Clinical Characteristics and Disease Course in Black Patients With Lymphomatoid Papulosis: A Case Series

the LyP lesions resulted in hyperpigmented macules and scars (Fig. 1A-C). Seven patients were followed for 1 to 8 years, and most the patients (4/7, 57%) had documentation of active disease on their last visit despite different treatment approaches.

Major racial differences in incidence among cutaneous lymphoma subtypes have been reported.^3,6 AA/Blacks have statistically higher incidence ratios of CTCL and MF than other races and a trend towards lower incidence of CD30+ LPD was found in a national US database, which included 31 AAs with CD30+ LPD.³ A more recent study of another database included 153 AA patients with CD30+ LPD who had a significantly shorter overall survival compared to Caucasians after adjusting for patient disease characteristics, socioeconomic factors and types of treatment.⁵ Our case series and three additional case reports suggest an indolent disease course of LyP in AA/Black patients. There are several possible explanations for the previous findings on poor survival in AA patients with CD30+ LPD. These results might be due to inclusion of ALCL and borderline cases with poorer prognosis compared to LyP. Another possibility is that patients with more aggressive CTCL variants (eg, transformed tumor-stage MF) might have been misdiagnosed or miscoded as CD30+ LPD. Our case series highlights the need for additional studies before clinical recommendations can be made regarding prognosis and treatment of LyP and CD30+ LPD in the different race groups. Our results support previous reports on an earlier-onset of disease seen in AA/Black patients with LyP⁵ as well as with other CTCL subtypes, such as MF.⁷ A delay in diagnosis of LyP is not uncommon since the self-healing crops of papules and nodules can be easily misdiagnosed as other malignant or inflammatory skin conditions (eg, arthropod bites). Most of the Black patients in our series had concurrent MF. The diagnosis of early-stage patch stage MF may be more difficult in Black skin where erythema is less pronounced compared to lighter skin types. Careful physical examination should be performed in Black patients who are diagnosed with LyP and no known history of MF. Once the diagnosis of LyP is made, several treatment approaches are possible. Noninterventional (“wait-and-see”) strategy is a legitimate approach, especially in patients with a limited number of lesions. Topical and skin-directed therapies (including topical steroids and phototherapy), and low-dose methotrexate are the best documented therapies for LyP.⁸ Our patients demonstrated heterogenous responses to different therapies used, with most of the patients still suffering from active disease after a follow-up periods of up to 8 years, highlighting that there is currently no curative therapy for LyP. The efficacy and safety of brentuximab vedotin, an antibody- drug conjugate directed against CD30, has recently been assessed for the treatment of LyP in 12 patients, including 2 AAs.⁹ Brentuximab vedotin was reported to be effective in treating LyP and has been suggested as a possible therapy in severe and refractory cases. Further studies are ongoing to optimize its dosing and to minimize adverse events.^9,10

In conclusion, a diagnosis of LyP should be considered in Black patients who present with recurring eruption of papules or nodules that resolve spontaneously. Patients with LyP should be carefully examined for concurrent or later development of MF. Although an indolent course may be expected in Black patients, residual hyperpigmentation and scars following resolution of the LyP lesions are common in this population, highlighting the need for better treatments of this disorder in the Black population.

The authors have no conflicts of interest to declare. This research was funded in part through the NIH/NCI Cancer Center Support Grant P30 CA008748.

 

1. Kempf W, Pfaltz K, Vermeer MH, et al. EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood. 2011;118(15):4024-4035. 
2. Angra K, Kennedy LJ, Rodney IJ. Lymphomatoid papulosis in a young adult of African descent. JAAD Case Rep. 2015;1(4):212-214. 
3. Bradford PT, Devesa SS, Anderson WF, Toro JR. Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases. Blood. 2009;113(21):5064-5073. 
4. Yu JB, Blitzblau RC, Decker RH, Housman DM, Wilson LD. Analysis of primary CD30+ cutaneous lymphoproliferative disease and survival from the Surveillance, Epidemiology, and End Results database. J Clin Oncol. 2008;26(9):1483-1488. 
5. Su C, Nguyen KA, Bai HX, et al. Ethnic disparity in primary cutaneous CD30(+) T-cell lymphoproliferative disorders: an analysis of 1496 cases from the US National Cancer Database. Br J Haematol. 2018;181(6):752-759. 
6. Geller S, Pulitzer M, Myskowski P, Kheterpal M. Low-G rade cutaneous b-cell lymphoma in African American Patients. J Drugs Dermatol. 2018;17(12):1334 - 1337. 
7. Su C, Nguyen KA, Bai HX, et al. Racial disparity in mycosis fungoides: An analysis of 4495 cases from the US National Cancer Database. J Am Acad Dermatol. 2017;77(3):497-502.e492. 
8. Fernandez-de-Misa R, Hernandez-Machin B, Servitje O, et al. First-line treatment in lymphomatoid papulosis: a retrospective multicentre study. Clin Exp Dermatol. 2018;43(2):137-143. 
9. Lewis DJ, Talpur R, Huen AO, Tetzlaff MT, Duvic M. Brentuximab vedotin for patients with refractory lymphomatoid papulosis: an analysis of phase 2 results. JAMA Dermatol. 2017;153(12):1302-1306. 
10. Geller S, Myskowski PL, Kim YH, Moskowitz A, Horwitz S. The optimal regimen of brentuximab vedotin for CD30(+) cutaneous lymphoma: are we there yet? Br J Dermatol. 2018;178(2):571 

Shamir Geller MD shamirgeller@gmail.com