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Co-Occurrence of Lymphomatoid Papulosis and Mycosis Fungoides in a Young Female: a Case Report Natalie Steinhoff, DO,* Brian Wanner,** Matthew H

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Co-Occurrence of Lymphomatoid Papulosis and Mycosis Fungoides in a Young Female: a Case Report Natalie Steinhoff, DO,* Brian Wanner,** Matthew H Co-Occurrence of Lymphomatoid Papulosis and Mycosis Fungoides in a Young Female: A Case Report Natalie Steinhoff, DO,* Brian Wanner,** Matthew H. Mahoney, MD*** *Dermatology Resident, PGY-3, Nova Southeastern University College of Osteopathic Medicine, Largo Medical Center, Largo, FL **Osteopathic Medical Student, 4th year, Des Moines University College of Osteopathic Medicine, Des Moines, IA ***Dermatologist and Clinical Faculty, Nova Southeastern University College of Osteopathic Medicine, Largo Medical Center, Largo, FL Disclosures: None Correspondence: Natalie Steinhoff, DO; NSUCOM/Largo Medical Center; 201 14th St. SW, Largo, FL 33770; [email protected] Abstract There have been reports of lymphomatoid papulosis (LyP) and mycosis fungoides (MF) presenting separately, simultaneously, and consecutively. LyP is a CD30+ lymphoproliferative disorder, and MF is the most common cutaneous T-cell lymphoma (CTCL). LyP most often presents with self-regressing, erythematous-to-brown papules or small nodules. Mycosis fungoides has a variety of clinical manifestations, but in early stages it commonly presents as an erythematous patch or plaque. Recognizing LyP is important due to its association with development of secondary malignancies, most commonly MF, Hodgkin’s disease, and anaplastic large-cell lymphoma (ALCL). We report a case of a 35-year-old woman with recurrent LyP lesions since childhood who went on to develop concurrent LyP and MF. We discuss the need to evaluate LyP patients for secondary lymphomas and provide a concise review of the literature focusing on clinical presentation, diagnosis, disease associations, and management. microabscesses (mycosis cells in the epidermis), Spontaneous self-regression of lesions is required Introduction 3 19 Lymphomatoid papulosis (LyP) is one of two and a band-like dermal infiltrate of lymphocytes. for establishing a clinical diagnosis of LyP. Variable expression of CD30 can be seen at all Identifying LyP is of great importance, because CD30+ cutaneous lymphoproliferative disorders 4 (the other being primary cutaneous anaplastic stages of MF. It is important to recognize early 10% to 30% of patients will develop a secondary MF, as one in 10 early-stage MF patients will lymphoma.1,16 Mycosis fungoides (MF), along large-cell lymphoma [PC-ALCL]). LyP is 20 slightly more common in males than females, with progress to advanced disease stages. with cutaneous ALCL, are the two most common a male-to-female ratio of about 1.4, and occurs most frequently in the fifth decade of life.12,19,20 LyP lesions can, however, occur at any age. LyP commonly presents in a singular or multiple distribution with erythematous-to-brown papules and/or nodules on the trunk or extremities.1,20 Half of LyP patients develop asymptomatic lesions that self-regress in weeks to months; the rest develop pruritus, ulceration, and necrosis, with scarring and/or postinflammatory hyper/ hypopigmentation as lesions involute.1,13,19,20,22 The clinical hallmark of LyP is the waxing and waning of lesions, which helps differentiate it from ALCL and CD30+ MF tumors.20 While the clinical picture for LyP is benign, careful microscopic examination often reveals a malignant- appearing histology.1,2,5,10,13 There are five recognized histopathological subtypes of LyP, of which type Figure 1. Erythematous, ulcerated nodule on the A is the most frequently encountered. Type A is Figure 3. Punch biopsy of posterior neck characterized by a wedge-shaped lymphocytic right buttock. demonstrating a wedge-shaped, perivascular infiltrate mixed with neutrophils, eosinophils, and interstitial inflammatory infiltrate. Focal and histiocytes. Type B looks similar to MF, with epidermal necrosis is also seen. small to medium lymphocytes with cerebriform nuclei and epidermotropism. Type C resembles ALCL, with sheets of large CD30+ lymphocytes. Type D has a predominantly CD8+ cytotoxic T-cell infiltrate, and type E is characterized by angiodestructive lymphocytic infiltrates that are both CD30+ and CD8+. Three other variants have been proposed, including type F, which involves a follicular LyP, and variants with γ/δ T-cell LyP phenotype and 6p25.3 rearrangement.20 MF is the most common CTCL and frequently presents as a patch or plaque, usually without lymph-node or visceral involvement. Advanced- stage MF encompasses extracutaneous disease or advanced skin lesions, such as tumors. The classic MF presentation is erythematous, scaly patches that more frequently occur in sun-protected areas and may advance into raised plaques, nodules or tumors.4 There are also reports of MF presenting with erythroderma or pigment alteration.3 On Figure 4. Posterior neck biopsy demonstrating a histology, MF presents with atypical T-cells with Figure 2. Erythematous, scaly patch on the lymphocytic infiltrate with admixed eosinophils cerebriform nuclei, epidermotropism, Pautrier left flank. and neutrophils. CO-OCCURRENCE OF LYMPHOMATOID PAPULOSIS AND MYCOSIS FUNGOIDES IN A YOUNG FEMALE: A CASE REPORT secondary lymphomas associated with LyP. (male-to-female ratio about 1.4), and a study by According to Wieser and Tetzlaff et al., MF should Wieser et al. concluded that male sex and histologic be considered in an LyP patient with erythematous subtypes B and C were associated with a higher rate patches or large, non-regressing lesions. The of secondary malignancy (type D was less frequently number and severity of LyP lesions do not predict associated). This is reasonable, as MF histologically the development of secondary malignancies, resembles type B LyP.19 Studies have conflicted, an important consideration in diagnosing and however, on that point; de la Garza Bravo et al. following LyP patients.20 suggested type A LyP is most commonly associated with MF.4 Case Report A 35-year-old female with a past medical history The etiologies of LyP and MF are unclear, but of recurrent LyP (diagnosed at age 10) and MF epidemiologic findings and biologic features of (diagnosed at age 28) presented with a painful red each disease point to the possibility of an infectious nodule on her right posterior neck and right buttock cause.15 Several studies have hypothesized a viral (Figure 1) and an erythematous, scaly patch on the cause of LyP but failed to detect Epstein-Barr virus, left flank (Figure 2). In the past, the patient’s LyP human herpesviruses 6, 7, or 8, or human T-cell lesions were treated with topical 0.05% clobetasol leukemia/lymphoma virus in LyP lesions.8-10,16 In cream and triamcinolone acetonide injections, regard to MF patients, some studies have found both with minimal improvement, and the MF human T-lymphotropic virus type I in blood lesions were treated with intermittent clobetasol. A work and cutaneous lesions. An equal number of Figure 5. Posterior neck biopsy demonstrating workup in 2009 for systemic disease was negative studies, however, provide evidence against a role for lymphocytes with positive immunoreactivity and included bloodwork, chest X-ray, and CT scans human T-lymphotropic virus type I. Studies have for CD30. of the chest and abdomen. also failed to show an association between MF and Epstein-Barr virus, human herpesvirus 6, 7, and 8, A punch biopsy from the right posterior neck cytomegalovirus, and Merkel cell polyomavirus.15 and left flank were collected. Both were fixed in Another study suggests hydrochlorothiazide may 10% neutral buffered formalin and submitted for act as an antigenic catalyst and lead to early MF. microscopic examination. The biopsy report of Jahan-Tigh et al. showed 29% of patients achieved the neck lesion noted a focally necrotic epidermis partial or complete remission after discontinuing above a wedge-shaped, perivascular and interstitial hydrochlorothiazide, and 20% of patients whose infiltrate of lymphocytes mixed with eosinophils CTCL preceded hydrochlorothiazide therapy and neutrophils (Figures 3, 4). Melanocytes had worsening of their rash after initiating demonstrated large, atypical nuclei. Additionally, the medication.6 occasional atypical mitotic figures were identified. Lymphocyte immunoreactivity demonstrated light While the etiologies of LyP and MF remain a positivity for CD8 and positivity for CD3, CD4, mystery, multiple studies have demonstrated LyP and CD30 (Figure 5). The biopsy report of the left and MF may share a common cellular origin and flank revealed a psoriasiform acanthotic epidermis could represent a continuum of a single disease above a band-like infiltrate of lymphocytes process.4 These studies demonstrated an identical (Figures 6, 7). Intraepidermal lymphocytes with pattern of monoclonal T-cell receptor (TCR) gene cerebriform nuclei were seen along with collections rearrangements in both LyP and MF lesions via of lymphocytes in the spinous zone with little or polymerase chain reaction and Southern blot analysis no spongiosis. The intraepidermal lymphocytes of biopsied tissue.1,2,4,21 As of April 2015, a total of demonstrated positive immunoreactivity for CD3 31/44 patients (70.45%) have shown identical TCR Figure 6. Punch biopsy from left flank and CD4 as well as negative immunoreactivity for gene rearrangement patterns in their respective LyP CD8 and CD30. Lymphocytes were also present and MF lesions. Basarab et al. reported identical demonstrating a band-like inflammatory within the follicular adnexal epithelium. TCR clones in three of 15 patients with LyP and infiltrate beneath an epidermis showing slight MF. 1 Chott et al. reported similar findings in two psoriasiform acanthosis. Lymphocytes are also Due to the recurring and self-limiting nature of out of three patients with LyP and MF.2 Wood et present within follicular adnexal epithelium. LyP, we opted for symptomatic treatment only. al. detected identical TCR clones in two patients The approach for MF will be treatment with with LyP and MF.21 Zackheim et al. found identical mechlorethamine or possibly narrow-band UV-B, TCR gene rearrangements in all seven of the along with extensive workup for systemic disease, patients with LyP and MF who had tissue available including complete blood count with differential, for clonality analysis.22 Six out of 12 patients in a blood chemistry, lactate dehydrogenase, chest X-ray, study by Gallardo et al.
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