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Acral Atrophic Papules, Petechia, and Patches of Alopecia—Diagnosis

Acral Atrophic Papules, Petechia, and Patches of Alopecia—Diagnosis Diagnosis: Syringotropic mycosis fungoides (MF). Microscopic findings Histopathologic examination of the biopsy specimens revealed a slightly acanthotic epidermis, with reticular fibroplasia of the papillary dermis. Intermediate-size lymphocytes with atypia were seen focally along the dermoepidermal junction. The eccrine coils were densely infiltrated with intermediate-size lymphocytes. Immunohistochemical analysis showed a CD4:CD8 ratio of greater than 10:1, and T-cell receptor gene rearrangement studies showed a positive T-cell clone. The diagnosis of syringotropic MF was made. Discussion Mycosis fungoides has a wide clinopathologic spectrum ranging from the classic patch-plaque MF to rare forms such as syringotropic MF.1 Syringolymphoid hyperplasia with alopecia, now recognized to be a premalignant form of syringotropic MF, was first described by Sarkany2 in 1969. When the cases of syringolymphoid hyperplasia with alopecia and syringotropic MF described in the literature are counted, the present case brings the total to 18 (13 men and 5 women).3 Because so few cases have been reported, it is difficult to characterize the clinical appearance of such lesions. In the literature, the mean age at onset is 47 years. Fifteen patients (83%) had associated alopecia and 5 (30%) had anhidrosis.4 Six had follicular lesions and 6 had punctuate erythema. Only 2 patients had erythematous punctuate lesions as their sole cutaneous manifestation. There is a tendency for an acral location as well as for the development of stellate atrophic and sclerotic papules. This can strongly simulate livedoid vasculopathy but without the characteristic punched-out ulcers. Histologically, the hallmark of the condition is dense infiltration of the eccrine coils with atypical lymphocytes. There may or may not be epidermotropism, as this is not a typical feature of the disease. As in conventional MF, the intraepithelial cells are almost always CD4 positive in syringotropic MF, and there are often abundant accompanying eosinophils. Also, fibrosis, which is a more impressive feature in syringotropic MF, both clinically and histologically, may be related to the presence of a chronic deep infiltrate, whereas in conventional MF the infiltrate is only superficially located. Furthermore, if biopsy specimens are obtained from nonglabrous sites with involvement, in almost all cases there will be follicular involvement as well.5 As in follicular MF, skin-directed therapy is often inadequate in syringotropic MF.3,6 Therapy with psoralen–UV-A or narrowband UV-B alone may not have enough penetration to reach the involved eccrine glands.6 Therefore, combination therapy with new drugs such as cytokines (interferon alfa) and retinoids (Targretin) is typically more effective.7 Superficial localized radiotherapy has also been used with some success. The prognosis in cases of syringotropic MF is likely similar to those of follicular MF, which we have previously shown to be a more aggressive variant of cutaneous T-cell lymphoma. 5 Return to Quiz Case. References 1. Latkowski JHeald P Cutaneous T-cell lymphomas. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz S , eds. Fitzpatrick's Dermatology in General Medicine.6th ed. New York, NY: McGraw-Hill Co; 2003:1537-1557Google Scholar 2. Sarkany I Patchy alopecia, anhidrosis, eccrine gland wall hypertrophy and vasculitis. Proc R Soc Med 1969;62 (2) 157- 159PubMedGoogle Scholar 3. Thein MRavat FOrchard GCalonje ERussell-Jones R Syringotropic cutaneous T-cell lymphoma: an immunophenotypic and genotypic study of five cases. Br J Dermatol 2004;151 (1) 216- 226PubMedGoogle ScholarCrossref 4. Kazakov DVBurg GKempf W Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol 2004;18 (4) 397- 415PubMedGoogle ScholarCrossref 5. Gerami PGuitart J Folliculotropic Sézary syndrome: a new variant of cutaneous T-cell lymphoma. Br J Dermatol 2007;156 (4) 781- 783PubMedGoogle ScholarCrossref 6. Haller AElzubi EPetzelbauer P Localized syringolymphoid hyperplasia with alopecia and anhidrosis. J Am Acad Dermatol 2001;45 (1) 127- 130PubMedGoogle ScholarCrossref 7. Hymes KB Choices in the treatment of cutaneous T-cell lymphoma. Oncology (Williston Park) 2007;21 (2) (suppl 1)18- 23PubMedGoogle Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Acral Atrophic Papules, Petechia, and Patches of Alopecia—Diagnosis

Archives of Dermatology , Volume 145 (1) – Jan 1, 2009
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Acral Atrophic Papules, Petechia, and Patches of Alopecia—Diagnosis

Abstract

Diagnosis: Syringotropic mycosis fungoides (MF). Microscopic findings Histopathologic examination of the biopsy specimens revealed a slightly acanthotic epidermis, with reticular fibroplasia of the papillary dermis. Intermediate-size lymphocytes with atypia were seen focally along the dermoepidermal junction. The eccrine coils were densely infiltrated with intermediate-size lymphocytes. Immunohistochemical analysis showed a CD4:CD8 ratio of greater than 10:1, and T-cell receptor gene...
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References (7)

Publisher
American Medical Association
Copyright
Copyright © 2009 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archdermatol.2008.557-b
Publisher site
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Abstract

Diagnosis: Syringotropic mycosis fungoides (MF). Microscopic findings Histopathologic examination of the biopsy specimens revealed a slightly acanthotic epidermis, with reticular fibroplasia of the papillary dermis. Intermediate-size lymphocytes with atypia were seen focally along the dermoepidermal junction. The eccrine coils were densely infiltrated with intermediate-size lymphocytes. Immunohistochemical analysis showed a CD4:CD8 ratio of greater than 10:1, and T-cell receptor gene rearrangement studies showed a positive T-cell clone. The diagnosis of syringotropic MF was made. Discussion Mycosis fungoides has a wide clinopathologic spectrum ranging from the classic patch-plaque MF to rare forms such as syringotropic MF.1 Syringolymphoid hyperplasia with alopecia, now recognized to be a premalignant form of syringotropic MF, was first described by Sarkany2 in 1969. When the cases of syringolymphoid hyperplasia with alopecia and syringotropic MF described in the literature are counted, the present case brings the total to 18 (13 men and 5 women).3 Because so few cases have been reported, it is difficult to characterize the clinical appearance of such lesions. In the literature, the mean age at onset is 47 years. Fifteen patients (83%) had associated alopecia and 5 (30%) had anhidrosis.4 Six had follicular lesions and 6 had punctuate erythema. Only 2 patients had erythematous punctuate lesions as their sole cutaneous manifestation. There is a tendency for an acral location as well as for the development of stellate atrophic and sclerotic papules. This can strongly simulate livedoid vasculopathy but without the characteristic punched-out ulcers. Histologically, the hallmark of the condition is dense infiltration of the eccrine coils with atypical lymphocytes. There may or may not be epidermotropism, as this is not a typical feature of the disease. As in conventional MF, the intraepithelial cells are almost always CD4 positive in syringotropic MF, and there are often abundant accompanying eosinophils. Also, fibrosis, which is a more impressive feature in syringotropic MF, both clinically and histologically, may be related to the presence of a chronic deep infiltrate, whereas in conventional MF the infiltrate is only superficially located. Furthermore, if biopsy specimens are obtained from nonglabrous sites with involvement, in almost all cases there will be follicular involvement as well.5 As in follicular MF, skin-directed therapy is often inadequate in syringotropic MF.3,6 Therapy with psoralen–UV-A or narrowband UV-B alone may not have enough penetration to reach the involved eccrine glands.6 Therefore, combination therapy with new drugs such as cytokines (interferon alfa) and retinoids (Targretin) is typically more effective.7 Superficial localized radiotherapy has also been used with some success. The prognosis in cases of syringotropic MF is likely similar to those of follicular MF, which we have previously shown to be a more aggressive variant of cutaneous T-cell lymphoma. 5 Return to Quiz Case. References 1. Latkowski JHeald P Cutaneous T-cell lymphomas. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz S , eds. Fitzpatrick's Dermatology in General Medicine.6th ed. New York, NY: McGraw-Hill Co; 2003:1537-1557Google Scholar 2. Sarkany I Patchy alopecia, anhidrosis, eccrine gland wall hypertrophy and vasculitis. Proc R Soc Med 1969;62 (2) 157- 159PubMedGoogle Scholar 3. Thein MRavat FOrchard GCalonje ERussell-Jones R Syringotropic cutaneous T-cell lymphoma: an immunophenotypic and genotypic study of five cases. Br J Dermatol 2004;151 (1) 216- 226PubMedGoogle ScholarCrossref 4. Kazakov DVBurg GKempf W Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol 2004;18 (4) 397- 415PubMedGoogle ScholarCrossref 5. Gerami PGuitart J Folliculotropic Sézary syndrome: a new variant of cutaneous T-cell lymphoma. Br J Dermatol 2007;156 (4) 781- 783PubMedGoogle ScholarCrossref 6. Haller AElzubi EPetzelbauer P Localized syringolymphoid hyperplasia with alopecia and anhidrosis. J Am Acad Dermatol 2001;45 (1) 127- 130PubMedGoogle ScholarCrossref 7. Hymes KB Choices in the treatment of cutaneous T-cell lymphoma. Oncology (Williston Park) 2007;21 (2) (suppl 1)18- 23PubMedGoogle Scholar

Journal

Archives of Dermatology – American Medical Association

Published: Jan 1, 2009

Keywords: alopecia,atrophic condition of skin,petechiae,papule

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