Lymphomatoid Papulosis: A Rarer Condition In Pediatric Population

INTRODUCTION

Lymphomatoid papulosis (LyP) is a benignchronic often relapsing skin condition thatbelongs to the CD30-positive cutaneouslymphoproliferative disorders. (1) LyPtypically presents as crops of lesions with a tendency to self-resolve, and morphologycan range from solitary to agminated ordiffuse papules and plaques to nodules ortumours. (2) Patients affected with lymphomatoid papulosis are at risk of developing another hematological disorder. Lyp is rare among adults and even rarer among children. (3) A 14-year-old girl diagnosed with LyP with acute-onset papulonodular lesions is presented.

CASE REPORT

A 14-year-old female patient presented withscattered papuloulcerative lesions andnodular lesions with necrotic crusts. Lesionsstarted as unilateral post-auricularerythematous nodule 2 weeks ago. Physicalexamination revealed erythematous nodular lesions of variable diameter with a necroticcrust in the centre, distributed mainly on the extremities, some parts of the trunk, breasts, lower abdomen and thighs. (Fig 1A, 1B, 1C) The lesions were painful but not associated with fever or systemic symptoms. No lymphadenopathy was noted but bilateral axillary lymph node enlargementwas seen on ultrasound. Laboratoryevaluation was normal. Testing for syphilisand HIV were negative.

A punch biopsy was performed of a lesion on the right forearm. Histopathological examination revealed large atypical lymphocytes, mature lymphocytes and eosinophils around perivascular, perifollicular and eccrine glands throughout the dermis. (Fig 2A, 2B) Immunohistochemical staining showed CD3 diffuse positive, CD7 expression was decreased and CD30 diffuse positive. (Fig 3A, 3B, 3C) The final histopathological diagnosis was CD30-positive lymphoproliferative disorder. We considered it to be LyP type A clinically, morphologically and immunohistochemically.

DISCUSSION AND CONCLUSIONS

LyP is a benign chronic skin condition that requires histopathology confirmation with immunophenotypic and genotypic assessment with the exclusion of malignancies. (1) Due to the increased risk of developing a secondary lymphoma, long-term follow-up examinations and proper patient counseling are warranted.(3) Pediatric LyP has garnered attention as a diagnosis with unique clinical andhistopathologic features. (2) While type A appears to be the dominant variantdescribed in children, types B, C, and eventhe newly described variants E and F mayoccur more often than previously reported. Further studies into the distinguishingfeatures of this disease will aid clinicians in recognizing LyP in the pediatric population. (1)