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Progressive eruptive histiocytomas
Progressive eruptive histiocytomas C D R Neil F. Gibbs, MC, USN, and CAPT Terence C. O'Grady, MC, USN Camp Pendleton
and San Diego, California We describe progressive eruptive lesions that have been appearing on a 50-year-old Filipino man for the past 9 years. They are characterized by smooth, brown, firm nodules mainly on the head and torso. Histologic examination shows dermal collections of lipid-filled histiocytes and Touton-type giant cells. A comparison with other types of previously described non-Langerhans cell histiocytoses is made. (J Am Acad Dermatol 1996;35:323-5.) The histiocytoses are a diverse group of conditions. W e describe a patient who does not easily fit into the present classification.
CASE REPORT A 50-year-old Filipino man had the progressive appearance of 2 to 5 mm firm, smooth, brown papules and nodules on his face, arms, and torso for 9 years (Fig. 1). The lesions grew to their final size in 6 to 12 months and did not change thereafter. The patient has non-insulin-dependent diabetes mellims treated with glyburide. A blood lipid panel showed normal cholesterol and high-density lipoprotein values and a slightly elevated trigiyceride level (234 mg/dl). X-ray examination of the skull showed no enlargement of the sella mrcica. Biopsy specimens showed well-circumscribed but not encapsulated collections of histiocytes with multiple Touton-type giant cells within the dermis (Figs. 2 and 3). The infiltrate splayed collagen bundles at the edges, producing an appearance of "collagen trapping." The histiocytes in the dermal infiltrate were not "foamy," but Oil red O staining of fresh frozen sections showed fipid deposition within them. An immunoperoxidase study showed that the histiocytes were negative for S-100 protein and factor XIII. They were positive for vimentin. Electron microscopy showed no Birbeck granules or Caputo bodies in the cytoplasm of the cells, and fipid droplets were not prominent. ORTHO
This article is made possible through an educational grant from the Dermatological Division, Ortho Pharmaceutical Corporation.
From the Department of Dermatology, Camp Pendleton Naval Hospital, and the Department of Dermatology, Naval Medical Center, San Diego. Opinions or assertions contained herein are the private views of the authors and are not to be considered as official or as reflecting the views of the Department of the Navy, Depamnent of Defense, or tile United States Government. Reprint requests: CDR Neil F. Gibbs, MC, USN, Department of Dermatology, Naval Hospital, Camp Pendleton, CA 92055 16/4/69647
Fig. 1. Papulonodular lesions on patient's chin.
Removal with scalpel, curette, or carbon dioxide laser produced cosmetically acceptable results. There has been no evidence of recurrence of treated lesions, but new lesions continue to appear.
DISCUSSION The classification of the histiocytoses has continued to expand. The original division into " X " and " n o n - X " has evolved into an extensive list based on the results of clinical and laboratory 323
324
Gibbs and O'Grady
Journal of the AmericanAcademyof Dermatology August 1996
Fig. 2. Biopsy specimen showing dome-shaped papule with lateral collarette and wedgeshaped infiltrate penetrating to deep reticular dermis (x20).
Fig. 3. Biopsy specimen showing infiltrate of numerous Touton-type giant cells. studies. A classification scheme has been recently proposed. 1 Our patient presents features that do not easily fit into this classification. The lack of S-100 staining
and the absence of Birbeck granules make Langerhans or indeterminate cell origin unlikely. The presence of skin involvement and the lack of lymph node involvement make interdigitating cell and follicular dendritic cell origin unlikely. We believe that our patient most likely has a condition involving an infiltrate of macrophage cell lineage. 1 A diagnosis of progressive nodular histiocytoma was considered. Some features, such as clinical lesions that range from small yellow-brown papules to larger dermal nodules with overlying telangiectasias, are not consistent with this diagnosis .2 In addition, in progressive nodular histiocytoma there is a tendency for lesions to coalesce on the face, producing a leonine appearance, 3, 4 and spontaneous resolution can occur.2, 5 These features were absent in our patient. Another consideration was generalized eruptive histiocytoma, which is characterized by widespread, symmetric, papular lesions appearing in crops. As in our patient there is no tendency for grouping of lesions; however, in generalized eruptive histiocytoma lesions usually clear spontaneously leaving brown macules or normal-appearing skin. Also, in generalized eruptive histiocytoma lipid stains are negative and giant cells are not seen. 6-8 Papular xanthoma involves the skin and occasionally the mucosa. It generally resolves spontaneously after a few years.4, 9 A progressive course lasting 5 years has been described in one patient. 1° Histologically, the lesions
Journal of the American Academy of Dermatology Volume 35, Number 2, Part 2
consist mainly o f foam cells, with essentially no nonlipidized histiocytes. M a n y intracytoplasmic lipid droplets are seen on electron microscopy. 1° In xanthoma disseminamm the papules tend quickly to merge into yellow-red plaques, which characteristically involve the lips, pharynx, and larynx. Diabetes insipidus is present in 50% o f patients.4, 9 Histologically, these lesions are characterized by foamy histiocytes. The collagen trapping and collarette formation in our patient's biopsy specimens suggested a dermatofibroma or dermal dendrocytoma. However, the lack o f factor XIII immtmostaining ruled against these diagnosesJ Finally, juvenile xanthogranulomas can occur in adults. Our patient has m a n y findings consistent with xanthogranuloma. 11-13 However, mature juvenile xanthogranulomas and xanthogranulomas contain mainly foamy cells, whereas in the mature lesions in our patient nonfoamy histiocytes and fibroblasts were most conspicuous. In addition, there have been no reports o f adult xanthogranulomas appearing continually for an extended period with no evidence of fading, as is the case in our patient.
Gibbs and O'Grady 325 REFERENCES 1. Wood GS, Haber RS. Novel histiocytoses considered in the context of histiocyte subset differentiation.Arch Dermatol 1993;129:210-4. 2. Burgdorf WHC, Kusch SL, Nix TE, et al. Progressive nodular histiocytoma. Arch Dermatol 1981;117:644-9. 3. Torres L, Sanchez JL, Pdvera A, et al. Progressive nodular hisfiocytosis. J Am Acad Dermatol 1993;29:278-80. 4. Gianotti F, Caputo R. Histiocytic syndromes: a review. J Am Acad Dermatol 1985;13:383-404. 5. Taunton OD, Yeshtmm D, Jarratt M. Progressive nodular histiocytoma. Arch Dermatol 1978;114:1505-8. 6. Winkelmann RK, Muller SA. Generalized eruptive histiocytoma. Arch Dermatol 1963;88:154-64. 7. Muller SA, Wolff K, Winkelmann RK. Generalized empfive histiocytoma. Arch Dermatol 1967;96:11-8. 8. Umbert IJ, Winkelmann RK. Eruptive histiocytoma. J Am Acad Dermatol 1989;20:958-64. 9. Winkelmann RK. Cutaneous syndromes of non-X histiocytosis. Arch Dermatol 1981;117:667-72. 10. Sanchez R, Raimer S, Peltier F. Papular xanthoma: a clinical, histologic, and ultrastmctural study. Arch Dermatol 1985;121:626-31. 11. Rodriguez J, Ackerman AB. Xanthogranuloma in adults. Arch Dermatol 1976;112:43-4. 12. Cohen BA, Hood A. Xanthogranuloma: report on clinical and histologic findings in 64 patients. Pediatr Dermatol 1989;6:262-6. 13. Malbos S, Guilhou J-J, Meynadier J, et al. Le xanthogranulome de l'adulte: Etude anatomo-clinique d'un cas. Dermatologica 1979;158:334-42.
and San Diego, California We describe progressive eruptive lesions that have been appearing on a 50-year-old Filipino man for the past 9 years. They are characterized by smooth, brown, firm nodules mainly on the head and torso. Histologic examination shows dermal collections of lipid-filled histiocytes and Touton-type giant cells. A comparison with other types of previously described non-Langerhans cell histiocytoses is made. (J Am Acad Dermatol 1996;35:323-5.) The histiocytoses are a diverse group of conditions. W e describe a patient who does not easily fit into the present classification.
CASE REPORT A 50-year-old Filipino man had the progressive appearance of 2 to 5 mm firm, smooth, brown papules and nodules on his face, arms, and torso for 9 years (Fig. 1). The lesions grew to their final size in 6 to 12 months and did not change thereafter. The patient has non-insulin-dependent diabetes mellims treated with glyburide. A blood lipid panel showed normal cholesterol and high-density lipoprotein values and a slightly elevated trigiyceride level (234 mg/dl). X-ray examination of the skull showed no enlargement of the sella mrcica. Biopsy specimens showed well-circumscribed but not encapsulated collections of histiocytes with multiple Touton-type giant cells within the dermis (Figs. 2 and 3). The infiltrate splayed collagen bundles at the edges, producing an appearance of "collagen trapping." The histiocytes in the dermal infiltrate were not "foamy," but Oil red O staining of fresh frozen sections showed fipid deposition within them. An immunoperoxidase study showed that the histiocytes were negative for S-100 protein and factor XIII. They were positive for vimentin. Electron microscopy showed no Birbeck granules or Caputo bodies in the cytoplasm of the cells, and fipid droplets were not prominent. ORTHO
This article is made possible through an educational grant from the Dermatological Division, Ortho Pharmaceutical Corporation.
From the Department of Dermatology, Camp Pendleton Naval Hospital, and the Department of Dermatology, Naval Medical Center, San Diego. Opinions or assertions contained herein are the private views of the authors and are not to be considered as official or as reflecting the views of the Department of the Navy, Depamnent of Defense, or tile United States Government. Reprint requests: CDR Neil F. Gibbs, MC, USN, Department of Dermatology, Naval Hospital, Camp Pendleton, CA 92055 16/4/69647
Fig. 1. Papulonodular lesions on patient's chin.
Removal with scalpel, curette, or carbon dioxide laser produced cosmetically acceptable results. There has been no evidence of recurrence of treated lesions, but new lesions continue to appear.
DISCUSSION The classification of the histiocytoses has continued to expand. The original division into " X " and " n o n - X " has evolved into an extensive list based on the results of clinical and laboratory 323
324
Gibbs and O'Grady
Journal of the AmericanAcademyof Dermatology August 1996
Fig. 2. Biopsy specimen showing dome-shaped papule with lateral collarette and wedgeshaped infiltrate penetrating to deep reticular dermis (x20).
Fig. 3. Biopsy specimen showing infiltrate of numerous Touton-type giant cells. studies. A classification scheme has been recently proposed. 1 Our patient presents features that do not easily fit into this classification. The lack of S-100 staining
and the absence of Birbeck granules make Langerhans or indeterminate cell origin unlikely. The presence of skin involvement and the lack of lymph node involvement make interdigitating cell and follicular dendritic cell origin unlikely. We believe that our patient most likely has a condition involving an infiltrate of macrophage cell lineage. 1 A diagnosis of progressive nodular histiocytoma was considered. Some features, such as clinical lesions that range from small yellow-brown papules to larger dermal nodules with overlying telangiectasias, are not consistent with this diagnosis .2 In addition, in progressive nodular histiocytoma there is a tendency for lesions to coalesce on the face, producing a leonine appearance, 3, 4 and spontaneous resolution can occur.2, 5 These features were absent in our patient. Another consideration was generalized eruptive histiocytoma, which is characterized by widespread, symmetric, papular lesions appearing in crops. As in our patient there is no tendency for grouping of lesions; however, in generalized eruptive histiocytoma lesions usually clear spontaneously leaving brown macules or normal-appearing skin. Also, in generalized eruptive histiocytoma lipid stains are negative and giant cells are not seen. 6-8 Papular xanthoma involves the skin and occasionally the mucosa. It generally resolves spontaneously after a few years.4, 9 A progressive course lasting 5 years has been described in one patient. 1° Histologically, the lesions
Journal of the American Academy of Dermatology Volume 35, Number 2, Part 2
consist mainly o f foam cells, with essentially no nonlipidized histiocytes. M a n y intracytoplasmic lipid droplets are seen on electron microscopy. 1° In xanthoma disseminamm the papules tend quickly to merge into yellow-red plaques, which characteristically involve the lips, pharynx, and larynx. Diabetes insipidus is present in 50% o f patients.4, 9 Histologically, these lesions are characterized by foamy histiocytes. The collagen trapping and collarette formation in our patient's biopsy specimens suggested a dermatofibroma or dermal dendrocytoma. However, the lack o f factor XIII immtmostaining ruled against these diagnosesJ Finally, juvenile xanthogranulomas can occur in adults. Our patient has m a n y findings consistent with xanthogranuloma. 11-13 However, mature juvenile xanthogranulomas and xanthogranulomas contain mainly foamy cells, whereas in the mature lesions in our patient nonfoamy histiocytes and fibroblasts were most conspicuous. In addition, there have been no reports o f adult xanthogranulomas appearing continually for an extended period with no evidence of fading, as is the case in our patient.
Gibbs and O'Grady 325 REFERENCES 1. Wood GS, Haber RS. Novel histiocytoses considered in the context of histiocyte subset differentiation.Arch Dermatol 1993;129:210-4. 2. Burgdorf WHC, Kusch SL, Nix TE, et al. Progressive nodular histiocytoma. Arch Dermatol 1981;117:644-9. 3. Torres L, Sanchez JL, Pdvera A, et al. Progressive nodular hisfiocytosis. J Am Acad Dermatol 1993;29:278-80. 4. Gianotti F, Caputo R. Histiocytic syndromes: a review. J Am Acad Dermatol 1985;13:383-404. 5. Taunton OD, Yeshtmm D, Jarratt M. Progressive nodular histiocytoma. Arch Dermatol 1978;114:1505-8. 6. Winkelmann RK, Muller SA. Generalized eruptive histiocytoma. Arch Dermatol 1963;88:154-64. 7. Muller SA, Wolff K, Winkelmann RK. Generalized empfive histiocytoma. Arch Dermatol 1967;96:11-8. 8. Umbert IJ, Winkelmann RK. Eruptive histiocytoma. J Am Acad Dermatol 1989;20:958-64. 9. Winkelmann RK. Cutaneous syndromes of non-X histiocytosis. Arch Dermatol 1981;117:667-72. 10. Sanchez R, Raimer S, Peltier F. Papular xanthoma: a clinical, histologic, and ultrastmctural study. Arch Dermatol 1985;121:626-31. 11. Rodriguez J, Ackerman AB. Xanthogranuloma in adults. Arch Dermatol 1976;112:43-4. 12. Cohen BA, Hood A. Xanthogranuloma: report on clinical and histologic findings in 64 patients. Pediatr Dermatol 1989;6:262-6. 13. Malbos S, Guilhou J-J, Meynadier J, et al. Le xanthogranulome de l'adulte: Etude anatomo-clinique d'un cas. Dermatologica 1979;158:334-42.