Pediatric Dermatology Vol. 31 No. 6 e120–e121, 2014
Isolated Benign Primary Cutaneous Plasmacytosis in a Child Abstract: Isolated benign primary cutaneous plasmacytosis in a child is a very rare and benign disease. Herein we present a case of this condition occurring in a child who showed good response to topical corticosteroid. Figure 1. A 1-cm 9 3-cm solitary erythematous plaque on the right knee.
Plasma cell proliferations can be seen in a wide spectrum of diseases. Primary cutaneous plasmacytosis (PCP) is a rare benign skin disorder of polyclonal plasma cell proliferation in the skin without underlying systemic diseases that cause secondary infiltration by plasma cells (1). PCP is characterized by solitary or multiple red-brown plaques, mainly involving the trunk, and histologically by the marked hyperplasia of benign mature plasma cells in the dermis (1). The majority of PCP cases occur in adults, but several cases of isolated benign PCP in children have been reported (1,2). PCP should be differentiated from other conditions that result in plasma cell infiltrates in the skin, such as secondary syphilis, primary cutaneous plasmacytoma, cutaneous lymphoid hyperplasia, and nodular amyloidosis (1–3). Herein we present a case of isolated PCP occurring in a child who had good response to topical corticosteroid. A 3-year-old girl had a 1-year history of an asymptomatic skin lesion on the right knee. Physical examination showed a 1-cm 9 3-cm solitary erythematous plaque (Fig. 1). She had no history of trauma, infections, or insect bites. The superficial lymph nodes, liver, and spleen were not palpable. There was no family history of malignancy or autoimmune disease. Her serum protein and immunoelectrophoresis were within normal limits. Serologic analysis for antinuclear antibodies was negative. Histologic examination of a skin biopsy specimen showed irregular acanthosis and dense inflammatory cell infiltrate consisting largely of mature plasma cells in the
DOI: 10.1111/pde.12449
e120
superficial and middermis (Fig. 2A, B). Immunoperoxidase stains for kappa and lambda light chains demonstrated polyclonal populations of plasma cells (Fig. 2C, D). The diagnosis of PCP was made by a combination of clinical and histologic findings without evidence of other systemic disease such as malignant conditions, autoimmune diseases, or infections (1). PCP could be excluded by demonstrating the polyclonality of infiltrating plasma cells. Cutaneous involvement of multiple myeloma was also excluded by the absence of immature forms of plasma cells and no evidence of M proteins in blood and urine (1,2,4). PCP in adults is thought to require close observation because there have been several reports of multiple cutaneous plasma cell infiltrates and extracutaneous manifestations, which lead to a fatal outcome. PCP in children has rarely been reported, and some authors have suggested that PCP in children may not be the same entity as PCP in adult. In addition, PCP in children is known to respond well to topical or systemic corticosteroids (2,4). The pathomechanism of idiopathic plasma cell infiltrates in PCP is unknown, although it has been hypothesized that high serum levels of interleukin-6 induces maturation of B-lymphocytes to plasma cells (5). Treatment options for PCP include systemic, topical, or intralesional injection of corticosteroids; topical tacrolimus; pulsed dye laser; and excision (2,3). In consideration of her age and cosmetic outcome, we initiated treatment with topical corticosteroids (0.3% diflucortolone valerate ointment). After 1 month, the lesion decreased in size and the indurated erythema had mostly disappeared. A topical steroid can be tried before considering more aggressive treatments in children.
© 2014 Wiley Periodicals, Inc.
Brief Report e121
A
B
C
D
Figure 2. (A) Dense lymphoplasma cell infiltration in the superficial and middermis (hematoxylin and eosin, 409). (B) Mature plasma cells without cellular atypia in the dermis (hematoxylin and eosin, 4009). Cells of the infiltrate stained for (C) kappa light chains and (D) lambda light chains.
REFERENCES 1. Arico M, Bongiorno MR. Primary cutaneous plasmacytosis in a child. Is this a new entity? J Eur Acad Dermatol Venereol 2002;16:164–167. 2. Ahn JJ, Yang YS, Shin MK et al. Case of isolated benign primary cutaneous plasmacytosis in a child. J Dermatol 2011;38:364–367. 3. Gilliam AC, Mullen RH, Oviedo G et al. Isolated benign primary cutaneous plasmacytosis in children: two illustrative cases. Arch Dermatol 2009;145:299–302. 4. Uhara H, Saida T, Ikegawa S et al. Primary cutaneous plasmacytosis: report of three cases and review of the literature. Dermatology 1994;189:251–255. 5. Yamamoto T, Katayama I, Nishioka K. Increased plasma interleukin-6 in cutaneous plasmacytoma: the effect of intralesional steroid therapy. Br J Dermatol 1997;137:631–636.
Hye Rang On, M.D.* Sang Eun Lee, M.D., Ph.D.* You Chan Kim, M.D., Ph.D.† Soo-Chan Kim, M.D., Ph.D.* *Department of Dermatology, Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea †Department of Dermatology, Ajou University School of Medicine, Seoul, Korea Address correspondence to Soo-Chan Kim, M.D., Department of Dermatology, Gangnam Severance Hospital, Yonsei University College of Medicine, Eonjuro 712, Gangnam-gu, Seoul 135–720, Korea, or e-mail: [email protected].
Isolated Benign Primary Cutaneous Plasmacytosis in a Child Abstract: Isolated benign primary cutaneous plasmacytosis in a child is a very rare and benign disease. Herein we present a case of this condition occurring in a child who showed good response to topical corticosteroid. Figure 1. A 1-cm 9 3-cm solitary erythematous plaque on the right knee.
Plasma cell proliferations can be seen in a wide spectrum of diseases. Primary cutaneous plasmacytosis (PCP) is a rare benign skin disorder of polyclonal plasma cell proliferation in the skin without underlying systemic diseases that cause secondary infiltration by plasma cells (1). PCP is characterized by solitary or multiple red-brown plaques, mainly involving the trunk, and histologically by the marked hyperplasia of benign mature plasma cells in the dermis (1). The majority of PCP cases occur in adults, but several cases of isolated benign PCP in children have been reported (1,2). PCP should be differentiated from other conditions that result in plasma cell infiltrates in the skin, such as secondary syphilis, primary cutaneous plasmacytoma, cutaneous lymphoid hyperplasia, and nodular amyloidosis (1–3). Herein we present a case of isolated PCP occurring in a child who had good response to topical corticosteroid. A 3-year-old girl had a 1-year history of an asymptomatic skin lesion on the right knee. Physical examination showed a 1-cm 9 3-cm solitary erythematous plaque (Fig. 1). She had no history of trauma, infections, or insect bites. The superficial lymph nodes, liver, and spleen were not palpable. There was no family history of malignancy or autoimmune disease. Her serum protein and immunoelectrophoresis were within normal limits. Serologic analysis for antinuclear antibodies was negative. Histologic examination of a skin biopsy specimen showed irregular acanthosis and dense inflammatory cell infiltrate consisting largely of mature plasma cells in the
DOI: 10.1111/pde.12449
e120
superficial and middermis (Fig. 2A, B). Immunoperoxidase stains for kappa and lambda light chains demonstrated polyclonal populations of plasma cells (Fig. 2C, D). The diagnosis of PCP was made by a combination of clinical and histologic findings without evidence of other systemic disease such as malignant conditions, autoimmune diseases, or infections (1). PCP could be excluded by demonstrating the polyclonality of infiltrating plasma cells. Cutaneous involvement of multiple myeloma was also excluded by the absence of immature forms of plasma cells and no evidence of M proteins in blood and urine (1,2,4). PCP in adults is thought to require close observation because there have been several reports of multiple cutaneous plasma cell infiltrates and extracutaneous manifestations, which lead to a fatal outcome. PCP in children has rarely been reported, and some authors have suggested that PCP in children may not be the same entity as PCP in adult. In addition, PCP in children is known to respond well to topical or systemic corticosteroids (2,4). The pathomechanism of idiopathic plasma cell infiltrates in PCP is unknown, although it has been hypothesized that high serum levels of interleukin-6 induces maturation of B-lymphocytes to plasma cells (5). Treatment options for PCP include systemic, topical, or intralesional injection of corticosteroids; topical tacrolimus; pulsed dye laser; and excision (2,3). In consideration of her age and cosmetic outcome, we initiated treatment with topical corticosteroids (0.3% diflucortolone valerate ointment). After 1 month, the lesion decreased in size and the indurated erythema had mostly disappeared. A topical steroid can be tried before considering more aggressive treatments in children.
© 2014 Wiley Periodicals, Inc.
Brief Report e121
A
B
C
D
Figure 2. (A) Dense lymphoplasma cell infiltration in the superficial and middermis (hematoxylin and eosin, 409). (B) Mature plasma cells without cellular atypia in the dermis (hematoxylin and eosin, 4009). Cells of the infiltrate stained for (C) kappa light chains and (D) lambda light chains.
REFERENCES 1. Arico M, Bongiorno MR. Primary cutaneous plasmacytosis in a child. Is this a new entity? J Eur Acad Dermatol Venereol 2002;16:164–167. 2. Ahn JJ, Yang YS, Shin MK et al. Case of isolated benign primary cutaneous plasmacytosis in a child. J Dermatol 2011;38:364–367. 3. Gilliam AC, Mullen RH, Oviedo G et al. Isolated benign primary cutaneous plasmacytosis in children: two illustrative cases. Arch Dermatol 2009;145:299–302. 4. Uhara H, Saida T, Ikegawa S et al. Primary cutaneous plasmacytosis: report of three cases and review of the literature. Dermatology 1994;189:251–255. 5. Yamamoto T, Katayama I, Nishioka K. Increased plasma interleukin-6 in cutaneous plasmacytoma: the effect of intralesional steroid therapy. Br J Dermatol 1997;137:631–636.
Hye Rang On, M.D.* Sang Eun Lee, M.D., Ph.D.* You Chan Kim, M.D., Ph.D.† Soo-Chan Kim, M.D., Ph.D.* *Department of Dermatology, Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea †Department of Dermatology, Ajou University School of Medicine, Seoul, Korea Address correspondence to Soo-Chan Kim, M.D., Department of Dermatology, Gangnam Severance Hospital, Yonsei University College of Medicine, Eonjuro 712, Gangnam-gu, Seoul 135–720, Korea, or e-mail: [email protected].
