Cutaneous Hematolymphoid and Histiocytic Proliferations in Children

Abstract

This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Because lymphoblastic leukemias and lymphomas are more frequent in the pediatric setting, cutaneous leukemic infiltrates are relatively common in this age-group. Similarly, histiocytic disorders are more common in children, particularly Langerhans cell histiocytosis and juvenile xanthogranuloma. Notably, the histiocytic disorders have undergone significant modifications on their nomenclature in the basis of the molecular characteristics that are present in them. A summary of the most frequent cutaneous hematopoietic disorders in children will be discussed further in this review.

The different faces of mycosis fungoides: results of a single‐center study

“…In the juvenile setting, MF tends to present under a distinct profile compared to its adult counterpart . The overexpression of atypical features, such as hypopigmented and folliculotropic lesions, as well as the absence of gender predilection (male to female ratio 1:1) are hallmark characteristics of this age group . In our cohort, only 23 patients (3.3%) were younger than 20 years at the time of MF onset, in line with relative published rates (0.5%‐7%).…”

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

“…1,2 Its clinical varieties range from benign cutaneous-limited diseases to life-threatening multiorgan failure. 2,3 This condition has recently been categorized into single and multisystem involvement. 2,4 A 1-month-female term infant was presented with a 3week-history of swelling at dorsal parts of both hands and feet along with a few well-defined hypopigmented indurated patches on her labia majora.…”

“…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 In addition, the pathognomonic birbeck granules were demonstrated under electron microscopic view. 3,4 Apart from the cutaneous manifestation, bone, endocrinological functions (particularly on hypothalamicepituitary axis and thyroid gland), central nervous system, lungs, liver, lymphatic systems, and bone marrow must be closely examined during disease prognosis. 4 In skin-limited diseases, a few cutaneous lesions can be treated with topical corticosteroids or closely observed without any treatments due to a possibility of spontaneous regression.…”