Primary cutaneous plasmacytosis

  • Kerkvliet A
  • Shipp D
  • Pride H
 et al. 
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Background: Cutaneous plasmacytosis is a rare entity that has been reported almost exclusively in Asian countries and is usually seen in adult males. Primary cutaneous plasmacytosis clinically is characterized by multiple red-brown plaques and nodules typically located on the trunk. The condition can be systemic if polyclonal hypergammaglobulinemia, lymphadenopathy, cutaneous lesions, and systemic symptoms such as fever, weight loss, and fatigue are present. We present a case of primary cutaneous plasmacytosis in a child. Case report: A 4-year-old white female presented to the dermatology clinic with a 6-month historyofacircular pruritic 1-cmerythematous and edematous plaque with a scant degree of scale on the right forearm. In addition, the patient had asymptomatic tiny umbilicated papules on the trunk and extremities, clinically consistent with molluscum contagiosum. No lymphadenopathy was noted. The patient had been treated with bactroban, amoxicillin, and hydrocortisone without improvement. The clinical impression was granulomatous reaction to molluscum, ruptured cyst, persistent nummular eczema, granuloma annulare, or other granulomatous process. A biopsy from the right forearm showed a dense lymphoplasmacytic lichenoid and vacuolar interface dermatitis with pseudoepitheliomatous hyperplasia. The differential diagnosis included arthropod bite, borrelial lymphocytoma, syphilis, and early lichen striatus. CD3 and CD20 revealed a reactive pattern of dermal lymphocytes. In situ hybridization staining for kappa and lambda light chains demonstrated a normal polyclonal restriction pattern. No clonal gene rearrangements were detected in the T and B cell populations. PAS stain was negative for fungus and a Steiner stain was negative for spirochetes. Serologic studies for syphilis and Borrelia burgdorferi were negative. The patient was treated with surgical excision, and has had no additional symptoms after 8 months of follow-up. Discussion: The exact pathophysiology of primary cutaneous plasmacytosis remains unclear. An association with multicentric Castleman disease has been postulated and development of malignant T-cell lymphoma has been reported. Primary cutaneous plasmacytosis is extremely rare in children, however, the dermatologist and dermatopathologist must be aware of this entity and its associations. It is essential to evaluate the patient for systemic disease and the development of a more aggressive disorder.

Author-supplied keywords

  • Asian
  • B lymphocyte
  • Borrelia burgdorferi
  • CD3 antigen
  • T cell lymphoma
  • adult
  • amoxicillin
  • angiofollicular lymph node hyperplasia
  • arthropod
  • biopsy
  • case report
  • cell population
  • child
  • cyst
  • dermatitis
  • dermatologist
  • dermatology
  • differential diagnosis
  • diseases
  • excision
  • fatigue
  • female
  • fever
  • follow up
  • forearm
  • fungus
  • gene rearrangement
  • granuloma annulare
  • granulomatosis
  • granulomatous inflammation
  • hospital
  • human
  • hydrocortisone
  • hypergammaglobulinemia
  • hyperplasia
  • in situ hybridization
  • lichen striatus
  • lichenoid
  • light chain
  • lymphadenopathy
  • lymphocyte
  • lymphocytoma
  • male
  • molluscum contagiosum
  • nummular dermatitis
  • papule
  • pathophysiology
  • patient
  • periodic acid Schiff stain
  • plasmacytosis
  • pseudomonic acid
  • skin defect
  • spirochete
  • stain
  • staining
  • syphilis
  • systemic disease
  • weight reduction

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  • A Kerkvliet

  • D Shipp

  • H Pride

  • T Ferringer

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