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Multiple Plaques on the Hands and Feet

Multiple Plaques on the Hands and Feet Diagnosis: Mycosis fungoides (MF) palmaris et plantaris (MFPP). Microscopic findings and clinical course The biopsy specimen from the hand showed a superficial and deep, perivascular and periadnexal infiltrate of mononuclear cells and focal dermal fibrosis. Many of the mononuclear cells displayed large nucleoli with cerebriform contours. Immunohistochemical stains showed that most of the cells expressed CD3, with a predominance of CD4 over CD8 cells. These findings supported the diagnosis of MF. Based on the number of large cells (more than 25% of the infiltrate), the patient was considered to have large-cell transformation. The swollen left toe, which was found to be superinfected with enterococci and flavobacteria, was treated successfully with 1 course of moxifloxacin. Over the next 2 years, the patient underwent chemotherapy (3 rounds of gemcitabine followed by 3 rounds of doxorubicin) and then topical nitrogen mustard therapy, which he is still receiving. He has also received bexarotene for the last 7 months. At his last follow-up, the total body surface area of involvement had decreased from 11% (plaque/tumor stage) to 3% (patch stage). No new tumors have formed to date. Discussion Mycosis fungoides, the most common cutaneous T-cell lymphoma, is characterized by clonal expansions of CD4+ (T-helper) T cells within the skin that are both epidermotropic and clonal. Clinically, MF is pleomorphic in its presentation, with lymphomatoid papules, patches, plaques, tumors, or exfoliative erythroderma that may occur individually or together in the same patient. The disease can be limited to a single lesion, or it can be extensive and involve the entire body surface as well as blood and internal viscera. Mycosis fungoides localized primarily to the palms and soles, known as MFPP, is a rare variant and has been described infrequently. In one study of 722 patients with MF, involvement of the palms and/or soles occurred at some point in the course of the disease in 11.5% of patients and was the primary or only site in 0.6% of patients.1 Most MFPP occurs in middle-aged patients (mean age, 55 years). Clinical manifestations of MFPP include patches, hyperkeratotic plaques, verrucous nodules that may ulcerate and become infected, vesicular dermatitis, pustular dermatosis, and nail dystrophy.1-5 In fact, MFPP may clinically mimic many common palmoplantar dermatoses, such as hand eczema, dyshidrotic eczema, contact dermatitis, dermatophyte infection, hypertrophic lichen planus, granuloma annulare, verruca, and keratoderma.1,4 The clinical course and treatment of MFPP remains favorable. Although low numbers (<5%) of Sézary cells have been shown to occur, no extracutaneous disease has been reported.6 Treatment of MFPP includes topical nitrogen mustard, electron beam therapy, radiation therapy, psoralen–UV-A, topical corticosteroids, local excision, carbon dioxide laser, and systemic chemotherapy.1,5 Many patients experience at least a partial response, and a majority enjoy extended periods of clinical remission. An additional important consideration is infection control. Patients with MF have an increased susceptibility to infections owing to disruptions in the integument and dysfunctional immune systems.7 The lesions of MF are sometimes accompanied by ulceration, necrosis, and invasion of pathogenic bacteria, which may lead to sepsis. In our clinical experience, infections must be controlled before other therapies can work. References 1. Resnik KSKantor GRLessin SR et al. Mycosis fungoides palmaris et plantaris. Arch Dermatol 1995;1311052- 1056PubMedGoogle ScholarCrossref 2. Toritsugi MSatoh THiguchi TYokozeki HNishioka K A vesiculopustular variant of mycosis fungoides palmaris et plantaris masquerading as palmoplantar pustulosis with nail involvement. J Am Acad Dermatol 2004;51139- 141PubMedGoogle ScholarCrossref 3. Moreno JCOrtega MConejo-Mir JSSanchez-Pedreno P Palmoplantar pustulosis as a manifestation of cutaneous T-cell lymphoma (mycosis fungoides). J Am Acad Dermatol 1990;23758- 759PubMedGoogle ScholarCrossref 4. Sandwich JTDavis LS Mycosis fungoides palmaris et plantaris. Arch Dermatol 1996;132971PubMedGoogle ScholarCrossref 5. Goldberg DJStampien TMSchwartz RA Mycosis fungoides palmaris et plantaris: successful treatment with the carbon dioxide laser. Br J Dermatol 1997;136617- 619PubMedGoogle ScholarCrossref 6. Stasko TVander Ploeg DEDe Villez RL Hyperkeratotic mycosis fungoides restricted to the palms. J Am Acad Dermatol 1982;7792- 796PubMedGoogle ScholarCrossref 7. Tsambiras PEPatel SGreene JNSandin RLVincent AL Infectious complications of cutaneous t-cell lymphoma. Cancer Control 2001;8185- 188PubMedGoogle Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Multiple Plaques on the Hands and Feet

Archives of Dermatology , Volume 143 (1) – Jan 1, 2007

Multiple Plaques on the Hands and Feet

Abstract

Diagnosis: Mycosis fungoides (MF) palmaris et plantaris (MFPP). Microscopic findings and clinical course The biopsy specimen from the hand showed a superficial and deep, perivascular and periadnexal infiltrate of mononuclear cells and focal dermal fibrosis. Many of the mononuclear cells displayed large nucleoli with cerebriform contours. Immunohistochemical stains showed that most of the cells expressed CD3, with a predominance of CD4 over CD8 cells. These findings supported the diagnosis of...
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Publisher
American Medical Association
Copyright
Copyright © 2007 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.143.1.109-e
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Mycosis fungoides (MF) palmaris et plantaris (MFPP). Microscopic findings and clinical course The biopsy specimen from the hand showed a superficial and deep, perivascular and periadnexal infiltrate of mononuclear cells and focal dermal fibrosis. Many of the mononuclear cells displayed large nucleoli with cerebriform contours. Immunohistochemical stains showed that most of the cells expressed CD3, with a predominance of CD4 over CD8 cells. These findings supported the diagnosis of MF. Based on the number of large cells (more than 25% of the infiltrate), the patient was considered to have large-cell transformation. The swollen left toe, which was found to be superinfected with enterococci and flavobacteria, was treated successfully with 1 course of moxifloxacin. Over the next 2 years, the patient underwent chemotherapy (3 rounds of gemcitabine followed by 3 rounds of doxorubicin) and then topical nitrogen mustard therapy, which he is still receiving. He has also received bexarotene for the last 7 months. At his last follow-up, the total body surface area of involvement had decreased from 11% (plaque/tumor stage) to 3% (patch stage). No new tumors have formed to date. Discussion Mycosis fungoides, the most common cutaneous T-cell lymphoma, is characterized by clonal expansions of CD4+ (T-helper) T cells within the skin that are both epidermotropic and clonal. Clinically, MF is pleomorphic in its presentation, with lymphomatoid papules, patches, plaques, tumors, or exfoliative erythroderma that may occur individually or together in the same patient. The disease can be limited to a single lesion, or it can be extensive and involve the entire body surface as well as blood and internal viscera. Mycosis fungoides localized primarily to the palms and soles, known as MFPP, is a rare variant and has been described infrequently. In one study of 722 patients with MF, involvement of the palms and/or soles occurred at some point in the course of the disease in 11.5% of patients and was the primary or only site in 0.6% of patients.1 Most MFPP occurs in middle-aged patients (mean age, 55 years). Clinical manifestations of MFPP include patches, hyperkeratotic plaques, verrucous nodules that may ulcerate and become infected, vesicular dermatitis, pustular dermatosis, and nail dystrophy.1-5 In fact, MFPP may clinically mimic many common palmoplantar dermatoses, such as hand eczema, dyshidrotic eczema, contact dermatitis, dermatophyte infection, hypertrophic lichen planus, granuloma annulare, verruca, and keratoderma.1,4 The clinical course and treatment of MFPP remains favorable. Although low numbers (<5%) of Sézary cells have been shown to occur, no extracutaneous disease has been reported.6 Treatment of MFPP includes topical nitrogen mustard, electron beam therapy, radiation therapy, psoralen–UV-A, topical corticosteroids, local excision, carbon dioxide laser, and systemic chemotherapy.1,5 Many patients experience at least a partial response, and a majority enjoy extended periods of clinical remission. An additional important consideration is infection control. Patients with MF have an increased susceptibility to infections owing to disruptions in the integument and dysfunctional immune systems.7 The lesions of MF are sometimes accompanied by ulceration, necrosis, and invasion of pathogenic bacteria, which may lead to sepsis. In our clinical experience, infections must be controlled before other therapies can work. References 1. Resnik KSKantor GRLessin SR et al. Mycosis fungoides palmaris et plantaris. Arch Dermatol 1995;1311052- 1056PubMedGoogle ScholarCrossref 2. Toritsugi MSatoh THiguchi TYokozeki HNishioka K A vesiculopustular variant of mycosis fungoides palmaris et plantaris masquerading as palmoplantar pustulosis with nail involvement. J Am Acad Dermatol 2004;51139- 141PubMedGoogle ScholarCrossref 3. Moreno JCOrtega MConejo-Mir JSSanchez-Pedreno P Palmoplantar pustulosis as a manifestation of cutaneous T-cell lymphoma (mycosis fungoides). J Am Acad Dermatol 1990;23758- 759PubMedGoogle ScholarCrossref 4. Sandwich JTDavis LS Mycosis fungoides palmaris et plantaris. Arch Dermatol 1996;132971PubMedGoogle ScholarCrossref 5. Goldberg DJStampien TMSchwartz RA Mycosis fungoides palmaris et plantaris: successful treatment with the carbon dioxide laser. Br J Dermatol 1997;136617- 619PubMedGoogle ScholarCrossref 6. Stasko TVander Ploeg DEDe Villez RL Hyperkeratotic mycosis fungoides restricted to the palms. J Am Acad Dermatol 1982;7792- 796PubMedGoogle ScholarCrossref 7. Tsambiras PEPatel SGreene JNSandin RLVincent AL Infectious complications of cutaneous t-cell lymphoma. Cancer Control 2001;8185- 188PubMedGoogle Scholar

Journal

Archives of DermatologyAmerican Medical Association

Published: Jan 1, 2007

References