Introduction: Folliculotropic mycosis fungoides is a rare and aggressive subtype of mycosis fungoides characterized by small to medium-sized malignant T-cells that typically infiltrate the hair follicle. It represents 4% of primary cutaneous lymphomas and less than 10% of patients with MF. Case Summary: We report 2 cases of 80-year old female, who presented with a 3-year history of an erythematous plaque on the left infraorbital area and forehead, and the other with an 8-month history of multiple erythematous indurated plaques on the left cheek, left eyebrow and malar area. Clinical presentation, histopathology and immunohistochemistry findings revealed a diagnosis of folliculotropic mycosis fungoides. Conclusion Folliculotropic mycosis fungoides has distinct clinical and microscopic features. Evaluation of clinical, pathologic, and immunophenotypic findings are important to identify this rare form of cutaneous T-cell lymphoma.
Mycosis Fungoides ; Lymphoma, T-Cell, Cutaneous ; Mucinosis, Follicular

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

Follicular mucinosis (FM) is an epithelial reaction pattern that is characterized by the accumulation of mucinous material in the epithelial hair follicle sheath and the sebaceous glands. Although various pharmacological agents have been employed in an attempt to treat FM, effective therapeutic options have remained elusive. We experienced a recalcitrant form of primary FM that we successfully treated with indomethacin and low-dose intralesional interferon alpha (IFN alpha), respectively. To the best of our knowledge, the primary type of FM that responded to indomethacin and low-dose IFN alpha, respectively, in a single case has not been reported in the English medical literature.
Hair Follicle ; Indomethacin ; Interferon-alpha ; Interferons ; Mucinosis, Follicular ; Mucins ; Sebaceous Glands

Follicular mucinosis, also known as alopecia mucinosa, is a rare disease characterized by mucinous follicular degeneration and may be accompanied by cutaneous lymphoma, particularly mycosis fungoides. Two categories of disease have been identified in patients with the benign primary form. The first occurs in younger patients with localized lesions and spontaneous regression within 2 years of the onset. The second occurs in elderly patients and shows a chronic relapsing, but benign course. In the second type, the patients are older and have widespread and infiltrated lesions that can progress to mycosis fungoides. We report a case of follicular mucinosis in a 5-year-old girl who had widely distributed lesions. We think that long-term follow-up is mandatory for the occurrence of lymphoma-associated disease.
Aged ; Follow-Up Studies ; Humans ; Lymphoma ; Mucinosis, Follicular ; Mucins ; Mycosis Fungoides ; Preschool Child ; Rare Diseases

Eosinophilic pustular folliculitis (Ofuji's disease) is characterized by inflammatory infiltration of the pilosebaceous units by numerous eosinophils. Clinically, recurrent crops of papules and plaques studded with sterile follicular pustules can be seen. Follicular mucinosis is a reaction pattern which occurs in the follicular epithelium and is characterized by a mucinous degeneration of the outer sheath of follicles and sebaceous glands. The condition can occur in various diseases. Herein, we report a case of a 46-year old woman who presented with a recurrent episode of papules and follicular pustules, revealing inflammatory infiltration in and around the pilosebaceous units and prominent eosinophils, in addition to follicular mucinosis.
Eosinophils* ; Epithelium ; Female ; Folliculitis* ; Humans ; Middle Aged ; Mucinosis, Follicular* ; Mucins ; Sebaceous Glands

No abstract available.
Lichens* ; Mucinosis, Follicular*

We report a case of alopecia mucinosa in a 9-year-old girl. She presented with several papules on an erythematous patch on the right side of the ala nasi, a hairless nodular lesion on the same side of the nasal canal. Histopathologic findings obtained from the nasal canal revealed typical features of alopecia mucinosa, such as reticular degeneration, cystic space formation and mucin deposition in the pilosebaceous follicle. The case displayed no evidence or clinical features of any malignant changes, and we suspect the case to be an acute form of alopecia mucinosa.
Alopecia* ; Child* ; Female ; Humans ; Mucinosis, Follicular* ; Mucins

We report a case of follicular mucinosis in a 12-year-old girl. She had an erythematous indurated plaque with an ill-defined border on the chin. A biopsy specimen showed reticular degeneration, cystic space formation, and abundant mucin deposition in pilosebaceous follicles. She had been treated with topical corticosteroid for 3 months. The skin lesion was completely resolved.
Biopsy ; Child ; Chin ; Female ; Humans ; Mucinosis, Follicular* ; Mucins ; Skin

We presented here a case of a 53-year-old male patient who was confirmed as follicular mucinosis associated with mycosis fungoides. He showed erythematous infiltrative papules and plaques on the face, scalp, trunk and extremities. Some of the lesions were observed as mycosis fungoides. A biopsy specimen showed both findings of follicular mucinosis and mycosis fungoides, such as reticular degeneration of follicles, deposit of mucin, Pautrier's microabscess, epidermotropism, rather dense perifollicular or perivascular infiltrates, and the presence of significant proportions of admixed eosinophils and plasma cells. To our best knowledge, this is the first reported case of follicular mucinosis associated with mycosis fungoides in Korea.
Biopsy ; Eosinophils ; Extremities ; Humans ; Korea ; Male ; Middle Aged ; Mucinosis, Follicular* ; Mucins ; Mycosis Fungoides* ; Plasma Cells ; Scalp

Cutaneous focal mucinosis is a rare disease which usually manifests itself as a single asymptomatic papule or nodule, but vesicular presentati:on has only rarely been reported. This condition is one of many mucinoses, including localized lichen myxedematosus, acral persistent papular mucinosis, alopecia mucinosa, follieular mucinosis, and myxoid cysts which present with localized cutaneous lesions. We report a rare vesicular form of cutaneous focal mucinosis in a 42-year-old caucasian male who showed a slowly growing, 0.5 cm-sized, asymptomatic vesicle of one-week duration on his right knee. A histopathological examination revealed a localized accumulation of amorphous basophilic material which was positive on colloidal iron staining at pH 2.5. However, it was negative at pH 0.4 in an alcian blue stain, and negative on colloidal iron staining after treatment with hyaluronidase. The amorphous material was judged to be hyaluronic acid from the results of the above special stains. Proliferation of fibroblasts was also observed. Electron microscopy showed well-develuped endoplasmic reticulum and secretory vesicles in the fibroblasts. The lesion could not be completely removed even after two succesive wide excisions.
Adult ; Alcian Blue ; Basophils ; Colloids ; Coloring Agents ; Endoplasmic Reticulum ; Fibroblasts ; Ganglion Cysts ; Humans ; Hyaluronic Acid ; Hyaluronoglucosaminidase ; Hydrogen-Ion Concentration ; Iron ; Knee ; Male ; Microscopy, Electron ; Mucinoses* ; Mucinosis, Follicular ; Rare Diseases ; Scleromyxedema ; Secretory Vesicles