Multiple painless papulonodules in a 3-year-old girl: type A lymphomatoid papulosis^☆

Dear Editor,

A 3-year-old girl, otherwise healthy, presented with a dermatosis characterized by asymptomatic erythematoviolaceous papules and nodules with slight desquamation on the trunk and lower limbs, one of them with spontaneous ulceration, evolving for 3 months (Figs. 1 and 2). She was in a good general condition and no lymph nodes were palpable. Her mother denied any previous infectious episodes. She had been applying a methylprednisolone aceponate cream for the previous 2 weeks without improvement. She had no significant past medical or family history. A skin biopsy of the lower right limb was performed (Fig. 3). A diagnosis of lymphomatoid papulosis was made.

Figure 1
Erythematoviolaceous papules and nodules with slight desquamation on the lower limbs, one of them with spontaneous ulceration (right thigh).

Figure 2
Erythematoviolaceous papules and nodules with slight desquamation on the abdomen and lower limbs.

Figure 3
Multifocal dermal infiltrate of small lymphocytes, some plasma cells and histiocytes, as well as several lymphoid cells with amphophilic cytoplasm and big nucleus with prominent nucleolus (Hematoxylin & eosin, ×100).

Histopathology showed a multifocal dermal infiltrate of small lymphocytes, some plasma cells, and histiocytes, as well as several lymphoid cells with amphophilic cytoplasm, a big nucleus with the prominent nucleolus, and positivity to CD30, consistent with a type A lymphomatoid papulosis (LyP). The patient was treated with clobetasol propionate 0.5mg/g ointment twice daily with progressive clearance of the lesions and has been symptom-free for the last four years.

LyP is a rare lymphoproliferative disorder that is included in the spectrum of primary cutaneous CD30-positive T-cell lymphoproliferative disorders by the World Health Organization classification of lymphoid tissue tumors.¹ It slightly affects more men than women and usually presents with papulonodular lesions on the limbs and trunk, sometimes with necrosis, developing in crops.²,³ Pruritus is a common feature.²

LyP is a recurrent disease, with a typical waning and waxing course of self-healing lesions.⁴ Lyp is seen most commonly in adults, although it has been suggested that the spontaneous resolution of lesions and their limited number in pediatric age may underestimate its frequency.² In fact, the clinical presentation and course of LyP in childhood differs very little from the adult form.² Albeit being rare among pediatric patients, LyP is one of the two most prevalent cutaneous lymphoproliferative diseases at this age, after mycosis fungoides.⁴

Over the years, the histopathological classification of LyP has been a changing scenario. Currently, five classically recognized histopathological subtypes have been defined, which vary based on the predominant cell type and tropism: type A (mixed cellular), type B (epidermotropic), type C (cohesive infiltrate), type D (epidermotropism), and type E (angiocentric and angiodestructive).¹,³,⁴ The histopathological characterization has no prognostic value nor guides the treatment algorithm. The most common pathologic subtype of LyP is type A (also observed in the present study’s patient), which is characterized by a wedge-shaped dense dermal perivascular lymphoid infiltrate with large atypical CD30-positive cells with Reed-Sternberg appearance.²,⁵

The differential diagnosis of LyP includes both forms of pityriasis lichenoides (pityriasis lichenoides et varioliformis acuta – PLEVA – and pityriasis lichenoides chronica – PLC), primary cutaneous anaplastic large cell lymphoma (ALCL), a papular variant of mycosis fungoides and arthropod bites. More remotely, but mainly in childhood, other diagnoses such as scabies, pityriasis rosea, guttate psoriasis, and eczema should be considered.¹, ², ³ Some clinical tips may help in differentiating these diseases. For instance, as opposed to LyP, primary cutaneous ALCL presents usually as a solitary lesion and PLEVA presents with smaller lesions that usually do not show a waxing and waning nature and heal without scarring.³

In the absence of molecular or immunohistochemical criteria to predict LyP evolution, the clinicopathological correlation and long-term follow-up are indispensable. In fact, although LyP has classically a benign and indolent clinical course, some cases progress to other types of lymphoma, such as primary cutaneous ALCL, mycosis fungoides, or Hodgkin disease.¹,⁴ Fortunately, in pediatric patients, progression to lymphoma is exceptional.² Moreover, some factors such as association with viral infections, atopic dermatitis, skin eosinophilic infiltration, and spontaneous complete remission strongly suggest that pediatric LyP could be considered a reactional disease rather than a neoplastic disorder.²

The long-term prognosis of pediatric LyP is generally considered to be good, with a 10-year disease-related survival rate of 100%.¹, ², ³ First-line treatment generally comprises therapeutic abstention or skin-directed modalities such as topical steroids.¹,³ Methotrexate and phototherapy are also commonly prescribed.⁵ There is no decreased risk of lymphoma in patients submitted to treatment, whereby the ‟wait and see” approach is also often applied.¹,⁵

• Financial support
  None declared.
• ☆
  Study conducted at the Centro Hospitalar Universitário de São João EPE, Porto, Portugal.

References

• ¹
  Martinez-Cabriales SA, Walsh S, Sade S, Shear NH. Lymphomatoid papulosis: an update and review. J Eur Acad Dermatol Venereol. 2020;34:59–73.
• ²
  Miquel J, Fraitag S, Hamel-Teillac D, Molina T, Brousse N, Prost Y, et al. Lymphomatoid papulosis in children: a series of 25 cases. Br J Dermatol. 2014;171:1138–46.
• ³
  Wieser I, Wohlmuth C, Nunez CA, Duvic M. Lymphomatoid papulosis in children and adolescents: a systematic review. Am J Clin Dermatol. 2016;17:319–27.
• ⁴
  Kempf W, Kazakov DV, Belousova IE, Mitteldorf C, Kerl K. Paediatric cutaneous lymphomas: a review and comparison with adult counterparts. J Eur Acad Dermatol Venereol. 2015;29:1696–709.
• ⁵
  Fernández-de-Misa R, Hernández-Machín B, Servitje O, Valentí Medina F, Maroñas-Jiménez L, Ortiz-Romero PL, et al. First-line treatment in lymphomatoid papulosis: a retrospective multicentre study. Clin Exp Dermatol. 2018;43:137–43.

Publication Dates

• Publication in this collection
  30 Sept 2022
• Date of issue
  Sep-Oct 2022

History

• Received
  24 July 2020
• Accepted
  08 Nov 2020
• Published
  13 July 2022