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‘Bizarre’ rash: adult-onset cutaneous Langerhans cell histiocytosis
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  1. Andre Boo Shern Khoo1,
  2. Jennifer Garioch2
  1. 1Salford Royal NHS Foundation Trust, Salford, Salford, UK
  2. 2Department of Dermatology, Norfolk and Norwich University Hospital, Norwich, UK
  1. Correspondence to Dr Andre Boo Shern Khoo, andre.khoo{at}cantab.net

https://doi.org/10.1136/bcr-2016-217904

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    Description

    A woman aged 80 years was admitted under the general physicians with a 2-week history of general malaise. She was referred to dermatology for an opinion on a rash which had been present for 6 months. Examination revealed an erythematous, keratotic and papular rash below her breasts (figure 1), in both axillae (figure 2), upper abdomen and lower back. The rash was asymptomatic. The patient had no previous history of skin disease. A 4 mm punch biopsy showed discrete collections of monocytoid and histiocytoid cells within the epidermis expressing S100, CD1a and focal variable Leucocyte Common Antigen (LCA)—features diagnostic of Langerhans cell histiocytosis (LCH). MRI of the head and CT imaging of her chest, abdomen and pelvis did not reveal any other focus of disease, and there were no lytic bone lesions.

    Figure 1
    Figure 1

    Cutaneous Langerhans cell histiocytosis on the chest.

    Figure 2
    Figure 2

    Cutaneous Langerhans cell histiocytosis rash in the axilla.

    LCH is a rare disease involving clonal proliferation of dendritic cells. The incidence of LCH in adults is ∼1–2 cases per million with a mean age of onset of 33 years.1 Cutaneous LCH can mimic common dermatoses, and can be easily misdiagnosed. The typical lesions described are papular, rose yellow in colour and often scaly. When LCH presents in the intertriginous areas, it can be mistaken for eczema, psoriasis or intertrigo.2

    Learning points

    • Langerhans cell histiocytosis (LCH) should be considered a differential diagnosis for common dermatoses if they fail to respond to conventional therapy.

    • Recognition of cutaneous LCH is key as the skin may be the earliest sign of disease, and multiorgan involvement can have significant systemic effects.

    References

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      1. Stockschlaeder M,
      2. Sucker C
      . Adult Langerhans cell histiocytosis. Eur J Haematol 2006;76:363–8. doi:10.1111/j.1600-0609.2006.00648.x
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    2. ↵
      1. Girschikofsky M,
      2. Arico M,
      3. Castillo D, et al
      . Management of adult patients with Langerhans cell histiocytosis: recommendations from an expert panel on behalf of Euro-Histio-Net. Orphanet J Rare Dis 2013;8:72. doi:10.1186/1750-1172-8-72
      OpenUrlCrossRefPubMed
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    Footnotes

    • Contributors ABSK was involved in the care and management of the patient and wrote up the case. JG was the consultant in charge of the care of the patient and reviewed the writeup prior to publication.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.