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Adult Disseminated Primary Papular Xanthoma Treated With Doxycycline

Adult Disseminated Primary Papular Xanthoma Treated With Doxycycline Adult disseminated primary papular xanthoma (pPX) is a rare histiocytic proliferation of dermal dendrocyte origin in the spectrum of non–Langerhans cell histiocytosis. Adult disseminated pPX shows a chronic course, and no effective treatment is known. Herein we describe a woman with cutaneous lesions associated with pPX that showed complete regression after treatment with oral doxycycline. Report of a Case A 29-year-old atopic woman consulted with us because of the progressive appearance of hundreds of asymptomatic papules 3 months earlier. Lesions started as a sudden crop of small papules that increased slowly in size and number. Crops of new lesions were admixed with older ones. Examination revealed hundreds of red-yellowish, nonconfluent, asymptomatic papules ranging in diameter from 0.5 to 1.0 cm (Figure 1A). Older lesions were flat and brownish. Lesions were diffusely distributed and tended to be grouped in skin folds but did not merge into plaques. In other locations such as the scalp, the abdominal wall, and the back, no clustering was observed. Solitary papules were seen elsewhere. Isolated mucosal lesions were also detected in tarsal and bulbar conjunctiva, gums, ears, and the upper airway. No adenopathies, visceromegalies, or signs of diabetes insipidus were present. Results of ophthalmic and neurologic explorations and extensive laboratory and imaging tests including plasma lipid and lipoprotein studies were normal. Figure 1. View LargeDownload Our patient had progressive appearance of hundreds of asymptomatic papules. A, Yellowish dome-shaped lesions on the back. B, Anetodermic scars after complete resolution of the lesions. Microscopically, lesions consisted of dermal sheets of xanthomatized histiocytes (Figure 2A) with numerous multinucleated Touton giant cells (Figure 2B). A scant inflammatory infiltrate was scattered throughout the lesions. Histiocytic cells expressed CD68 and factor XIIIa, whereas CD1a and S-100 results were negative. Ultrastructurally, no Birbeck granules or laminated or “comma” bodies were present. On the basis of clinicopathologic findings, the diagnosis of adult disseminated pPX was established. Figure 2. View LargeDownload Histopathologic findings of our patient's lesions. A, Dermal histiocytic aggregates with scarce inflammatory infiltrate (hematoxylin-eosin, original magnification ×40). B, Foamy histiocytes and Touton cells (hematoxylin-eosin, original magnification ×400). C, Histiocytic cells are factor XIIIa positive (immunoperoxidase stain, original magnification ×100). Treatment with oral isotretinoin and cyclosporin had no effect. Finally, the patient started treatment with oral doxycycline (200 mg/d), and cutaneous and mucosal lesions resolved in a few weeks leaving anetodermic scars (Figure 1B). Twelve months later, the patient stopped therapy and did not experience recurrence in the following 6 months. Comment The adult disseminated form of pPX is a very rare disease with only 8 cases reported, to our knowledge.1-8 All cases reported with follow-up showed a persistent and progressive course, and no specific therapy is known (Table). To our knowledge, this is the first report of disseminated pPX in an adult that showed resolution of the mucocutaneous lesions soon after administration of oral doxycycline. Table. View LargeDownload Reported Cases of Adult Disseminated Primary Papular Xanthoma The underlying mechanism of this event remains unclear. Doxycycline is a bacteriostatic antimicrobial agent, and the success in this case may suggest a microbial origin of pPX. However, this drug shows anti-inflammatory and anticollagenolytic properties also.9 In addition, doxycycline shows an antiproliferative and proapoptotic effect in cultured mast cells, both malignant and nonmalignant.10 Interestingly, there is a close up-regulative and physical relationship between cutaneous dermal dendrocytes and mast cells.11 However, the cause of pPX is unknown, and all the etiopathogenic pathways, including up-regulation of mast cells, may be involved in the possible effect of doxycycline in this patient. Our case suggests that doxycycline may be used as a successful therapy in patients with pPX (and maybe in other non–Langerhans cell histiocytosis too), but this should be confirmed in other cases of this disease. Correspondence: Dr Bastida, Servicio de Dermatología, Hospital Universitario “Doctor Negrín,” Barranco de la Ballena s n Las Palmas de Gran Canaria 35010, Spain (jbastidai@meditex.es). Financial Disclosure: None reported. Previous Presentation: This case report was presented in part as a poster at the Spanish Ear, Nose, and Throat Society meeting; September 30, 2006; Granada, Spain. Back to top Article Information Acknowledgment: We acknowledge Rokea A. el-Azhary, MD, for her suggestion to use tetracyclines to treat this patient. References 1. Hu CHWinkelmann RK Unusual normolipidemic cutaneous xanthomatosis: a comparison of two cases illustrating the differential diagnosis. Acta Derm Venereol 1977;57421- 429PubMedGoogle Scholar 2. Beurey JLamaze MWeber MDelrous JLKremer BChaulieu Y Xanthoma disseminatum (syndrome de Montgomery). Ann Dermatol Venereol 1979;106353- 359PubMedGoogle Scholar 3. Thomas RHMiller NEPayne CMEBlack MM Papular xanthoma associated with primary dysbetalipoproteinaemia. J R Soc Med 1982;75906- 908PubMedGoogle Scholar 4. Sanchez RLRaimer SSPeltier FSwedo J Papular xanthoma: a clinical, histologic and ultrastructural study. Arch Dermatol 1985;121626- 631PubMedGoogle ScholarCrossref 5. Bundino SZina AMAloi F Papular xanthoma: clinical, histological and ultrastructural study. Dermatologica 1988;177382- 385PubMedGoogle ScholarCrossref 6. Pfennigsdorf SLieb WE Papulöse Xanthome der lider. Klin Monatsbl Augenheilkd 1997;210113- 115PubMedGoogle ScholarCrossref 7. Caputo RPassoni ECavicchini S Papular xanthoma associated with angiokeratoma of Fordyce: considerations on the nosography of this rare non-Langerhans cell histiocytomatosis. Dermatology 2003;206165- 168PubMedGoogle ScholarCrossref 8. Breier FZelger BReiter HGschnait FZelger BWH Papular xanthoma: a clinicopathological study of 10 cases. J Cutan Pathol 2002;29200- 206PubMedGoogle ScholarCrossref 9. Weinberg JM The anti-inflammatory effects of tetracyclines. Cutis 2005;75 ((4) (suppl)) 6- 11PubMedGoogle Scholar 10. Sandler CNurmi KLindstedt KA et al. Chemically modified tetracyclines induce apoptosis in cultured mast cells. Int Immunopharmacol 2005;51611- 1621PubMedGoogle ScholarCrossref 11. Chu AC Histiocytoses. Burns TBreathnach SCox NGriffiths Ceds. Rook's Textbook of Dermatology. 3 Malden, Mass Blackwell Publishing2004;52.1- 52.23Google Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Adult Disseminated Primary Papular Xanthoma Treated With Doxycycline

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Publisher
American Medical Association
Copyright
Copyright © 2007 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.143.5.667
pmid
17519219
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Abstract

Adult disseminated primary papular xanthoma (pPX) is a rare histiocytic proliferation of dermal dendrocyte origin in the spectrum of non–Langerhans cell histiocytosis. Adult disseminated pPX shows a chronic course, and no effective treatment is known. Herein we describe a woman with cutaneous lesions associated with pPX that showed complete regression after treatment with oral doxycycline. Report of a Case A 29-year-old atopic woman consulted with us because of the progressive appearance of hundreds of asymptomatic papules 3 months earlier. Lesions started as a sudden crop of small papules that increased slowly in size and number. Crops of new lesions were admixed with older ones. Examination revealed hundreds of red-yellowish, nonconfluent, asymptomatic papules ranging in diameter from 0.5 to 1.0 cm (Figure 1A). Older lesions were flat and brownish. Lesions were diffusely distributed and tended to be grouped in skin folds but did not merge into plaques. In other locations such as the scalp, the abdominal wall, and the back, no clustering was observed. Solitary papules were seen elsewhere. Isolated mucosal lesions were also detected in tarsal and bulbar conjunctiva, gums, ears, and the upper airway. No adenopathies, visceromegalies, or signs of diabetes insipidus were present. Results of ophthalmic and neurologic explorations and extensive laboratory and imaging tests including plasma lipid and lipoprotein studies were normal. Figure 1. View LargeDownload Our patient had progressive appearance of hundreds of asymptomatic papules. A, Yellowish dome-shaped lesions on the back. B, Anetodermic scars after complete resolution of the lesions. Microscopically, lesions consisted of dermal sheets of xanthomatized histiocytes (Figure 2A) with numerous multinucleated Touton giant cells (Figure 2B). A scant inflammatory infiltrate was scattered throughout the lesions. Histiocytic cells expressed CD68 and factor XIIIa, whereas CD1a and S-100 results were negative. Ultrastructurally, no Birbeck granules or laminated or “comma” bodies were present. On the basis of clinicopathologic findings, the diagnosis of adult disseminated pPX was established. Figure 2. View LargeDownload Histopathologic findings of our patient's lesions. A, Dermal histiocytic aggregates with scarce inflammatory infiltrate (hematoxylin-eosin, original magnification ×40). B, Foamy histiocytes and Touton cells (hematoxylin-eosin, original magnification ×400). C, Histiocytic cells are factor XIIIa positive (immunoperoxidase stain, original magnification ×100). Treatment with oral isotretinoin and cyclosporin had no effect. Finally, the patient started treatment with oral doxycycline (200 mg/d), and cutaneous and mucosal lesions resolved in a few weeks leaving anetodermic scars (Figure 1B). Twelve months later, the patient stopped therapy and did not experience recurrence in the following 6 months. Comment The adult disseminated form of pPX is a very rare disease with only 8 cases reported, to our knowledge.1-8 All cases reported with follow-up showed a persistent and progressive course, and no specific therapy is known (Table). To our knowledge, this is the first report of disseminated pPX in an adult that showed resolution of the mucocutaneous lesions soon after administration of oral doxycycline. Table. View LargeDownload Reported Cases of Adult Disseminated Primary Papular Xanthoma The underlying mechanism of this event remains unclear. Doxycycline is a bacteriostatic antimicrobial agent, and the success in this case may suggest a microbial origin of pPX. However, this drug shows anti-inflammatory and anticollagenolytic properties also.9 In addition, doxycycline shows an antiproliferative and proapoptotic effect in cultured mast cells, both malignant and nonmalignant.10 Interestingly, there is a close up-regulative and physical relationship between cutaneous dermal dendrocytes and mast cells.11 However, the cause of pPX is unknown, and all the etiopathogenic pathways, including up-regulation of mast cells, may be involved in the possible effect of doxycycline in this patient. Our case suggests that doxycycline may be used as a successful therapy in patients with pPX (and maybe in other non–Langerhans cell histiocytosis too), but this should be confirmed in other cases of this disease. Correspondence: Dr Bastida, Servicio de Dermatología, Hospital Universitario “Doctor Negrín,” Barranco de la Ballena s n Las Palmas de Gran Canaria 35010, Spain (jbastidai@meditex.es). Financial Disclosure: None reported. Previous Presentation: This case report was presented in part as a poster at the Spanish Ear, Nose, and Throat Society meeting; September 30, 2006; Granada, Spain. Back to top Article Information Acknowledgment: We acknowledge Rokea A. el-Azhary, MD, for her suggestion to use tetracyclines to treat this patient. References 1. Hu CHWinkelmann RK Unusual normolipidemic cutaneous xanthomatosis: a comparison of two cases illustrating the differential diagnosis. Acta Derm Venereol 1977;57421- 429PubMedGoogle Scholar 2. Beurey JLamaze MWeber MDelrous JLKremer BChaulieu Y Xanthoma disseminatum (syndrome de Montgomery). Ann Dermatol Venereol 1979;106353- 359PubMedGoogle Scholar 3. Thomas RHMiller NEPayne CMEBlack MM Papular xanthoma associated with primary dysbetalipoproteinaemia. J R Soc Med 1982;75906- 908PubMedGoogle Scholar 4. Sanchez RLRaimer SSPeltier FSwedo J Papular xanthoma: a clinical, histologic and ultrastructural study. Arch Dermatol 1985;121626- 631PubMedGoogle ScholarCrossref 5. Bundino SZina AMAloi F Papular xanthoma: clinical, histological and ultrastructural study. Dermatologica 1988;177382- 385PubMedGoogle ScholarCrossref 6. Pfennigsdorf SLieb WE Papulöse Xanthome der lider. Klin Monatsbl Augenheilkd 1997;210113- 115PubMedGoogle ScholarCrossref 7. Caputo RPassoni ECavicchini S Papular xanthoma associated with angiokeratoma of Fordyce: considerations on the nosography of this rare non-Langerhans cell histiocytomatosis. Dermatology 2003;206165- 168PubMedGoogle ScholarCrossref 8. Breier FZelger BReiter HGschnait FZelger BWH Papular xanthoma: a clinicopathological study of 10 cases. J Cutan Pathol 2002;29200- 206PubMedGoogle ScholarCrossref 9. Weinberg JM The anti-inflammatory effects of tetracyclines. Cutis 2005;75 ((4) (suppl)) 6- 11PubMedGoogle Scholar 10. Sandler CNurmi KLindstedt KA et al. Chemically modified tetracyclines induce apoptosis in cultured mast cells. Int Immunopharmacol 2005;51611- 1621PubMedGoogle ScholarCrossref 11. Chu AC Histiocytoses. Burns TBreathnach SCox NGriffiths Ceds. Rook's Textbook of Dermatology. 3 Malden, Mass Blackwell Publishing2004;52.1- 52.23Google Scholar

Journal

Archives of Dermatology – American Medical Association

Published: May 1, 2007

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