Unveiling Langerhans cell histiocytosis presenting as hidradenitis suppurativa: A case report and systematic review

Abstract

Introduction and importance: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of abnormal Langerhans cells, often presenting with symptoms that mimic common dermatological conditions such as hidradenitis suppurativa (HS). Accurate diagnosis is essential because LCH can affect multiple organ systems and necessitates distinct therapeutic approaches.

Case presentation: We report a rare case of a 39-year-old male with a 7-year history of diabetes insipidus (DI), who presented with polyuria, polydipsia, and enlarging purulent lesions in the axilla and groin. MRI revealed a pituitary lesion, and subsequent histopathological examination confirmed LCH. The patient underwent surgical interventions to address recurring cerebrospinal fluid (CSF) leaks and manage the underlying LCH.

Clinical discussion: Histopathology confirmed LCH with positive markers for CD1a and S-100 proteins. Post-surgery, the patient's symptoms, including polyuria and polydipsia, resolved without complications. There was no recurrence of CSF leakage or other LCH-related symptoms during follow-up. This case illustrates the diagnostic challenges of LCH when it mimics common conditions such as HS and underscores the importance of a multidisciplinary approach, particularly when standard treatments are ineffective.

Conclusion: Surgical intervention was pivotal in the resolution of symptoms, highlighting the necessity for timely and accurate diagnosis to improve outcomes in multifocal LCH cases.

Keywords: Langerhans-cell histiocytosis; Neurosurgery; Pituitary lesions; Rare dermatological disorders; Suppurative hidradenitis.

Fig. 1

A. Pre-operative clinical photograph showing purulent lesions and inflammatory skin changes on the patient's scalp. B. Pre-operative clinical photograph showing inflammatory skin lesions and nodules in the patient's lumbar region. C. Pre-operative clinical photograph of purulent lesions and skin changes in the patient's groin region.

Fig. 2

A. Pre-operative transverse MRI image showing an 18x17x12 mm lesion in the hypothalamic region and proximal pituitary stalk, with mild surrounding tissue swelling and displacement of adjacent structures. B. Pre-operative coronal MRI image illustrating the lesion involving the hypothalamic-pituitary region with associated mass effect on nearby structures.

Fig. 3

Pre-operative axial CT scan revealing a hypodense lesion in the hypothalamic region and proximal pituitary stalk, with no significant involvement of surrounding bone structures.

Fig. 4

A. Langerhans cells admixed with some lymphocyte and eosinophils, ×400, H&E stained. B. Langerhans cells with horse shoe and bilobed nuclei (arrows)and scattered lymphocytes and eosinophils, ×1000, H&E stain. C. CD1a positive Langerhans cells, ×400.

Fig. 5

Post-operative coronal MRI image showing resolution of the lesion in the hypothalamic-pituitary region after surgical intervention, with no visible residual mass or significant postoperative complications.

Fig. 6

Post-operative axial CT scan confirming the absence of any remaining mass in the hypothalamic region.

Fig. 7

PRISMA flowchart of the study selection process.