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Year : 2017  |  Volume : 18  |  Issue : 1  |  Page : 69-71

Papular urticaria or Langerhan's cell histiocytosis: Look and think before labeling!

1 Department of Dermatology, Cutis Academy of Cutaneous Sciences, Bengaluru, Karnataka, India
2 Nirmal Skin and Hair Clinic, Bengaluru, Karnataka, India

Date of Web Publication12-Dec-2016

Correspondence Address:
Samipa Samir Mukherjee
Department of Dermatology, Cutis Academy of Cutaneous Sciences, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.179490

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How to cite this article:
Mukherjee SS, Chandrashekar B S, Raj R P, Nischal K C. Papular urticaria or Langerhan's cell histiocytosis: Look and think before labeling!. Indian J Paediatr Dermatol 2017;18:69-71

How to cite this URL:
Mukherjee SS, Chandrashekar B S, Raj R P, Nischal K C. Papular urticaria or Langerhan's cell histiocytosis: Look and think before labeling!. Indian J Paediatr Dermatol [serial online] 2017 [cited 2021 May 6];18:69-71. Available from: https://www.ijpd.in/text.asp?2017/18/1/69/179490


Atopic dermatitis is a chronic inflammatory inherited pruritic condition of the skin marked by recurrent flares and remissions. It is clinically characterized by xerosis, inflammation, scaling, erythema, sometimes oozing, and lichenification. Although the exact pathophysiology of atopic dermatitis is unknown an impairment in the barrier function of the skin, the role of autoallergens, dendritic cells, T-cells and Staphylococcus aureus have been implicated. We herein report a case of insect bite reaction in an atopic child who was misdiagnosed as a case of Langerhans cell (LC) histiocytosis based on histopathology and also emphasize the fact that LC hyperplasia in the epidermis is a phenomenon in atopic dermatitis.

A 9-month-old male child with a history of atopy presented with itchy erythematous lesions, discrete to grouped at places, scattered over the body predominantly on the exposed areas. The child was treated with topical steroids, emollients, and oral antihistamines and followed up after 10 days. The lesions worsened and increased in extent with newer lesions developing over the face and arms. Clinical examination revealed erythematous papulovesicles and tumid plaques scattered over the arms, legs, trunk, and face [Figure 1],[Figure 2],[Figure 3]. Few lesions showed superficial erosions with serous exudate and crusting; a biopsy was considered from the papulovesicles as the lesions did not show any sign of improvement with topical steroids.
Figure 1: Tumid plaque with papulovesicles on arm

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Figure 2: Oval plaques with papulovesicles on the trunk

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Figure 3: Dull red plaques with papules on the right lower limb

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Histopathology revealed superficial and deep perivascular lymphohistiocytic infiltrate with mild spongiosis. Some of the cells in the epidermis and upper dermis appeared large with irregular as well as occasional reniform nucleus suggestive of prominent LC [Figure 4]. Mild amount of mucin deposition was noted in the upper dermis. The histopathology was reported to be consistent with LC histiocytosis and immunohistochemistry was requested for the confirmation of the same. CD1a and S100 highlighted the scattered LC in the epidermis whereas it was negative for dermis.
Figure 4: Infiltration of the epithelium with inflammatory cells few with reniform nuclei, ×40

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It was thus concluded that the prominent LCs seen in histopathology were most likely associated with atopic dermatitis, and the absence of a dermal component did not support the diagnosis of LC histiocytosis.

Atopic dermatitis although clinically described in 1935 has an elusive pathophysiology. The atopic trinity is characterized by barrier dysfunction, allergy, and pruritus. The role of dendritic cells has been studied extensively in atopic dermatitis. Wollenberg et al. described that in the lesional skin plasmacytoid dendritic cells are almost absent whereas LCs and inflammatory dendritic epidermal cells (IDECs) are present.[1],[2] LC and IDEC play an important role in the uptake and presentation of allergens to Th1/Th2 cells and possibly also to regulatory T-cells. LC produces interleukin-16 that specifically recruits CD4 T-cells.[3] A proliferation of LCs in the epidermis and upper dermis is a known phenomenon which happens due to the alteration of immunity.

The histologic picture is nonspecific characterized by spongiosis with parakeratosis in the epidermis and upper dermal edema and vascular dilatation. Literature reports that there is an increased number of immunoglobulin E bearing LC in the epidermis.[4],[5] In our case also, there was a proliferation of LC only in the epidermis which was mistaken as a feature of LC histiocytosis. However, as immunohistochemistry did not reveal any dermal component of LC, it was concluded that hyperplasia of LC in the epidermis was due to the underlying atopic dermatitis.

We report this case to highlight the fact that epidermal hyperplasia of LC is known to occur in atopic dermatitis, and, therefore, histopathology showing the same should not be mistaken as a case of LC histiocytosis in the absence of a dermal component of LC which can be confirmed with immunohistochemistry.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Wollenberg A, Wagner M, Günther S, Towarowski A, Tuma E, Moderer M, et al. Plasmacytoid dendritic cells: A new cutaneous dendritic cell subset with distinct role in inflammatory skin diseases. J Invest Dermatol 2002;119:1096-102.  Back to cited text no. 1
Wollenberg A, Kraft S, Hanau D, Bieber T. Immunomorphological and ultrastructural characterization of Langerhans cells and a novel, inflammatory dendritic epidermal cell (IDEC) population in lesional skin of atopic eczema. J Invest Dermatol 1996;106:446-53.  Back to cited text no. 2
Reich K, Heine A, Hugo S, Blaschke V, Middel P, Kaser A, et al. Engagement of the Fc epsilon RI stimulates the production of IL-16 in Langerhans cell-like dendritic cells. J Immunol 2001;167:6321-9.  Back to cited text no. 3
Bruynzeel-Koomen C, van Wichen DF, Toonstra J, Berrens L, Bruynzeel PL. The presence of IgE molecules on epidermal Langerhans cells in patients with atopic dermatitis. Arch Dermatol Res 1986;278:199-205.  Back to cited text no. 4
Barker JN, Alegre VA, MacDonald DM. Surface-bound immunoglobulin E on antigen-presenting cells in cutaneous tissue of atopic dermatitis. J Invest Dermatol 1988;90:117-21.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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