Indeterminate cell histiocytosis successfully treated with phototherapy

Abstract

First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome.

Indeterminate cell histiocytosis in a Chinese patient with progressive and extensive nodular lesions and mixed indeterminate cell and macrophage‐monocyte lineage

“…This immunoprofile is in keeping with previous reports of ICH . The presence of MNGs has been reported in a few cases of ICH, admixed within the neoplastic histiocytoid cells . However, MNGs with nuclei having a wreath‐like configuration resembling Touton giant cells and the presence of foamy histiocytes as seen in our patient have not previously been described.…”

“…Due to its rarity, there is no established treatment for ICH so far and various treatment modalities have been reported . These include systemic chemotherapy such as intravenous vinblastine, thalidomide and methotrexate, pravastatin, phototherapy including psoralen ultraviolet A, narrow band ultraviolet B,, electron beam therapy, electrodesiccation and topical therapy including topical tar and 5% 5‐fluorouracil cream . Although most patients undergo spontaneous involution, careful follow‐up of all patients with ICH is recommended because visceral involvement and leukemia have been reported to occur.…”

Indeterminate cell histiocytosis: A case treated with ultraviolet B narrow band phototherapy (NB UVB)

“…Moreover, although UVB can only penetrate the superficial skin layers, it is crucial to consider that IC could be a stage in the maturation process of Langerhans cells and UVB treatment is known to reduce the number of epidermal Langerhans cells. 42 In conclusion, our experience with NB UVB further confirms the few data present in the literature 43,44 and suggest that this phototherapy source could represent an effective and reliable treatment option in patients with indolent forms of ICH.…”

Perianal Langerhans cell histiocytosis: a rare presentation in an adult male

“…The definitive diagnosis is confirmed by diffuse positivity for CD1a, S100 protein, and langerin (CD 207). The diffuse positivity for S100 protein and langerin expression 24 excluded the diagnosis of indeterminate cell histiocytosis (ICH), which shares many features with LCH, including CD1a positivity. A lack of Birbeck granules by electron microscopy is another characteristic feature of ICH that discriminates it from LCH.…”