Mycosis fungoides with follicular mucinosis follicular pilotropic mycosis fungoides

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Some patients with mycosis fungoides present with follicular papules and plaques characterized histopathologically by abundant deposits of mucin within hair follicles that are surrounded by a more or less dense infiltrate of T lymphocytes (Figs 2.43 & 2.44) [77]. The hair follicles are infiltrated by the lymphocytes (pilotropism). The epidermis between affected follicles may be spared or involved by the disease ('epidermal mucinosis') (Fig. 2.45). Alopecia resulting from destruction of the follicles is common (alopecia mucinosa), either generalized or localized (Fig. 2.46). Itching is severe and represents a major problem in this variant of mycosis fungoides, and may be non-responsive to standard treatments.

A variant of mycosis fungoides with marked involvement of the hair follicles but without deposition of mucin has also been described (pilotropic mycosis fungoides) (Fig. 2.47); its relationship with follicular mucinosis-associated mycosis fungoides is unclear, but it seems that pilotropic mycosis fungoides represents a distinct clinicopathological variant of the disease [78].

In patients with marked involvement of the hair follicles, with or without deposition of mucin, a localized eruption of small infundibular cysts and comedones infiltrated by neo-plastic T lymphocytes can be observed (mycosis fungoides with eruptive cysts and comedones) (Figs 2.48 & 2.49). The clinical picture is similar to that observed in 'milia en plaques'.

We believe that 'idiopathic generalized follicular mucinosis' represents a variant of mycosis fungoides with marked

Fig. 2.43 Mycosis fungoides with follicular mucinosis. Follicular erythematous papules and plaques on the thigh.

Fig. 2.44 Mycosis fungoides with follicular mucinosis. Mucin deposits within a hair follicle with destruction of the follicle. Note lymphocytes infiltrating the hair follicle ('pilotropism').
  1. 2.45 Mycosis fungoides with follicular mucinosis. Mucin deposits within two hair follicles as well as within the interfollicular epidermis ('epidermal mucinosis'). Note also patchy lichenoid infiltrate of lymphocytes with epidermotropism.
  2. 2.43 Mycosis fungoides with follicular mucinosis. Follicular erythematous papules and plaques on the thigh.
  3. 2.45 Mycosis fungoides with follicular mucinosis. Mucin deposits within two hair follicles as well as within the interfollicular epidermis ('epidermal mucinosis'). Note also patchy lichenoid infiltrate of lymphocytes with epidermotropism.

deposition of mucin within hair follicles, and cases of progression to late-stage mycosis fungoides and death have been well documented [77]. We have also observed patients with clear-cut mycosis fungoides-associated follicular muci-nosis who went into clinical remission after conventional

Fig. 2.46 Mycosis fungoides with follicular mucinosis. Note partial loss of hairs within the affected skin ('alopecia mucinosa') and superficial erosions representing scratch artefacts resulting from intense itching.
Mycosis Fungoides Itching
Fig. 2.47 Pilotropic mycosis fungoides. Dense perifollicular infiltrate of lymphocytes without deposits of mucin within the hair follicle.
Fig. 2.49 Mycosis fungoides with eruptive cysts and comedones. Dense infiltrate of lymphocytes involving the wall of the cysts.

treatments, and who subsequently relapsed with skin lesions showing clinical and histopathological features of 'idiopathic' follicular mucinosis, again suggesting that this condition represents a variant of mycosis fungoides. Even 'benign' localized follicular mucinosis may represent a variant of mycosis fungoides, conceptually and biologically similar to localized pagetoid reticulosis and 'unilesional' mycosis fungoides [77].

Some authors report a worse prognosis in mycosis fun-goides associated with follicular mucinosis in comparison to patients with 'common' mycosis fungoides [79].

papules on the left hand simulating clinically the picture of a wart. Fig 2.53 I^Ga^d p^t^d retkijb^ (detail °f Fig. 2.52).

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