Background: An unusual and not yet reported clinical presentation of pure cutaneous Langerhans cell histiocytosis (LCH) that was a diagnostic dilemma.
Objective: To bring to light the newer presentation of this disease.
Method: The case was managed on clinical grounds initially as cellulitis. However, after the investigations and confirmation of the diagnosis, she was managed symptomatically.
Results: Pure cutaneous LCH may manifest in early adulthood with features of cellulitis, erythematous edematous vesicular plaques, and nodules distributed in the flexural acral areas.
Conclusion: Pure cutaneous LCH may have an acute late onset with unusual morphology as cellulitis, inverse acral distribution, and self-healing.