Ann Dermatol.  2014 Jun;26(3):385-391.

Two Clinically Unusual Cases of Folliculotropic Mycosis Fungoides: One with and the Other without Syringotropism

• Bakar O
• Seckin D
• Demirkesen C
• Baykal C
• Buyukbabani N

Abstract

Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, and it rarely exhibits predilection for hair follicle and eccrine gland infiltration. Here, we present 2 similar cases that display folliculotropism with varying amounts of follicular mucinosis, with and without syringotropism. The features observed in both cases were cystic, comedo-like, acneiform lesions; generalized involvement with loss of body hair; pruritus; and hidradenitis suppurativa-like lesions. Hypohidrosis as well as nail and palmoplantar involvement with lichen planopilaris-like clinical features were unique characteristics of the first case. Despite the well-known aggressive behavior of follicular mycosis fungoides, the presented cases had a subtle, slowly progressive, but persistent, clinical course. Folliculotropic and syringotropic mycosis fungoides are variants of cutaneous T-cell lymphoma. Clinical presentations might be challenging, and multiple, deep biopsy specimens containing adnexal structures are required for this critical diagnosis. Aggressive treatment may not be necessary in cases having an indolent course, especially in those with syringotropism.

Keyword

Adnexal; Eccrine gland; Follicle; Lymphoma; Mycosis fungoides; Neoplasm

MeSH Terms

Biopsy
Diagnosis
Eccrine Glands
Hair
Hair Follicle
Hidradenitis
Hypohidrosis
Lichens
Lymphoma
Lymphoma, T-Cell, Cutaneous
Mucinosis, Follicular
Mycosis Fungoides*
Pruritus

Figure

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  Fig. 1 (A) Acneiform papules, pustules, and comedo-like lesions on the chest of the patient in Case 1. (B) Nodulocystic lesions, acneiform papules, pustules, and comedo-like lesions located on the back of the patient in Case 1. (C) Total alopecia of the scalp and eyebrows, and comedo-like lesions located on the forehead of the patient in Case 1. (D) Lichenoid purple papules on the lateral chest wall of the patient in Case 1. (E) Purple lichenoid macules and patches with hyperpigmented borders on the axillary region with loss of axillary hair of the patient in Case 1. (F) Spiny hyperkeratotic 1 to 3-mm papules and erythematous scales on the soles of the patient in Case 1. (G) Scaly erythematous patches on the palms of the patient in Case 1. (H) Pterygium formation and anonychia on the nails of the patient in Case 1.

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  Fig. 2 (A) Biopsy specimen taken from the scalp of the patient in Case 1. Mycosis fungoides associated with follicular mucinosis; band-like infiltration of atypical lymphocytes in the upper dermis, showing epidermotropism (H&E, ×100). (B) Biopsy specimen taken from the scalp of the patient in Case 1. Atypical lymphocytes within the hair follicle (H&E, ×200). (C) Biopsy specimen taken from the scalp of the patient in Case 1. Accumulation of mucopolysaccharides within the follicular epithelium (Alcian blue staining, ×200).

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  Fig. 3 Biopsy specimens taken from the trunk of the patient in Case 1. (A) Folliculocentric infiltration of atypical cells without follicular mucinosis. Note the enlargement of the follicular infundibulum with keratotic plugging (H&E, ×100). (B) CD4+ lymphocytes within the follicle epithelium (H&E, ×200).

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  Fig. 4 Syringotropism: atypical lymphocytes within and around the eccrine glands observed in the biopsy specimen taken from the palm of the patient in Case 1 (H&E, ×200).

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  Fig. 5 (A) Loss of eyebrows and eyelashes; milia-like and comedo-like cystic lesions on the eyelids and forehead of the patient in Case 2. (B) Comedo-like lesions and alopecia of the vertex with some uninvolved scalp hair of the patient in Case 2. (C) Loss of body hair, and cystic lesions observed on the chest of the patient in Case 2. (D) Hidradenitis suppurativa-like lesions, comedones, and loss of axillary hair of the patient in Case 2.

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  Fig. 6 Biopsy specimens taken from the left arm of the patient in Case 2. (A) Panoramic view, follicular dilatation, clear appearance of follicular epithelium, and dense perifollicular infiltrate (H&E, ×100). (B, C) Close-up view showing dense lymphocytic infiltrate, intraepithelial lymphocytes, and basophilic mucin accumulation (H&E; B: ×200, C: ×400). (D) Same follicle; mucin stains blue with colloidal iron. Plugging is also visible (colloidal iron staining, ×200).

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