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Case Reports: Rare disease
Adult multisystem Langerhans cell histiocytosis with cutaneous involvement manifesting as extensive flexural erosions
  1. Lachlan Dat Wah Lau1,
  2. Mi Vu1,
  3. Alex Nirenberg2 and
  4. Laura Scardamaglia1,3
  1. 1Department of Dermatology, Western Health, Footscray, Victoria, Australia
  2. 2Department of Pathology, Western Health, Footscray, Victoria, Australia
  3. 3Department of Medicine, Faculty of Medicine Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia
  1. Correspondence to Dr Lachlan Dat Wah Lau; lachlanlau2118{at}gmail.com

Abstract

Langerhans cell histiocytosis is a great imitator of other diseases with an often-delayed diagnosis leading to a concerning delay in commencing treatment. We present the case of a male who was previously diagnosed with Hailey-Hailey disease, in whom several treatment options had failed, was referred to the dermatology team for evaluation of a 3-month atypical, extensive, painful and pruritic cutaneous flexural eruption. On systems review, he reported a 2-year history of polyuria and polydipsia. Repeat skin biopsy revealed a prominent histiocytic infiltrate on histopathology with corresponding positive expression of Langerin (CD207), S100, CyclinD1 and p-ERK on immunohistochemistry staining. An MRI of the brain demonstrated posterior pituitary enhancement. The clinical presentation, biopsy and investigations confirmed a diagnosis of a multisystem Langerhans cell histiocytosis, which resulted in longstanding patient morbidity. With considerable multidisciplinary teamwork, a gradual and sustained resolution of his lesions, pain, polyuria and polydipsia was achieved.

  • dermatology
  • pituitary disorders

https://doi.org/10.1136/bcr-2022-254205

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    Footnotes

    • Contributors Responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms and critical revision for important intellectual content: LDWL, MV, AN and LS. Gave final approval of the manuscript: MV, AN and LS.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

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