tract infection, connective tissue diseases, thyroiditis, vaccinations, and pregnancy. Sweet's syndrome has also been associated with malignancy. Seven percent of reported cases are associated with solid minors, and more than 10% are
associated with hematologic malignancy, most commonly acute myeloid and myetomonocytic leukemias. The cutaneous lesions may occur before or during the course of the leukemia, during remission, or as an indication of relapse. Vesicobullous, necrotic, and ulcerative lesions are more common in association with leukemia. Atypical lesions are more likely to arise on mucous membranes and at sites of trauma. The cutaneous infiltrate consists primarily of mature neutrophils. In the patient with leukemia, treatment other than corticosteroids may avoid infectious complications. Indomethacin, naproxen, potassium iodide, dapsone, and colchicine may be effective. For this series, the recommended choices are as follows: 11, c; 12, a; 13, d; 14, d; 15, e; 16, a; 17, e; 18, e; 19, e. Laura J. Haygood, MD Ted Rosen, MD Houston, Texas
DISCUSSION OF QUESTIONS 20-29
BIBLIOGRAPHY
Case 20. Chondrodermatitis nodularis helicis (c) Main histologic features • Localized acanthosis • Fibrinoid necrosis of papillary dermis • Granulation tissue • Perichondritis
BIBLIOGRAPHY
Chan HL, Lee YS, Kuo TT. Sweet's syndrome: clinicopathologic study of eleven cases. Int J Dermatol 1994;33:425-32. Cohen PR, Kurzrock R. Sweet's syndrome and cancer. Clin Dermatol 1993; 11:149-57. Kemmett D, Hunter JA. Sweet's syndrome: a clinicopathologic review of twenty-nine cases. J Am Acad Dermatol 1990; 23503-7. Su WPD, Liu HN. Diagnostic criteria for Sweet's syndrome. Curls 1986;37:167-74. von den Dfiesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol 1994;31:535-56.
Bottomley WW, Goodfield MD. Chondrodermatitis nodularis helicis occurring with systemic sclerosis: An under-reported association? Clin Exp Dermatol 1994;19:219-20. Graham JH, Johnson WC, Helwig EB. Dermal pathology. Hagerstown, MD: Harper and Row, 1972:773-4.
Case 21. TrichoepitheUoma
(d)
Main histologic features • Epithelial islands of basaloid cells in a trabecular pattern
Journal of the American Academy of Dermatology
800
Self-Assessment examination answers
November 1996
• Fibrotic stroma surrounding islands • Keratinaceous cysts
• Increased dermal hyaluronic acid • Orthohyperkeratosis
BIBLIOGRAPHY
BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Linage, 1990:596-9. Smith KJ, Skelton HG, Holland TT. Recent advances and controversies concerning adnexal neoplasms. Dermatol Clin 1992; 10:117-60.
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:113-8. George R, Kurian S, Jacob M, et al. Diagnostic evaluation of the lupus band test in discoid and systemic lupus erythematosus. Int J Dermatol 1995;34:170-3.
Case 22. Lichen planus (a)
Case 26. Hidrocystoma (b)
Main histologic features • Bandlike lymphocyte infiltrate in papillary dermis • Basal layer vacuolization • Orthohyperkeratosis
Main histologic features • Variably shaped dermal cyst lined by one ortwo rows of cuboidal epithelial cells • In areas with apocrine differentiation, an outer layer of myoepithelial cells may be present.
BIBLIOGRAPHY
BIBLIOGRAPHY
Akasu R, From L, Kahn HJ. Lymphocyte and macrophage subsets in active and inactive lesions of lichen planus. Am J Dermatopathol 1993; 15:217-23. Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990;98-100.
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:624-5. Thorisdottir K, Maloney ME. Stump the experts: apocrine hidrocystoma. Dermatol Surg 1995;21:122, 188.
Case 23. Pilomatrixoma (b)
Case 27. Transient acantholytic dermatosis
(c)
Main histologic features • Circumscribed cystlike amaor of uniform basaloid cells • Anucleate shadow cells • Dystrophic calcification
Main histologic features • Focal area of basilar to upper epidermal acantholysis • Variable parakeratosis • Variable acanthosis
BIBLIOGRAPHY
BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk: Appleton-Lange, 1990:605-7. Kaddu S, Soyer HP, Cerroni L, et al. Clinical and histopathologic specmma of pilomatricomas in adults. Int J Dermatol 1994;33:705-8.
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:624-5. Quarterman MJ, Davis LS. Transient acantholytic dermatosis in a postoperative febrile patient. Int J Dermatol 1995;34:113.
Case 24. Porokeratosis (c) Main histologic features • Cornoid lamella • Variable epidermal apoptosis beneath comoid lamella BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:403-5. Kanitakis J, Misery L, Nicolas JF, et al. Disseminated superficial porokeratosis in a patient with AIDS. Br J Derrnatol 1994; 131:284-9.
Case 25. Lupus erythematosus (c) Main histologic features • Variable epidermal atrophy • Basal cell layer vacuolization • Variable epidermal layer apoptosis • Superficial and deep perivascular and periadnexal lymphocytic infiltrate
Case 28. Scabies (a) Main histologic feature • Female mite in the space between the stratum c o m e u m and granular cell layer BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:392-3. Schlesinger I, Oelrich DM, Tyring SK. Crested (Norwegian) scabies in patients with AIDS: the range of clinical presentations. South Med J 1994;87:352-6.
Case 29. Keloid (d) Main histologic feature • Dermal whorls and bundles of hyalinized collagen BIBLIOGRAPHY
Farmer ER, Hood AF, editors. Pathology of the skin. Norwalk, CT: Appleton-Lange, 1990:414. Sahl WJ Jr, Clever H. Cutaneous scars: part II. Int J Dermatol 1994;33:763-9.