 Abstract | | | Mycosis fungoides is the commonest type of primary cutaneous T cell lymphoma. Hypopigmented mycosis fungoides is an uncommon variant usually observed in dark-skinned individuals, especially children. Large plaque parapsoriasis, a disease of the middle-aged, and with no racial and geographical predilection, can be regarded as the clinically benign end of the mycosis fungoides disease spectrum. Case of a 24-year-old male, with asymptomatic hypopigmented lesions with characteristics of large plaque parapsoriasis, and vitiligo-like skin lesions with characteristics of mycosis fungoides developing over pre-existing hypopigmented lesions, with no systemic features, is presented for its unusual clinical features and conspicuous histopathological findings.
Keywords: Mycosis fungoides, Vitiligo-like, Large plaque parapsoriasis
How to cite this article:
Das JK, Sengupta S, Gangopadhyay AK. Vitiligo-like lesions of mycosis fungoides coexisting with large plaque parapsoriasis: An association or a spectrum?. Indian J Dermatol 2006;51:120-2 |
How to cite this URL:
Das JK, Sengupta S, Gangopadhyay AK. Vitiligo-like lesions of mycosis fungoides coexisting with large plaque parapsoriasis: An association or a spectrum?. Indian J Dermatol [serial online] 2006 [cited 2014 Mar 7];51:120-2. Available from: http://www.e-ijd.org/text.asp?2006/51/2/120/26934 |
| Introduction | |  |
Mycosis fungoides (MF), the commonest primary cutaneous T cell lymphoma (CTCL), is generally associated with an indolent clinical course.[1] It is a neoplastic growth of mature helper T cells.[2] Though it is not rare worldwide, it is rarest among the Asians and commonest among the Blacks.[3]
The cutaneous manifestations of MF typically show three basic phases: an early, erythematous, scaly eczema-like patch stage; a plaque stage; and a tumor stage.[2] Hypopigmented mycosis fungoides is an uncommon variant. It is found mostly in dark-skinned individuals, especially children, although it has been reported in adults and children of Caucasians.[1],[3],[4],[5]
Parapsoriasis is a group of uncommon but not rare disorders, characterized by persistent, scaly, inflammatory eruptions. The current, generally accepted classification includes three entities: large plaque parapsoriasis (LPP), small plaque parapsoriasis (SPP), and pityriasis lichenoides.[6] Clinically, LPP lesions are mildly pruritic or asymptomatic patches or very thin plaques, with oval or irregular shape. Most lesions are bigger than 5 cm in diameter. They typically show fine wrinkles and scanty scales.[6] On histological examination, an interface infiltrate, and in the more advanced lesions, definite epidermotropism are seen.[6] The nosological position of SPP and LPP is highly controversial, and so is their relationship with MF.[7],[8],[9],[10]
| Case Report | | |
A 24-year-old male presented with the history of hypopigmented scaly patches, developing gradually on the trunk and buttocks over the past four years. It started as asymptomatic hypopigmented scaly patches on the left buttock, followed quickly by appearance of new similar lesions on the right buttock, both axillae and on lateral aspects of the trunk. The lesions increased in size gradually for a few months. One vitiligo-like patch was noticed appearing superimposed on the pre-existing hypopigmented patch on the left buttock about two years back; it spread to assume the current size in one year. Another vitiligo-like patch was noticed on right buttock about one year back. The second vitiligo-like patch continued to increase in size till the time of examination. New asymptomatic hypopigmented scaly patches continued to appear all the time till the patient came to us.
There was no history of systemic features such as fever, lassitude, or weight loss. On examination, there were many asymptomatic hypopigmented oval/irregular shaped, well-defined macules, 3 cm to 12 cm in diameter, on the trunk [Figure - 1]. The macules showed finely wrinkled slightly scaly surface. There was no induration and no telangiectasia. The vitiligo-like lesions were on the buttocks, overlying on and intermingling with the hypopigmented patches. The larger lesion on the left buttock was irregular in outline, measuring about 15 cm along the lesion along the long axis [Figure - 2]. No lymphadenopathy was noted. General examination was unremarkable. Histological examination of a vitiligo-like lesion on the left buttock showed an interface infiltrate of mononuclear cells with prominent epidermotropism, with invading cells lying singly as well as in groups [Figure - 3]. Histological examination of a hypopigmented lesion on the chest showed an interface infiltrate of mononuclear cells with epidermotropism at a few places [Figure - 4]. No atypical cells were seen in either of the lesions. Haemogram, routine blood biochemistry, chest X-ray and urine analysis were normal. There was no atypical cell in the peripheral blood. the patient was put on PUVASOL in a standardized dosage schedule that was previously used by us.[11]
| Discussion | | |
Hypopigmented mycosis fungoides is an uncommon variant, with 113 cases (having hypopigmented lesions either as the sole manifestation of the diseases presenting in association with erythematous patches) documented in the literature till date.[4] Vitiligo-like lesions are rare. In 2000, Cribier and co-workers reported four cases of vitiligo with histologic features simulating MF, but it has been opined that these cases might have represented MF and not vitiligo.[4] Almost all cases of hypopigmented MF have been found in non-Caucasian races, and is more prevalent in children.[1],[2],[3],[4],[5] Histologically, hypopigmented MF lacks epidermal atrophy and demonstrates moderate to marked exocytosis resembling pagetoid reticulosis. The present case is very typical in this regard. Hypopigmented MF often shows a T suppressor CD 8 positive phenotypes although CD 4 positive phenotype has also been reported.[4] Monoclonal proliferation of T lymphocytes, characteristic but not diagnostic of MF, has been found to be confined mainly to the epidermis in this variant.[4] The pathogenesis of hypopigmentation if MF is still unclear.
The nosological position of SPP and LPP and their relationship with MF is a highly debated issue.[7],[8],[9],[10] Similar features such as widespread epidermal expression of human leukocyte antigen (HLA-DR), a predominance of CD[4] cell subsets, and frequent CD7 antigen deficiency are shown in LPP and in mycosis fungoides lesions in immunohistological studies.[6]
The T cells mediating most inflammatory skin diseases belong to the skin associated lymphoid tissue (SALT). Mycosis fungoides is a SALT T cell neoplasm, i.e. a malignancy of a T cell circuit rather than one particular tissue Trafficking of mycosis fungoides tumor cells has been detected even in patients with very early stage disease with lesions clinicopathologically consistent with LPP. So LPP, too, may be considered as a monoclonal proliferation of SALT cells having the capacity to traffic between skin and extracutaneous sites, at least in some cases.[6],[12]
A meta-analysis of four large and carefully studied series has shown that in cases presenting clinically as LPP, the possibility to progress to MF is about 10%.[6],[13] Studies of LPP/early MF have suggested a dominant clonal density in 1 to 10 percent, and in advanced MF, a density of 50 percent or more is found.[6] An increase in dominant T cell clonal density resulting from mutations that confer increasing growth autonomy to the neoplastic T cell clone accompanies the change from LPP through various stages of MF disease spectrum. These studies, however, could not be done here due to lack of infrastructural facilities. Thus large plaque parapsoriasis may be considered as an early 'plaque' phase of mycosis fungoides, and this has considerable prognostic and therapeutic significance. The present case is reported as it clinically represents the transition from LPP to MF.
| References | | |
| 1. | MacKie RM. Cutaneous lymphomas and lymphocytic infiltrates. In : Champion RH, Burton JL, Burns DA, Breathnach SM. eds. Rook/Wilkinson/Ebling Textbook of Dermatology. vol 1, 6th ed. Blackwell Science Ltd: 1998. p. 2373-84.  | | 2. | Lambroza E, Kohen SR, Phelps R, Lebwohl M, Braverman IM, DiCostanzo D. Hypopigmented varioant of mycosis fungoides: Demography, histopathology and treatment of seven cases. J Am Acad Dermatol 1995;32:987-93. | | 3. | Latkowski J, Heald P. Cutaneous T cell lymphoma. In : Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SL, ed. Dermatology in General Medicine. 6th ed. McGraw-Hill: New York; 2003. p. 1537-58. | | 4. | Ardiog M, Borroni G, Muscardin L, Kerl H, Cerroni L. Hypopigmented mycosis fungoides in Caucasian patients:a clinicopathologic study of 7 cases. J Am Acad Dermatol 2003;49:264-70. | | 5. | Akaraphanth R, Douglass MC, Lim HW. Hypopigmented mycosis fungoides: Treatment and 6.5 year follow-up of 9 patients. J Am Acad Dermatol 2000;42:33-9. [PUBMED] [FULLTEXT] | | 6. | Wood GS, Hu CH. Parapsoriasis. In : Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, editors. Dermatology in General Medicine. 6th ed. McGraw-Hill: New York; 2003. p. 450-5. | | 7. | Ackerman AB. If small plaque (digitate) parapsoriasis is a cutaneous T-cell lymphoma, even an 'abortive' one, it must be mycosis fungoides! Arch Dermatol 1996;132:562-6. [PUBMED] | | 8. | Burg G, Dummer R. Small plque (digitate0 parapsoriasis is an 'abortive cutaneous T cell lymphoma' and is not mycosis fungoides. Arch Dermatol 1995;131:336-8. [PUBMED] | | 9. | Burg G, Dummer R, Andeas H, Kempf W, Kadin M. From inflammation to neoplasia Mycosis fungoides evolves from reactive inflammatory conditions (lymphoid infiltrates) transforming into neoplastic plaques and tumors. Arch Dermatol 2001;137:949-52. | | 10. | Rubegni P, Aloe GD, Renzo MD, Pompella G, Pasqui AL, Auteri A, et al. Cytokine production profile of peripheral blood mononuclear cells in patients with lage-plaque parapsoriasis. Arch Dermatol 2001;137:966-7. | | 11. | Das J, Gangopadhyay A. Mycosis fungoides with unusual vitiligo-like presentation. Indian J Dermatol Venereol Leprol 2004;70:304-6. | | 12. | Haeffner AC, Smoller BR, Zepter K, Wood GS. Differentiation and clonality of lesional lymphocytes in small plaaue parapsoriasis. Arch Dermatol 1995;131:321-4. | | 13. | Lambert WC, Everett MA. The nosology of Parapsoriasis. J Am Acad Dermatol 1981;5:373-95. |
Figures [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4] |
