Adult multisystem Langerhans cell histiocytosis with cutaneous involvement manifesting as extensive flexural erosions

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.