Folliculotropic mycosis fungoides with large-cell transformation presenting as dissecting cellulitis of the scalp

Follicular mycosis fungoides (MF) is a rare variant of cutaneous T-cell lymphoma (CTCL) in which malignant lymphocytes preferentially infiltrate hair follicles. This report describes a patient with follicular mycosis fungoides presenting in a manner similar to dissecting cellulitis of the scalp with nonhealing, draining nodular lesions. Follicular mucinosis associated with folliculotropic mycosis fungoides resulted in follicular disruption and deep dissecting cellulitis. Large-cell transformation of CTCL was present in the initial diagnostic scalp and axillary lymph node specimens. The patient died from progressive CTCL 9 months following initial diagnosis despite electron beam radiation, topical mechlorethamine, interferon-α, and systemic chemotherapy. This case indicates that large-cell transformation of folliculotropic mycosis fungoides is an aggressive form of CTCL, and that folliculotropic mycosis fungoides can give rise to lesions which resemble dissecting cellulitis of the scalp. Upregulation of intercellular adhesion molecule-1 (ICAM-1) on follicular epithelium adjacent to lymphocyte function-associated antigen-1 (LFA-1)-positive folliculotropic lymphoma cells in this report provides insight into lymphocyte homing mechanisms in folliculotropic MF.     

Transient iatrogenic immunodeficiency‐related B‐cell lymphoproliferative disorder of the skin in a patient with mycosis fungoides/Sézary syndrome

Immunodeficiency‐related lymphoproliferative disorders (IR‐LPD) may occur in the setting of immunosuppressive therapy with methotrexate and TNF‐α antagonists. As far as we are aware, this is the first report of an Epstein‐Barr virus‐associated B‐cell lymphoproliferative disorder, secondary to methotrexate therapy in a patient with mycosis fungoides/Sézary syndrome. Curry JL, Prieto VG, Jones DM, Vega F, Duvic M, Diwan AH. Transient iatrogenic immunodeficiency‐related B‐cell lymphoproliferative disorder of the skin in a patient with mycosis fungoides/Sézary syndrome.     

Mycosis fungoides/Sézary syndrome: report of an unusual case

Introduction: Sézary syndrome (SS) is an uncommon form of cutaneous T cell lymphoma (CTCL) with a classical triad of lymphadenopathy, characteristic circulating lymphoma cells (Sézary cells) and erythrodermatous skin involvement with classical mycosis fungoides (MF)‐like histological picture. Case report: A 32‐year‐old woman presented with this classical triad; however, her skin involvement, histologically, was in the form of folliculotropic MF, rather than the usual classical form of MF. Conclusions: In the vast majority of cases, the cutaneous involvement in SS resembles conventional MF, histologically. One case of CD30‐positive CTCL with pilotropic MF has been reported. However, English literature does not describe any case of SS with folliculotropic MF with typical immunophenotype of SS thus far. We presume that this case represents the first report of SS with folliculotropic MF histologically, displaying the typical CD30‐negative immunophenotype.     

Rapidly progressing mycosis fungoides presenting as follicular mucinosis

Follicular mucinosis can occur as a primary idiopathic disorder or can arise in association with benign or malignant disease, most notably mycosis fungoides. We describe a patient with an aggressive folliculotropic variant of mycosis fungoides that initially presented as follicular mucinosis with alopecia. One month after the diagnosis of follicular mucinosis, a diagnosis of mycosis fungoides was made, and 3 months later inguinal lymph node involvement with mycosis fungoides developed. A skin biopsy specimen demonstrated prominent follicular mucinosis with folliculotropism of atypical cells and intrafollicular Pautrier's microabscesses. As demonstrated in this case, follicular mucinosis can be a presenting sign of rapidly progressive mycosis fungoides. In our review of follicular mucinosis and its association with mycosis fungoides, we found that the folliculotropic variant of mycosis fungoides appears more commonly to have an aggressive course than classic mycosis fungoides.     

CD25+ folliculotropic Sézary syndrome with CD30+ large cell transformation

Folliculotropic Sézary syndrome is a rare and unique variant of cutaneous T‐cell lymphoma (CTCL) characterised by both follicular and leukaemic involvement of mycosis fungoides (MF). It is associated with a more aggressive clinical course and fatal outcomes. Large cell transformation (LCT) of mycosis fungoides/Sézary syndrome is also associated with an aggressive disease course and shortened survival, requiring an intensive therapeutic approach. This report describes a case of folliculotropic Sézary syndrome with CD30+ LCT. Most of the larger lymphocytes in the lesions were positive for CD25, the expression of which is associated with advanced CTCL. In addition, we review the literature on this unusual CTCL and provide evidence that this entity represents a distinct clinicopathological entity occasionally associated with extracutaneous involvement and LCT.     

Folliculotropic mycosis fungoides in a pediatric patient mimicking black dot tinea capitis

Immunosuppression following organ transplantation is a known risk factor for the development of lymphoproliferative disorders. Mycosis fungoides, a rare entity in pediatric patients, has seldom been reported as a post‐transplant lymphoproliferative disorder. We report a case of folliculotropic mycosis fungoides in a pediatric patient following liver transplantation that was initially diagnosed as tinea capitis.     

CD30+ T-cell lymphoma in a patient with psoriasis treated with ciclosporin and infliximab

There is a known relationship between the use of immunosuppressive therapies and the development of lymphoproliferative malignancies. These lymphomas are mainly B-cell nonHodgkin's lymphomas associated with Epstein-Barr virus. Most cases concern classical immunosuppressive treatments including ciclosporin and methotrexate. A relationship between the new antitumour necrosis factor (TNF)-alpha agents and lymphoproliferative malignancies is debated. Patients with psoriasis on immunosuppressive therapies, mainly ciclosporin, are considered to have a low risk of developing lymphoid proliferation. We report a patient with erythrodermic psoriasis treated with ciclosporin and infliximab who developed a CD30+ T-cell lymphoma. This lymphoma regressed after stopping these treatments. In this case, the anti-TNF-alpha agent may have played a role in association with ciclosporin in the development of the lymphoproliferative disorder. Whereas the combination of anti-TNF-alpha therapies with methotrexate has been well studied, their combination with ciclosporin has been evaluated only in a few patients. Psoriatic patients who may require anti-TNF-alpha treatment have often been or will be treated with ciclosporin. The combination of ciclosporin and anti-TNF-alpha warrants further investigation.     

Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis

Eosinophilic pustular folliculitis (EPF) is an inflammatory disease characterized by repeated pruritic follicular papules and pustules arranged in arcuate plaques, and folliculotropic infiltration of eosinophils. The diagnosis of EPF is occasionally difficult and problematic because EPF may share the clinical appearance and histological findings with other diseases. Moreover, EPF has several clinical subtypes, including the classical type, infantile type and immunosuppression‐associated type. Because the therapies of EPF are relatively specific as compared to eczematous disorders, accurate diagnosis is essential for the management of EPF. Clinical differential diagnoses include tinea, acne, rosacea, eczematous dermatitis, granuloma faciale, autoimmune annular erythema, infestations and pustular dermatosis. Histologically, cutaneous diseases with eosinophilic infiltrates can be differentially diagnosed. Follicular mucinosis, mycosis fungoides and other cutaneous T‐cell lymphomas are the most important differential diagnoses both clinically and histopathologically. It should be kept in mind particularly that the initial lesions of cutaneous T‐cell lymphoma resemble EPF.     

Mycosis fungoides/Sézary syndrome: report of an unusual case

Introduction:  Sézary syndrome (SS) is an uncommon form of cutaneous T cell lymphoma (CTCL) with a classical triad of lymphadenopathy, characteristic circulating lymphoma cells (Sézary cells) and erythrodermatous skin involvement with classical mycosis fungoides (MF)-like histological picture.
Case report:  A 32-year-old woman presented with this classical triad; however, her skin involvement, histologically, was in the form of folliculotropic MF, rather than the usual classical form of MF.
Conclusions:  In the vast majority of cases, the cutaneous involvement in SS resembles conventional MF, histologically. One case of CD30-positive CTCL with pilotropic MF has been reported. However, English literature does not describe any case of SS with folliculotropic MF with typical immunophenotype of SS thus far. We presume that this case represents the first report of SS with folliculotropic MF histologically, displaying the typical CD30-negative immunophenotype.     

Durable remission of folliculotropic mycosis fungoides achieved with a combined topical treatment with cytarabine and carmustine

Folliculotropic mycosis fungoides (MF) is an uncommon subtype of MF which has a more aggressive natural history and is more resistant to treatment than other forms of MF. In this article, an innovative method of treatment is presented. We describe a successful use of topical cytarabine combined with topical carmustine in a patient with a folliculotropic MF. Based on our patient history we suggest this method as an effective therapy option for patients with folliculotropic MF unresponsive to other forms of treatment.     

Folliculotropic mycosis fungoides driven by DOCK8 immunodeficiency syndrome

DOCK8 immunodeficiency syndrome (DIDS) represents a rare primary immunodeficiency associated with cutaneous viral infections, allergy, and increased risk of malignancy. We report a case of folliculotropic mycosis fungoides with spontaneous resolution occurring in a patient with DIDS.     

Syringotropic cutaneous T-cell lymphoma: an immunophenotypic and genotypic study of five cases

There is uncertainty about the exact nosological relationship between mycosis fungoides, follicular mucinosis, syringolymphoid hyperplasia with alopecia (SLHA) and syringotropic cutaneous T-cell lymphoma (CTCL). We report the clinical, histological, immunophenotypic and genotypic characteristics of a series of five patients (three men and two women) with syringotropic CTCL. We also review the 15 cases of SLHA previously reported in the literature. We conclude that syringotropic CTCL is a distinct clinicopathological variant of mycosis fungoides which may present on its own with characteristic punctate erythema or more commonly in association with folliculotropic lesions. Syringotropic CTCL is characterized histologically by infiltration of sweat glands by atypical lymphocytes in association with syringolymphoid hyperplasia. Cases of SLHA represent a syringotropic form of CTCL in association with follicular involvement, and such cases need to be investigated using T-cell receptor gene analysis of both skin and blood. Only limited conclusions on prognosis can be derived from our preliminary data. However, a review of the literature suggests that the prognosis does not differ significantly from other types of mycosis fungoides of equivalent stage.     

Folliculotropic T-cell lymphocytosis (mucin-poor follicular mucinosis)

A 48-year-old man presented with multiple asymptomatic patches of hair loss over his trunk and limbs associated with focal keratotic follicular plugs. Multiple skin biopsies showed a panfollicular lymphocytic infiltrate associated with follicular hyperkeratinization, minimal follicular spongiosis, focal basaloid follicular hyperplasia but no overt follicular mucinosis. The lymphocytes were small and there was no atypia. Immunoperoxidase stains showed that the follicular lymphocytes were T cells and predominantly CD4 positive with HLADr (LN3) expressed on their surface. There were insufficient clinical or histopathological features to make a diagnosis of folliculotropic T-cell lymphoma. This case currently may be classified best as folliculotropic T-cell lymphocytosis and may represent a mucin-poor counterpart of follicular mucinosis. Such cases may pursue an indolent course or may evolve to folliculotropic T-cell lymphoma, mycosis fungoides or anaplastic lymphoma. The term folliculotropic T-cell lymphocytosis may be useful for similar cases lacking clinical or histological criteria for lymphoma and lacking follicular mucinosis.     

以弥漫性秃发为首发表现的亲毛囊性蕈样肉芽肿一例

亲毛囊性蕈样肉芽肿属于蕈样肉芽肿的一种亚型，侵袭性较强，预后差，早期诊断尤为重要。本文报道一例免疫组化联合TCR基因重排确诊的以弥漫性秃发为首发表现亲毛囊性蕈样肉芽肿一例。     

Treatment of cutaneous T-cell lymphomas with TP-5. Evaluation of the clinical effect in 8 patients

Eight patients with cutaneous T-cell lymphomas (6 patients with mycosis fungoides, 1 patient with Sézary's syndrome, 1 patient with low-grade malignant lymphoma, unclassified) were treated with TP-5, a synthetic pentapeptide having the same biologic activity as the thymic hormone thymopoietin. TP-5 was administered three times weekly at a dosage of 50 mg subcutaneously in 6 patients, 50 mg intravenously in 1 patient, and 100 mg subcutaneously in 1 patient. Clinical evaluation at the end of the trial disclosed improvement in 4 patients (2 patients with mycosis fungoides, 1 patient with Sézary's syndrome, 1 patient with low-grade malignant lymphoma, unclassified), deterioration in 3 patients with mycosis fungoides and no change in 1 patient with mycosis fungoides. As TP-5 evidently exerts some effect on cutaneous T-cell lymphomas, further investigations of its therapeutic potential in this group of diseases seem worthwhile.     

Mycosis fungoides: An important differential diagnosis for acquired palmoplantar keratoderma

Mycosis fungoides is the most common subtype of primary cutaneous lymphoma and has several clinical variants. We report a 74‐year‐old man presenting with an acquired palmoplantar keratoderma initially diagnosed and treated as psoriasis with suboptimal improvement. Several months later the patient developed patches and plaques that were histologically consistent with mycosis fungoides. These lesions were ameliorated with the treatment of the underlying mycosis fungoides and the palmoplantar keratoderma resolved promptly with radiotherapy. This case highlights the importance of considering mycosis fungoides as an infrequent but serious cause of acquired palmoplantar keratoderma.     

ALK‐positive primary cutaneous T‐cell‐lymphoma (CTCL) with unusual clinical presentation and aggressive course

Anaplastic lymphoma kinase (ALK) expression is uncommon in primary cutaneous T‐cell‐lymphomas (CTCL). We report the case of a patient who was initially diagnosed with small plaque parapsoriasis, and eventually developed an unusual manifestation of CTCL 6 years later. The disease was characterized by aggressively ulcerating plaques and tumors of the entire skin. Histopathology revealed monoclonal proliferation of atypical T‐lymphocytes and CD30‐positive blasts with expression of ALK and identification of an ATIC‐ALK fusion protein. Extensive staging confirmed the primary cutaneous origin of the lymphoma. After failure of several conventional treatments including polychemotherapy, the patient finally achieved remission after receiving brentuximab‐vedotin, alemtuzumab and subsequent allogeneic stem cell transplantation. In the following, the patient developed inflammatory cutaneous lesions that pathologically showed no evidence for lymphoma relapse or classical cutaneous graft‐versus‐host disease. The patient responded to immunosuppression, but finally died from multi‐organ failure due to sepsis 8 months after stem cell transplantation. This is a rare instance of ALK positivity in a CTCL, most likely resembling CD30+ transformed mycosis fungoides, because it was not typical for cutaneous anaplastic large cell lymphoma (ALCL). In contrast to its role in systemic ALCL as favorable prognostic marker, ALK expression here was associated with an aggressive course.     

Granulomatous slack skin: a distinct disorder or a variant of mycosis fungoides?

About 75% of cutaneous lymphomas belong to the group of T-cell lymphomas. Mycosis fungoides is the most common entity in this group. Granulomatous slack skin is a rare form of cutaneous T-cell lymphoma closely related to mycosis fungoides. We present here a patient with areas of lax skin for several years who developed a generalized erythroderma with associated immunoactivation and a deterioration in his general condition. This report discusses clinically and histologically the differential diagnoses, namely granulomatous slack skin and granulomatous mycosis fungoides, and suggests that these 2 disorders are only variants in the broad spectrum of a single disease.     

Disseminated CD8‐positive,CD30‐positive cutaneous lymphoproliferative eruption with overlapping features of mycosis fungoides and primary cutaneous anaplastic large cell lymphoma following remote solitary lesional presentation

CD8‐positive, CD30‐positive cutaneous lymphoproliferative disorders constitute a rare subset of T‐cell lymphoproliferative conditions, including variants of primary cutaneous anaplastic large cell lymphoma (ALCL), mycosis fungoides, lymphomatoid papulosis type D, cutaneous gamma‐delta T‐cell lymphoma and cutaneous peripheral T‐cell lymphoma. These entities share overlapping clinical, histopathologic and immunophenotypic features, presenting both a clinical and pathological diagnostic challenge. Presented here is a 73‐year‐old man with a disseminated, indolent CD30+, CD8+ cutaneous lymphoproliferative disorder with overlapping clinical and histopathological features of both mycosis fungoides and primary cutaneous ALCL, as well as features of lymphomatoid papulosis. To our knowledge, this is the first case of a generalized CD8+, CD30+ eruption with features of both mycosis fungoides and primary cutaneous ALCL arising following an episode of solitary primary cutaneous CD8‐positive ALCL.