Cutaneous Langerhans cell histiocytosis presenting with hypopigmented lesions: Report of two cases and review of literature

Abstract

Langerhans cell histiocytosis is a rare group of disorders that results from the abnormal proliferation and accumulation of dendritic-derived cells in various organs of the body, such as the skin and bones. Hypopigmented macules are a rare cutaneous presentation of Langerhans cell histiocytosis that may pose a diagnostic dilemma when no other findings of Langerhans cell histiocytosis are present at the time of examination. We present 2 cases of the hypopigmented variant of Langerhans cell histiocytosis, including a case with histopathologic features of regression, and a review of the literature. These cases highlight the importance of including Langerhans cell histiocytosis in the differential diagnosis of an infant with hypopigmented macules and papules.

Hand and feet swelling with indurated hypopigmented patches in the diaper area: Clue to diagnose Langerhans cell histiocytosis

“…2,4,5 Erosive or granulomatous mucosal lesions, particularly on oral and genital sites are occasionally reported. 3,4,6 Recent articles showed that multiple discrete hypopigmented macules and/or papules located on the scalp, trunk, back, skin folds or in the seborrheic or diaper area either with or without co-existing characteristic cutaneous eruptions of LCH, are the rare manifestation of cutaneous LCH. 4,6 However, seborrheic dermatitis, postinflammatory hypopigmentation, clear cell papulosis, granuloma gluteale infantum, severe diaper irritant contact dermatitis and hypopigmented macules in tuberous sclerosis complex should be in the differential diagnosis if lesions are present in diaper area.…”

“…Histologically, the haematoxylin and eosin staining section demonstrated dermal aggregates of Langerhans cells, eosinophilic cytoplasmic histiocytes with characteristic chromatin dense kidney-shaped nuclei. 2,3 Immunohistochemically, positive staining for S100, CD1a and Langerin (CD207) are required to confirm the diagnosis, 2,3,6 especially the latter two as the most specific marker of LCH. 1 In addition, the pathognomonic birbeck granules were demonstrated under electron microscopic view.…”

“…1,2,5 A combination of systemic corticosteroids and immunosuppressants is initially indicated for widespread cutaneous and multisystem diseases. 1,2,5,6 Thus, our patient was classified into the multisystem group with a presence of extensive bone involvement and a few cutaneous lesions. Oral prednisolone with intravenous vinblastine was initially administered.…”