Childhood Hypopigmented Mycosis Fungoides: A Rare Diagnosis

Abstract

Primary cutaneous lymphomas (PCL) are rare in pediatrics. Mycosis fungoides (MF) is the most frequent PCL diagnosed in childhood. There are various clinical variants of MF, including the hypopigmented MF (HMF). We present a 5-year-old boy with an 18-month history of progressive, generalized, nonpruritic hypopigmented lesions with central lacy erythema. He had no improvement with emollients. Skin biopsy showed typical features of HMF. He was treated with topical corticosteroids and tacrolimus and narrow-band ultraviolet B (NBUVB) phototherapy, with good response. HMF may mimic multiple skin disorders. Unusual hypopigmented skin lesions should be biopsied. Though phototherapy is effective, recurrence is common.

Hypopigmented Mycosis Fungoides in Younger Patients: A Mimicker of Common Hypopigmented Inflammatory Rashes

“…5 On immunohistochemical findings, epidermotropism and CD8+ T-cells predominate, and their cytotoxic effect are likely the cause for the hypopigmented patches, which differs from other MF variants. 1,4,5,9,11 Additional studies show similar histopathology of skin biopsy specimens upon staining with the predominance of CD8 infiltrates, as well as CD4 infiltrates, mixed CD4/CD8, CD7 loss, atypical lymphocytes within the epidermis forming Pautrier microabscesses, and areas of decreased epidermal melanin. [3][4][5]11,12 Researchers have suggested patients presenting with a scaly erythema component correlates more with an advanced stage compared to only hypopigmented lesions on histopathology.…”

“…4,5,12,13 Typically, patients are asymptomatic, without burning or pruritus of the affected areas; however, pruritus, increased skin sensitivity, skin atrophy, and some cases of peripheral lymphadenopathy have been reported. 4,5,9 The presenting lesion distribution typically occurs on non-sun-exposed areas of the skin located on the extremities, trunk, below the waistline on buttocks, and spares the face, hands, palms and soles, although facial involvement has been reported. 2,4,5,[7][8][9]11,14 Patients rarely progress beyond stage IB, and remain in patch stage.…”

“…3,5 HMF occurs more commonly in populations with darker skin phototypes. 1,2,4,5,[7][8][9] The presenting hypopigmented achromic lesions closely resemble commonly seen inflammatory rashes with pigmentation loss, however the distribution and subtle uncommon features can provide clues clinically. Treatment is similar to that of vitiligo and atopic dermatitis.…”

Hypopigmented Mycosis Fungoides in an 11-Year-Old Palestinian Boy

“…A case series of 34 juvenile onset mycosis fungoides patients from the United States reported that the majority of the pediatric patients presented with clinical stages IA and IB, just as presented in our case. Presentation at later stages of the disease which manifests with lymph node and visceral organ involvement can occur but is exceedingly rare [ 11 ].…”

Response to “Mycosis fungoides in pediatric population: comprehensive review on epidemiology, clinical presentation, and management”

“…There are various clinical variants of MF, including hypopigmented MF which is a rare variant. 2 It is a form of early patch/plaque-stage MF characterized by the presence of hypopigmented patches and affects relatively younger individuals with dark complexion. 3 The therapeutic approach to childhood MF is similar to the adult cases.…”