Lymphomatoid papulosis (LYP) is a rare cutaneous CD30+ lymphoproliferative disorder (LPD), first described by the dermatologist Warren L. Macaulay in 1968. Primary cutaneous CD30+ LPDs are the second-most common group of cutaneous T-cell lymphomas (CTCLs) after mycosis fungoides (MF) and represent approximately 25% of all CTCLs. LPDs include LYP, primary cutaneous anaplastic large cell lymphoma (PcALCL), and CD30+ borderline lesions. LYP is characterized by recurrent self-healing papulonecrotic or papulonodular eruptions that follow a waxing and waning course over the years. Although the histopathological features are malignant, the disease is considered benign due to its excellent prognosis. However, 10%–20% of patients with LYP develop lymphoid neoplasms in adults. The disease is well documented in adults, whereas LYP is rare in the pediatric population and its comprehensive data in children are scarce in the literature.
We describe a case of a 16-year-old adolescent with LYP.
A 16-year-old boy presented with a 4-year history of asymptomatic, recurrent, papulonodular, and few necrotic lesions on the lower extremities, arms, trunk, right axilla, and right inguinal areas. The lesions started healing with hyperpigmentation and some with atrophic scars. There was no history of atopy or any other systemic symptoms. He was otherwise healthy, well-developed adolescent. No family member had a similar complaint. On physical examination, there were clusters of discrete, nontender, erythematous papules, infiltrated nodules, and a few necrotic ulcers in the abovementioned anatomical sites [Figures 1 and 2]. There were healed hyperpigmented macules and a few atrophic scars. No generalized lymphadenopathy or hepatosplenomegaly was noted. The rest of the physical examination was normal.
Complete blood count, differential counts, and serum chemistry with liver function studies were normal. Serum lactate dehydrogenase was 162 U/l (normal: 120–246 U/l). Serological tests for HIV, hepatitis C virus, and hepatitis B virus were negative. Chest radiograph and abdominal scan were normal. Two 5 mm punch biopsies were taken from the active papular lesion and necrotic ulcer. Histopathological examination from both specimens revealed a dense diffuse infiltrate of atypical cells with vesicular nuclei involving the whole of reticular dermis and extending to subcutis. There were no eosinophils or plasma cells in the infiltrate. Scant epidermal component with no evidence of epidermotropism was present [Figures 3 and 4]. On immunohistochemistry (IHC), these atypical cells were positive for CD3, CD4, CD30 (stains 50% cells), MUM-1, perforin, granzyme, TIA, and focally positive for CD56 and negative for CD20, CD8, ALK, and Epstein–Barr virus [Figure 5]. These features are suggestive of type A LYP. Based on the clinical, histopathological features and IHC studies, a diagnosis of CD30+ LYP was made.
He was treated with narrowband ultraviolet B (NBUVB) phototherapy twice weekly for 3 months. The majority of the lesions regressed. The patient is currently under follow-up. His parents were counseled about the possible risk of malignancy and the importance of lifelong follow-up.
In general, LYP presents similarly in adults and children. LYP can occur at any age in adults (peak incidence 5th decade), and there is no sex predilection. According to the recently published systemic review of LYP in children, the mean age among the pediatric cases reported was 9.3 ± 4.6 years with the youngest patient being 11 months age. Compared with adults, more males were affected than females. The clinical features of children with LYP did not differ greatly from the presentation in adults. Lesions were red to brown, papules or nodules. These healed either with post inflammatory hypo- or hyperpigmentation, with or without scar formation. The common anatomical sites affected were the lower extremities, followed by the upper extremities and trunk. The face, buttocks, and genitals were less frequently involved. Histopathological findings were the same in both children and adults. LYP is broadly classified into five subtypes (A, B, C, D, and E) on the basis of histopathological findings. The characteristic features are wedge-shaped dense dermal infiltrate of lymphoid cells with numerous eosinophils, neutrophils, and atypical small and large lymphocytes which are CD30 positive. Epidermotropism and pautrier abscesses are usually not seen. The predominant histopathologic subtype A was seen in children. Pityriasis lichenoides (PL) and associated lymphomas were diagnosed before, with and after LYP in 19 and 14 cases, respectively. Of these 14 (5.6%) associated lymphomas, two children developed systemic anaplastic large cell lymphoma (ALCL), one with hypopigmented MF and the rest with PcALCL. CD30 staining was reported in 104 and CD8 positivity in 15 children.
Miquel et al. in their series of 25 children with LYP observed lesions on unusual sites such as genitals, tongue, and oral cavity. Pruritus was the chief symptom. The mean age at onset was 7.5 years. Unlike previous studies, PL in 36% of cases, atopic dermatitis in 28%, and nonspecific infections in 28% of cases were associated with LYP. Complete remission was observed in 44% of cases. None of their patients developed lymphomas even after 10-year follow-up. Histopathological subtype A was predominant (82%). A marked eosinophilic infiltrate was present in 44% of cases. The authors were of the opinion that pediatric LYP might be a reactional disease rather than a neoplastic disorder. Due to the high frequency of atopy in these children, they believed atopic dermatitis may be a potential predisposing factor for LYP in children.
Nijsten et al. also found more than two-thirds of their patients were atopics, which was significantly more than the expected prevalence of atopy in the general population. Of the 35 LYP patients, 3 (9%) developed Non-Hodgkin lymphomas (NHL). None of their patients had died of extracutaneous disease. Compared with the general population, patients with childhood-onset of LYP have a significantly increased risk of developing NHL. NHL (MF and ALCL) may occur concurrently with LYP or follow LYP in children.
Childhood LYP should be differentiated from three conditions: cutaneous/systemic ALCL, PL, and arthropod or insect bites. In general, cutaneous ALCL tumoral lesions are larger (>2 cm) than LYP lesions and present as solitary nodules in 80% of cases and progressive in nature. Whereas LYP lesions are smaller (<2 cm), more diffuse, self-limited, and do not progress with time. To distinguish cutaneous ALCL and LYP, longitudinal observation is often necessary because of overlapping histopathological and clinical features. Clinically, PL lesions are smaller than LYP lesions and do not usually show waxing and waning course. Although CD30 expression is disease-specific for LYP, CD30+ PL cases have been reported. In such cases, it is much more difficult to differentiate LYP and PL. Insect bite eruptions are commonly seen on the uncovered areas of the body, particularly extremities and associated with severe itching and tissue eosinophilia. Such arthropod bites may be misleading with the lesions of CD30 absent childhood LYP.
LYP exhibits favorable prognosis. The 5-year cumulative survival rate for children and adults is excellent (99%). Therefore a “wait-and-watch” strategy can be justified in the management of LYP, as there is no proved therapeutic modality that alters the course of the disease or prevents LYP-associated lymphomas. Multidrug chemotherapy should be avoided because of its severe adverse effects. For disseminated, troublesome lesions, phototherapy (psoralen+ultraviolet light A (PUVA)/NBUVB) and low-dose methotrexate are the first-line therapies. In view of the increased risk for secondary lymphomas, all children with LYP should be monitored throughout their lives.
LYP is a rare disease in children. The clinical, histopathological features and the risk of developing secondary lymphomas are similar to that of adult LYP. Clinicopathological correlation is crucial to diagnose these cases to avoid erroneous treatments. The disease is benign and prognosis is excellent. Due to the increased risk (10%–20%) of developing a secondary lymphoma, long-term follow-up and proper patient counseling is mandatory.
Declaration of consent
The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
1. Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas Blood. 2019;133:1703–14
2. Kempf W. Cutaneous CD30-positive lymphoproliferative disorders Surg Pathol Clin. 2014;7:203–28
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. Miquel J, Fraitag S, Hamel-Teillac D, Molina T, Brousse N, de Prost Y, et al Lymphomatoid papulosis in children: A series of 25 cases Br J Dermatol. 2014;171:1138–46
5. Nijsten T, Curiel-Lewandrowski C, Kadin ME. Lymphomatoid papulosis in children: A retrospective cohort study of 35 cases Arch Dermatol. 2004;140:306–12
6. Queller JN, Bognet RA, Kozic H, Lee JB, Sahu J, Hyde PM. A case of mycosis fungoides and lymphomatoid papulosis occurring simultaneously in a child J Clin Aesthet Dermatol. 2012;5:46–8
7. Min JA, Oh ST, Kim JE, Cho BK, Chung NG, Park HJ. Lymphomatoid papulosis followed by anaplastic large cell lymphoma in a pediatric patient Ann Dermatol. 2010;22:447–51
8. Rifkin S, Valderrama E, Lipton JM, Karayalcin G. Lymphomatoid papulosis and Ki-1+anaplastic large cell lymphoma occurring concurrently in a pediatric patient J Pediatr Hematol Oncol. 2001;23:321–3