Hypopigmented Mycosis Fungoides: A Clinical and Histopathology Analysis in 9 Children

Abstract

Background: Hypopigmented mycosis fungoides (HMF) is an uncommon variant of mycosis fungoides.Aims: To study the clinical and histopathology presentation in children with HMF. Method:We reviewed 9 children diagnosed with HMF. The clinical data were collected and analyzed.Result: Eight boys and 1 girl were included, with a median onset age of 7.4 year old and median age of diagnosis of 10.5 year old. Multiple hypopigmented patches were observed in all patients, and 5 patients exhibited multiple scaly erythema at the center of hypopigmented patches. Histopathology showed atypical lymphocytes with hyperchromatic, irregular, and cerebriform nuclei, infiltrated in the epidermis and dermis. Pautrier's microabscesses was noted in 6 of 9 patients, and papillary dermal fibroplasia was noted in 6 of 9 patients. CD8 predominance was detected in 4 of 6 patients. Four patients were simultaneously subjected to skin biopsy on hypopigmented patches and scaly erythema simultaneously. Compared with hypopigmented specimens, erythema biopsy detected deeper and denser infiltration of atypical lymphoid cells in 3 of 4 patients, higher CD4 + /CD8 + ratio in 4 of 4 patients, more CD5 loss in 2 of 4 patients, and more CD7 loss in 2 of 4 patients. TCR gene monoclonal rearrangement was detected in 2 of 5 patients. Narrowband ultraviolet B phototherapy was applied in 7 patients. One of 7 patients achieved complete response, and 6 of 7 patients achieved partial response. No recurrence was noted with the median follow-up period of 6 months. Conclusion:HMF could occur in young patients, with indolent and benign course. HMF could gradually seem as scaly erythema based on hypopigmented patches. The histopathology indicated a more advanced stage of the scaly erythema lesions than hypopigmented patches.

Basement membrane thickness helps to differentiate hypopigmented mycosis fungoides from clinical and pathological mimickers

“…1,3,10 Our study found that "tagging" lymphocytes is a constant feature in HMF lesions. This goes hand in hand with the findings of Khopkar et al 3 and Chen et al 11 Meanwhile, we found Pautrier's microabscesses in about 60% of cases, which is approximate to that described by Chen et al (67%) 11 and higher than that described by Khopkar et al (40%). 3 Furthermore, we found wiry fibroplasia in all cases in contrast to the 67 and 60% reported by the previously mentioned studies, respectively.…”

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

“…It was documented that hypopigmented MF in pediatrics showed CD8+ T cell predominance with a reduced CD4 : CD8 ratio [ 14 , 15 ]. In our case, immunohistochemical staining showed CD8+ T cell predominance over CD4+ T cells.…”

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

“…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. 12…”

“…[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. 12…”

“…The patient will most commonly present with ill-defined hypopigmented patches or plaques, possible scaly erythema, in areas not typically associated with vitiligo. 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.…”