Type B Follicular Lymphomatoid Papulosis
Total Page：16
File Type：pdf, Size：1020Kb
Type B Follicular Lymphomatoid Papulosis Paru R. Chaudhari, BA; Patrick Emanuel, MD; Jacob Levitt, MD We report a 48-year-old woman who presented Case Report with waxing and waning papulovesicles of 4 years’ A 48-year-old obese woman with a history of geni- duration. Histologic examination revealed a fol- tal herpes presented to our clinic with intermittent liculotropic, small cell dominant, T cell lympho- nonpruritic papulovesicles on her torso of 4 years’ cytic infiltrate unaccompanied by follicular mucin duration. The papulovesicles would wax and wane deposition and with scattered intrafollicular and approximately every 3 months, and she associated perifollicular CD301 cells. A diagnosis of type B them with flulike symptoms of fatigue and myalgia. follicular lymphomatoid papulosis (LyP), rather She had seen 2 other dermatologists for her condi- than mycosis fungoides (MF), was made on the tion and both diagnosed folliculitis after multiple basis of her clinical presentation, which included skin biopsies. A retrospective review of these prior spontaneously resolving, waxing and waning pap- biopsies revealed a relatively dense folliculotropic ules that healed with scarring. Type B follicular infiltrate. She took several medications throughout LyP is a rare form of LyP. the last 4 years; however, the onset of her skin con- Cutis. 2010;85:307-311. dition had no consistent temporal association with medication intake. On physical examination, she CUTISwas noted to have red papulovesicles on the trunk nitially described by Macaulay1 in 1968, lympho- and arms, some with excoriation and ulceration and matoid papulosis (LyP) is a disorder characterized some with scale (Figure 1). No lymphadenopathy or I by remitting and recurring papules and nod- hepatosplenomegaly was present. Complete blood ules that contain atypical lymphocytes. Individual cell count, erythrocyte sedimentation rate, blood lesions usually persist for weeks to months and then chemistries, antinuclear antibody titer, rheumatoid spontaneously heal, often with scarring. The World factor, and rapid plasma reagin levels were all within HealthDo Organization and EuropeanNot Organization for reference Copy range or negative. Research and Treatment of Cancer (WHO-EORTC) A 3-mm skin biopsy revealed a folliculotropic classification system labels LyP as a primary cutaneous and patchy perivascular infiltrate in the superfi- CD301 lymphoproliferative disorder (CD301 LPD).2 cial dermis with small perivascular aggregates in Approximately 20% of patients with this condition the mid dermis (Figure 2). Small to medium–sized have or will eventually develop an additional cutane- lymphocytes also were seen infiltrating the overly- ous or extracutaneous lymphoid dyscrasia, most com- ing epidermis adjacent to the follicle. The infil- monly mycosis fungoides (MF), anaplastic large cell trate consisted of a population of polymorphous lymphoma (ALCL), or Hodgkin lymphoma.3,4 A rare lymphocytes including larger atypical lymphocytes. variant of LyP, follicular LyP, was presented by Pierard The dominant small cell lymphoid populace had et al5 in 1980. The infiltrate in this variant surrounds nuclear atypia and contour irregularities including hair follicles. A total of 12 cases of follicular LyP have occasional cerebriform outlines. No admixed neu- been reported in the literature according to a PubMed trophils were noted and rare eosinophils were pres- search of articles indexed for MEDLINE using the ent. Immunohistochemical studies revealed that the term follicular lymphomatoid papulosis.5-11 We present population of larger atypical lymphocytes exhibited an additional case of follicular LyP. characteristic cytoplasmic staining for CD30. This population was found predominantly in the hair follicle (Figure 3). Review of prior biopsies taken From the Department of Dermatology, The Mount Sinai School of from the patient revealed a similar though more Medicine, New York, New York. sparse population. Additional immunohistochemi- The authors report no conflict of interest. 1 Correspondence: Jacob Levitt, MD, Department of Dermatology, cal markers revealed diffuse CD3 and no staining The Mount Sinai Medical Center, 5 E 98th St, 5th Floor, Box 1048, for CD79a or CD20, confirming an exclusively New York, NY 10029 ([email protected]). T cell infiltrate. Alcian blue staining at pH 2.5 VOLUME 85, JUNE 2010 307 Copyright Cutis 2010. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Follicular Lymphomatoid Papulosis failed to demonstrate follicular mucinosis. Stain- and clinical correlation is essential for a correct ing for CD4 and CD8 cells was not performed and diagnosis. Based on the clinical presentation, we lim- was not thought to have any diagnostic utility in ited the differential diagnosis to type B follicular LyP this case. Additional immunohistochemical stain- and folliculotropic MF. Further workup for systemic ing for herpes simplex virus (HSV) types 1 and 2 malignancy, including flow cytometry and positron was negative. emission tomography–computed tomography, was The histologic differential diagnosis included negative. T-cell receptor (TCR) g chain gene poly- type B follicular LyP, folliculotropic MF, follicular- merase chain reaction (PCR) was performed and did based drug reactions, folliculotropic pityriasis not demonstrate a clonally rearranged population. lichenoides chronica, mucin-poor alopecia muci- Taken together with the clinical features, the find- nosa, and folliculocentric HSV. Many drugs have ings are most consistent with type B follicular LyP. been reported to cause an LyP-type reaction, includ- The patient currently is deferring therapy. ing anticonvulsants, antihypertensives, and even gold acupuncture, but the clinical picture of waxing Comment and waning papules in this patient without cor- Lymphomatoid papulosis generally occurs in adults relation to drug intake ruled out a drug-induced (average age, 45 years) with a male predominance.2 pseudolymphoma.12 Pityriasis lichenoides chronica, This disease falls under the umbrella of CD301 LPDs. which generally heals without scarring, and alopecia In addition to LyP, CD301 LPDs include primary mucinosa, which presents with alopecia, also were cutaneous CD301 large cell lymphoma of anaplas- ruled out based on the clinical presentation.13,14 tic, immunoblastic, or pleomorphic cytomorphology, Chronic or healed herpetic lesions may resemble the as well as borderline types.2,11 CD30 is a cytokine histologic findings seen in LyP, particularly if there belonging to the tumor necrosis factor superfam- are no classic cytologic features of herpes infection ily. Additional CD301 T cell infiltrates that do not (ie, effaced chromatin with beading of the nuclear fall under the CD301 LPD umbrella include drug- membrane, multinucleation, hypereosinophilia of induced eruptions and chronic or healed herpetic the cytoplasm, acantholysis).CUTIS An atypical lymphoid infections.13 Hyperplasia of CD301 cells also may infiltrate including a population of CD301 cells has be seen in arthropod assaults, most notably in asso- been well-described in chronic or healed herpetic ciation with scabies infestation.16,17 Histologically infection. However, CD301 cells in HSV infection distinguishing CD301 LPDs from other causes of typically are scattered throughout the infiltrate, CD301 infiltrates can be challenging, occasionally while they are clustered in LyP, as seen in our case. necessitating clinical correlation to arrive at the In addition, herpetic infections often have a vari- correct diagnosis. ableDo number of admixed B lymphocytes,Not15 which was LymphomatoidCopy papulosis contains atypical lym- not a prominent feature in this case. Negative stain- phoid cells that usually express CD4 (90%), but ing for HSV provided additional evidence against CD42CD81 and CD42CD82 variants occasionally this diagnosis. In rare cases, the histologic features of are encountered. Typically there is variability of LyP and herpetic infection may be indistinguishable, the T-cell antigens, CD2, CD3, CD5, and CD7.3 Figure 1. Follicular-based erythematous papules with central scarring on the chest of a 48-year-old woman. 308 CUTIS® Copyright Cutis 2010. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Follicular Lymphomatoid Papulosis CUTIS A Do Not Copy Figure 2. A folliculotropic and perivascular dermal infiltrate of small to medium–sized lym- phocytes. Small to medium–sized lymphocytes also infiltrated the overlying epidermis (A)(H&E, original magnification 3100). Within the follicle, B the infiltrate included larger atypical lympho- cytes (B)(H&E, original magnification 3400). Lymphomatoid papulosis has been histopathologi- and histiocytes.11,13 Occasionally, type A lesions cally classified into 3 different subtypes: A, B, and C. have multinucleated or Reed-Sternberg–like cells. Overlap between the 3 variants has been well- Type C LyP also contains CD301 cells, but they are described.11 Type A LyP, or the histiocytic type, greater in number and often arranged in clusters or consists of a wedge-shaped infiltrate and is the most sheets; therefore, type C LyP histologically resem- common presentation of LyP. The infiltrate contains bles ALCL but still is differentiated by its clinical large anaplastic CD301 cells, often accompanied by presentation. Anaplastic large cell lymphoma usu- inflammatory cells such as neutrophils, eosinophils,