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Current Dermatology Reports
Published in: Current Dermatology Reports 4/2013

01-12-2013 | Pediatrics (R Sidbury, Section Editor)

Pityriasis Lichenoides and Cutaneous T Cell Lymphoma: An Update on the Diagnosis and Management of the Most Common Benign and Malignant Cutaneous Lymphoproliferative Diseases in Children

Authors: Markus D. Boos, Sara S. Samimi, Alain H. Rook, Albert C. Yan, Ellen J. Kim

Published in: Current Dermatology Reports | Issue 4/2013

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Abstract

Although more common with advanced age, the entire spectrum of benign and malignant cutaneous lymphoproliferative disorders can present in the pediatric population. Nevertheless, these conditions are often mistaken for other more common, benign dermatoses, resulting in delayed diagnosis. Standardized management of these conditions is also lacking. The purpose of this review is to provide an update on the most common benign and malignant cutaneous lymphoproliferative disorders in children: pityriasis lichenoides and mycosis fungoides. The presentation, evaluation and management of each of these conditions are discussed.
Literature
1.
2.
Baum CL, Link BK, Neppalli VT, Swick BL, Liu V. Reappraisal of the provisional entity primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma: a series of 10 adult and pediatric patients and review of the literature. J Am Acad Dermatol. 2011;65:739–48.PubMedCrossRef
3.
• Fernandes NF, Rozdeba PJ, Schwartz RA, Kihiczak G, Lambert WC. Pityriasis lichenoides et varioliformis acuta: a disease spectrum. Int J Dermatol. 2010;49:257–61. This review delves into the clinical spectrum of PLEVA with Mucha Haberman disease as a severe variant, its proposed etiologies, histology and potential treatment regimens.PubMedCrossRef
4.
Ersoy-Evans S, Greco MF, Mancini AJ, Subaşi N, Paller AS. Pityriasis lichenoides in childhood: a retrospective review of 124 patients. J Am Acad Dermatol. 2007;56:205–10.PubMedCrossRef
5.
• Hapa A, Ersoy-Evans S, Karaduman A. Childhood pityriasis lichenoides and oral erythromycin. Pediatr Dermatol. 2012;29:719–24. This retrospective analysis of 24 children demonstrated the efficacy of oral erythromycin in patients with both PLEVA and PLC, with a maximum response noted after administration for 3 months.PubMedCrossRef
6.
7.
Kim JE et al. Pityriasis lichenoides et varioliformis acuta and pityriasis lichenoides chronica: comparison of lesional T-cell subsets and investigation of viral associations. J Cutan Pathol. 2011;38:649–56.PubMedCrossRef
8.
Bowers S, Warshaw EM. Pityriasis lichenoides and its subtypes. J Am Acad Dermatol. 2006;55:557–72. quiz 573–576.PubMedCrossRef
9.
Machan M, Loren R, Fraga G, Liu D. Pityriasis lichenoides et varioliformis acuta associated with subcutaneous immunoglobulin administration. J Am Acad Dermatol. 2012;67:e151–2.PubMedCrossRef
10.
Gunatheesan S, Ferguson J, Moosa Y. Pityriasis lichenoides et varioliformis acuta: a rare association with the measles, mumps and rubella vaccine. Australas J Dermatol. 2012;53:e76–8.PubMedCrossRef
11.
Gil-Bistes D, Kluger N, Bessis D, Guillot B, Raison-Peyron N. Pityriasis lichenoides chronic after measles-mumps-rubella vaccination. J Dermatol. 2012;39:492–3.PubMedCrossRef
12.
13.
Wahie S, Hiscutt E, Natarajan S, Taylor A. Pityriasis lichenoides: the differences between children and adults. Br J Dermatol. 2007;157:941–5.PubMedCrossRef
14.
Skinner RB, Levy AL. Rapid resolution of pityriasis lichenoides et varioliformis acuta with azithromycin. J Am Acad Dermatol. 2008;58:524–5.PubMedCrossRef
15.
Ersoy-Evans S, Altaykan A, Sahin S, Kölemen F. Phototherapy in childhood. Pediatr Dermatol. 2008;25:599–605.PubMedCrossRef
16.
• Ersoy-Evans S, Hapa AA, Boztepe G, Sahin S, Kölemen F. Narrowband ultraviolet-B phototherapy in pityriasis lichenoides chronica. J Dermatol Treat. 2009;20:109–13. This retrospective analysis of 25 patients demonstrated that nbUVB therapy is an effective mode of therapy with a complete response in 48 % and a partial response of 44 % of patients with PLC.CrossRef
17.
Perrin BS, Yan AC, Treat JR. Febrile ulceronecrotic Mucha-Habermann disease in a 34-month-old boy: a case report and review of the literature. Pediatr Dermatol. 2012;29:53–8.PubMedCrossRef
18.
Tsianakas A, Hoeger PH. Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumour necrosis factor-alpha. Br J Dermatol. 2005;152:794–9.PubMedCrossRef
19.
Kaufman WS et al. Febrile ulceronecrotic Mucha-Habermann disease (pityriasis lichenoides et varioliformis acuta fulminans) presenting as Stevens-Johnson syndrome. Pediatr Dermatol. 2012;29:135–40.PubMedCrossRef
20.
21.
Smith JJL, Oliver GF. Febrile ulceronecrotic Mucha-Habermann disease associated with herpes simplex virus type 2. J Am Acad Dermatol. 2009;60:149–52.PubMedCrossRef
22.
Meziane L et al. Febrile ulceronecrotic Mucha-Habermann disease: treatment with infliximab and intravenous immunoglobulins and review of the literature. Dermatol Basel Switz. 2012;225:344–8.CrossRef
23.
Zhang L, Liang Y, Liu Y, Ma L. Febrile ulceronecrotic Mucha-Habermann’s disease with pulmonary involvement. Pediatr Dermatol. 2010;27:290–3.PubMedCrossRef
24.
Marenco F, Fava P, Fierro MT, Quaglino P, Bernengo MG. High-dose immunoglobulines and extracorporeal photochemotherapy in the treatment of febrile ulceronecrotic Mucha-Habermann disease. Dermatol Ther. 2010;23:419–22.PubMedCrossRef
25.
26.
Criscione VD, Weinstock MA. Incidence of cutaneous T-cell lymphoma in the United States, 1973-2002. Arch Dermatol. 2007;143:854–9.PubMedCrossRef
27.
Tan E, Tay YK, Giam YC. Profile and outcome of childhood mycosis fungoides in Singapore. Pediatr Dermatol. 2000;17:352–6.PubMedCrossRef
28.
Wain EM, Orchard GE, Whittaker SJ, Spittle MF, Russell-Jones R. Outcome in 34 patients with juvenile-onset mycosis fungoides: a clinical, immunophenotypic, and molecular study. Cancer. 2003;98:2282–90.PubMedCrossRef
29.
Quaglino P, Zaccagna A, Verrone A, Dardano F, Bernengo MG. Mycosis fungoides in patients under 20 years of age: report of 7 cases, review of the literature and study of the clinical course. Dermatol Basel Switz. 1999;199:8–14.CrossRef
30.
Nanda A et al. Mycosis fungoides in Arab children and adolescents: a report of 36 patients from Kuwait. Pediatr Dermatol. 2010;27:607–13.PubMedCrossRef
31.
Koch SE, Zackheim HS, Williams ML, Fletcher V, LeBoit PE. Mycosis fungoides beginning in childhood and adolescence. J Am Acad Dermatol. 1987;17:563–70.PubMedCrossRef
32.
Meister L, Duarte AM, Davis J, Perez JL, Schachner LA. Sézary syndrome in an 11-year-old girl. J Am Acad Dermatol. 1993;28:93–5.PubMedCrossRef
33.
Fink-Puches R et al. The spectrum of cutaneous lymphomas in patients less than 20 years of age. Pediatr Dermatol. 2004;21:525–33.PubMedCrossRef
34.
35.
Hanna S, Walsh N, D’Intino Y, Langley RGB. Mycosis fungoides presenting as pigmented purpuric dermatitis. Pediatr Dermatol. 2006;23:350–4.PubMedCrossRef
36.
Baykal C, Büyükbabani N, Kaymaz R. Familial mycosis fungoides. Br J Dermatol. 2002;146:1108–10.PubMedCrossRef
37.
Hodak E et al. Familial mycosis fungoides: report of 6 kindreds and a study of the HLA system. J Am Acad Dermatol. 2005;52:393–402.PubMedCrossRef
38.
Schneider BF, Christian M, Hess CE, Williams ME. Familial occurrence of cutaneous T cell lymphoma: a case report of monozygotic twin sisters. Leukemia. 1995;9:1979–81.PubMed
39.
Vassallo C et al. Mycosis fungoides in childhood: description and study of two siblings. Acta Derm Venereol. 2007;87:529–32.PubMedCrossRef
40.
Neuhaus IM, Ramos-Caro FA, Hassanein AM. Hypopigmented mycosis fungoides in childhood and adolescence. Pediatr Dermatol. 2000;17:403–6.PubMedCrossRef
41.
• Pope E et al. Mycosis fungoides in the pediatric population: report from an international Childhood Registry of Cutaneous Lymphoma. J Cutan Med Surg. 2010;14:1–6. This retrospective analysis of 22 patients from the Childhood Registry of Cutaneous Lymphoma demonstrated that patch stage and hypopigmented MF were the most commonly represented variants in cases of pediatric MF.PubMed
42.
Ben-Amitai D, David M, Feinmesser M, Hodak E. Juvenile mycosis fungoides diagnosed before 18 years of age. Acta Derm Venereol. 2003;83:451–6.PubMedCrossRef
43.
Ardigó M, Borroni G, Muscardin L, Kerl H, Cerroni L. Hypopigmented mycosis fungoides in Caucasian patients: a clinicopathologic study of 7 cases. J Am Acad Dermatol. 2003;49:264–70.PubMedCrossRef
44.
Ally MS et al. Solitary mycosis fungoides: a distinct clinicopathologic entity with a good prognosis. J Am Acad Dermatol. 2012;67:736–44.PubMedCrossRef
45.
Ranawaka RR, Abeygunasekara PH, de Silva MVC. Hypopigmented mycosis fungoides in type V skin: a report of 5 cases. Case Rep Dermatol Med. 2011;2011:1–6.CrossRef
46.
Ko JW, Seong JY, Suh KS, Kim ST. Pityriasis lichenoides-like mycosis fungoides in children. Br J Dermatol. 2000;142:347–52.PubMedCrossRef
47.
Agar NS et al. Survival outcomes and prognostic factors in mycosis fungoides/sezary syndrome: validation of the revised international society for cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer Staging Proposal. J Clin Oncol. 2010;28:4730–9.PubMedCrossRef
48.
LeBoit PE. Alopecia mucinosa, inflammatory disease or mycosis fungoides: must we choose? And are there other choices? Am J Dermatopathol. 2004;26:167–70.PubMedCrossRef
49.
Vollmer RT. Mycosis fungoides and follicular mucinosis. Arch Dermatol. 2002;138:1613–4. author reply 1614–1615.PubMedCrossRef
50.
Brown HA, Gibson LE, Pujol RM, Lust JA, Pittelkow MR. Primary follicular mucinosis: long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement. J Am Acad Dermatol. 2002;47:856–62.PubMedCrossRef
51.
Cerroni L, Fink-Puches R, Bäck B, Kerl H. Follicular mucinosis: a critical reappraisal of clinicopathologic features and association with mycosis fungoides and Sézary syndrome. Arch Dermatol. 2002;138:182–9.PubMedCrossRef
52.
Zvulunov A, Shkalim V, Ben-Amitai D, Feinmesser M. Clinical and histopathologic spectrum of alopecia mucinosa/follicular mucinosis and its natural history in children. J Am Acad Dermatol. 2012;67:1174–81.PubMedCrossRef
53.
•• Alikhan A, Griffin J, Nguyen N, Davis DMR, Gibson LE. Pediatric follicular mucinosis: presentation, histopathology, molecular genetics, treatment, and outcomes over an 11-year period at the Mayo Clinic. Pediatr Dermatol. 2013;30:192–8. These two studies demonstrated that pediatric FM is infrequently associated with other hematologic malignancies and concluded that FM could not be equated with early folliculotropic MF in children.PubMedCrossRef
54.
Plaza JA, Morrison C, Magro CM. Assessment of TCR-beta clonality in a diverse group of cutaneous T-Cell infiltrates. J Cutan Pathol. 2008;35:358–65.PubMedCrossRef
55.
French LE et al. Identification of clonal T cells in the blood of patients with systemic sclerosis: positive correlation with response to photopheresis. Arch Dermatol. 2001;137:1309–13.PubMedCrossRef
56.
57.
Zelenetz AD et al. Non-Hodgkin’s lymphomas, version 3.2012. J Natl Compr Cancer Netw Jnccn. 2012;10:1487–98.
58.
Pearce MS et al. Radiation exposure from CT scans in childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study. Lancet. 2012;380:499–505.PubMedCrossRef
59.
•• Miglioretti DL, et al. The use of computed tomography in pediatrics and the associated radiation exposure and estimated cancer risk. JAMA Pediatr. 2013:1–8. doi:10.​1001/​jamapediatrics.​2013.​311. These two studies demonstrated an increased risk of radiation-induced malignancy in the pediatric population following CT scans and argue for the responsible use of CT scans in the medical evaluation of children.
60.
61.
Crowley JJ, Nikko A, Varghese A, Hoppe RT, Kim YH. Mycosis fungoides in young patients: clinical characteristics and outcome. J Am Acad Dermatol. 1998;38:696–701.PubMedCrossRef
62.
Kim YH, Martinez G, Varghese A, Hoppe RT. Topical nitrogen mustard in the management of mycosis fungoides: update of the Stanford experience. Arch Dermatol. 2003;139:165–73.PubMedCrossRef
63.
Kavala M et al. Treatment of childhood mycosis fungoides with narrow-band phototherapy. Int J Dermatol. 2009;48:203–4.PubMedCrossRef
64.
Pugashetti R, Koo J. Phototherapy in pediatric patients: choosing the appropriate treatment option. Semin Cutan Med Surg. 2010;29:115–20.PubMedCrossRef
65.
Berthelot C, Rivera A, Duvic M. Skin directed therapy for mycosis fungoides: a review. J Drugs Dermatol Jdd. 2008;7:655–66.
66.
Zackheim HS, Kashani-Sabet M, Amin S. Topical corticosteroids for mycosis fungoides. Experience in 79 patients. Arch Dermatol. 1998;134:949–54.PubMedCrossRef
67.
Querfeld C et al. Long-term follow-up of patients with early-stage cutaneous T-cell lymphoma who achieved complete remission with psoralen plus UV-A monotherapy. Arch Dermatol. 2005;141:305–11.PubMedCrossRef
68.
Stern RS, Nichols KT, Väkevä LH. Malignant melanoma in patients treated for psoriasis with methoxsalen (psoralen) and ultraviolet A radiation (PUVA). The PUVA follow-up study. N Engl J Med. 1997;336:1041–5.PubMedCrossRef
69.
Stern RS. Risk of squamous cell carcinoma and methoxsalen (Psoralen) and UV-A radiation (PUVA): a meta-analysis. Arch Dermatol. 1998;134:1582–5.PubMedCrossRef
70.
Hearn RMR, Kerr AC, Rahim KF, Ferguson J, Dawe RS. Incidence of skin cancers in 3867 patients treated with narrow-band ultraviolet B phototherapy. Br J Dermatol. 2008;159:931–5.PubMedCrossRef
71.
Kim YH, Jensen RA, Watanabe GL, Varghese A, Hoppe RT. Clinical stage IA (limited patch and plaque) mycosis fungoides. A long-term outcome analysis. Arch Dermatol. 1996;132:1309–13.PubMedCrossRef
72.
Kim YH, Liu HL, Mraz-Gernhard S, Varghese A, Hoppe RT. Long-term outcome of 525 patients with mycosis fungoides and Sezary syndrome: clinical prognostic factors and risk for disease progression. Arch Dermatol. 2003;139:857–66.PubMedCrossRef
73.
Talpur R et al. Long-term outcomes of 1,263 patients with mycosis fungoides and sezary syndrome from 1982 to 2009. Clin Cancer Res. 2012;18:5051–60.PubMedCrossRef
Metadata
Title
Pityriasis Lichenoides and Cutaneous T Cell Lymphoma: An Update on the Diagnosis and Management of the Most Common Benign and Malignant Cutaneous Lymphoproliferative Diseases in Children
Authors
Markus D. Boos
Sara S. Samimi
Alain H. Rook
Albert C. Yan
Ellen J. Kim
Publication date
01-12-2013
Publisher
Springer US
Published in
Current Dermatology Reports / Issue 4/2013
Electronic ISSN: 2162-4933
DOI
https://doi.org/10.1007/s13671-013-0054-x

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