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Review
. 2013 Nov-Dec;88(6):954-60.
doi: 10.1590/abd1806-4841.20132336.

Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology

Fabricio Cecanho Furlan  1 , José Antonio Sanches  2
Affiliations
Review

Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology

Fabricio Cecanho Furlan et al. An Bras Dermatol. 2013 Nov-Dec.

Abstract
in English, Portuguese

Several distinct clinical forms of mycosis fungoides have been described. Hypopigmented mycosis fungoides should be regarded as a subtype of mycosis fungoides, insofar as it presents some peculiar characteristics that contrast with the clinical features of the classical form. Most patients with hypopigmented mycosis fungoides are younger than patients typically diagnosed with classical mycosis fungoides. In addition to typical dark-skinned individuals impairment, hypopigmented mycosis fungoides has also been described in Asian patients. The prognosis for hypopigmented mycosis fungoides is much better than for classical mycosis fungoides: hypopigmented mycosis fungoides is diagnosed when there are only patches of affected skin, and lesions usually will not progress beyond terminal stages, although they can persist for many years. Diagnosis should involve clinicopathologic correlation: skin biopsy analysis often reveals intense epidermotropism, characterized by haloed, large, and atypical CD8+ lymphocytes with convoluted nuclei, in contrast to mild to moderate dermal lymphocytic infiltrate. These CD8+ cells, which participate in T helper 1-mediated immune responses, prevent evolution to mycosis fungoides plaques and tumors and could be considered the main cause of the inhibition of melanogenesis. Therefore, hypopigmentation could be considered a marker of good prognosis for mycosis fungoides.

Ultimamente diferentes formas clínicas da micose fungoide têm sido descritas. A micose fungoide hipocromiante pode ser considerada um subtipo da micose fungoide, apresentando algumas características peculiares que contrastam com os achados da forma clássica da micose fungoide. A maioria dos pacientes com micose fungoide hipocromiante são mais jovens que aqueles acometidos pela micose fungoide clássica. Esta variante é descrita principalmente em indivíduos melanodérmicos (afroamericanos e asiáticos). O prognóstico é melhor que o observado para a forma clássica: ao diagnóstico, os pacientes apresentam somente "patches", que tendem a perdurar por longos períodos, sem evolução para estágios mais avançados. O diagnóstico é feito através da correlação clinicopatológica: biópsia da lesão cutânea frequentemente revela intenso epidermotropismo, caracterizado por linfócitos CD8+ atípicos, grandes, com halo e núcleo convoluto, contrastando com o infiltrado dérmico leve a moderado. Estas células CD8+, que participam do perfil de resposta T helper-1, impediriam a evolução da doença para o desenvolvimento de placas infiltradas e tumores, além de determinar a inibição da melanogênese nas lesões hipocrômicas. Portanto, a hipocromia poderia ser considerada um marcador de bom prognóstico na micose fungoide.

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Conflict of interest statement

Conflict of interest: None

Figures

FIGURE 1
FIGURE 1
Usual clinical presentation: typical hypopigmented patches on chest (A), abdomen (B) and right buttock and thigh (C)
FIGURE 3
FIGURE 3
Unusual forms of hypopigmented patches: guttata (A) – buttocks and thighs, and vitiligoid lesions (B) – left thigh
FIGURE 4
FIGURE 4
Histopathologic findings. This skin biopsy section shows parakeratosis (a), lymphocytes at all levels of the epidermis, including the basal cell layer (b) and upper dermis lymphocytic infiltrate (c)
FIGURE 5
FIGURE 5
Immunohistochemical staining (CD3, CD4 and CD8) of a HMF case. Sequence represents CD3 (A), CD4 (B) and CD8 (C) staining. This patient presented a predominance of CD8+ over CD4+ cells in neoplastic epidermotropism)
FIGURE 2
FIGURE 2
Usual clinical presentation: hypopigmented patches on the back (A) and lower limbs (B)
See this image and copyright information in PMC

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