Indian Journal of Dermatology
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CASE REPORT
Year : 2006  |  Volume : 51  |  Issue : 2  |  Page : 115-117
Hypopigmented mycosis fungoides in a twenty-year-old Saudi woman with fair skin


1 Department of Dermatology, Qatif Central Hospital, Qatif, Kingdom of Saudi Arabia
2 Department of Pathology, Qatif Central Hospital, Qatif, Kingdom of Saudi Arabia
3 Department of Pathology, Salmaniya Medical Complex and Arabian Gulf University, Bahrain, Saudi Arabia

Correspondence Address:
N A Ansari
Department of Pathology, Arabian Gulf University, P. O. Box - 22979, Bahrain, Saudi Arabia

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.26932

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   Abstract 

The hypopigmented variant of mycosis fungoides (MF) is charactrized by the presence of hypopigmented patches as the sole manifestation of the disease. This variant is usually observed in the dark skin of African or Asian individuals particularly children, but nevertheless is an uncommonly reported presentation of the disease. The incidence of hypopigmented MF in Caucasian patients and others of fair skin color is rare. We describe hypopigmented lesions arising in a twenty-year-old Saudi woman with type IV skin color. The diagnosis of mycosis fungoides stage IA was made based on the clinico-radiological and histological features. The patient responded well to PUVA therapy and is currently clear of lesions. MF should be considered in the differential diagnosis of hypopigmented macular lesions in patients of any skin color and age. Multiple skin biopsies must be performed on different occasions so as not to miss the diagnosis; and long term follow up is recommended since recurrence is common and occasional cases behave aggressively.


Keywords: Mycosis fungoides, Hypopigmented patch


How to cite this article:
Al-Ratrout J T, Al-Nazer M, Ansari N A. Hypopigmented mycosis fungoides in a twenty-year-old Saudi woman with fair skin. Indian J Dermatol 2006;51:115-7

How to cite this URL:
Al-Ratrout J T, Al-Nazer M, Ansari N A. Hypopigmented mycosis fungoides in a twenty-year-old Saudi woman with fair skin. Indian J Dermatol [serial online] 2006 [cited 2019 Nov 15];51:115-7. Available from: http://www.e-ijd.org/text.asp?2006/51/2/115/26932



   Introduction Top


Mycosis fungoides (MF) is a malignant neoplasm of T lymphocytes that presents in the skin. The natural history is that of a chronic, slowly progressive disorder. It typically occurs in adults, with a peak onset in middle age. The typical cutaneous presentation consists of an early erythematous scaly patch, followed by a plaque stage and a tumor stage. In addition several unusual variants have been observed, including granulomatous, pustular, purpuric, bullous, hyperpigmented, hyperkeratotic, verrucous, and follicular forms.[1] During the last three decades a hypopigmented macular form of MF has emerged in the literature with a total of a little over 100 cases reported to date. [2],[3],[4],[5],[6],[7],[8],[9]

The patient described is a twenty-year-old Saudi woman, fair in color with multiple hypopigmented lesions treated initially as pityriasis alba and pityriasis versicolor, diagnosed ultimately as mycosis fungoides and finally responding well to PUVA therapy.


   Case Report Top


A pregnant twenty-year-old Saudi woman with type IV skin color (Fitzpatrick classification) presented with a two year history of asymptomatic pale patches on the neck and trunk. During the course of her pregnancy new eruptions appeared on the upper and lower limbs. A variety of topical treatments such as steroid and antifungals were tried without improvement. She was otherwise healthy, with no personal or family history of atopy.

On examination there were multiple circular/oval macules and patches ranging in size from 1 to 2 cm. All lesions were hypopigmented with a hyperpigmented rim, many had very fine surface scales, and some of them displayed mild erythema [Figure - 1]. There was no cutaneous sensory deficit, and no organomegaly or lymphadenpopathy. Scrapings for fungal infection were negative. Wood's light examination was negative for dermatophyte infection and the color was accentuated.

After she had delivered safely, a 4 mm punch biopsy specimen was taken from the patient's flank. Histological examination was suggestive of MF but further biopsies were recommended for confirmation. A second biopsy was taken four months later from a hypopigmented lesion on the upper arm. Histopathological examination revealed the presence of a lymphocytic infiltrate in the upper dermis [Figure - 2]. the epidermis showed irregular acanthosis, and epidermotropism of lymphocytes was a prominent feature with occasional clustering and  Pautrier microabscess More Details formation [Figure - 3]. The majority of the dermal and epidermal lymphoid cells were T cells as demonstrated by positive staining for CD3 on immunohistochemistry [Figure - 4][Figure - 5][Figure - 6].

Results of full blood count, blood biochemistry, urine analysis, and serology including IgA, IgE, and ANA, were all negative. Sezary cells were not identified on peripheral blood smear. Roentgenographic bone examination showed no evidence of osteoarticular lesions. Roentgenographic and CT scan of the chest showed no abnormalities.

A diagnosis of mycosis fungoides was made based upon the clinical and histological features, and appropriately staged as IA with the additional help of radiology.

Therapy with PUVA was carried out twice every week. After eight months of treatment all lesions repigmented. The frequency of PUVA administration was reduced to once a week maintenance dose. Three years later new hypopigmented macular lesions appeared on the right shoulder and axilla. Subsequent biopsy revealed features of non-specific dermatitis and all appropriate investigations were repeated and found to be normal. The dose of PUVA was increased but maintained at a frequency of once a week. After five months of treatment all lesions repigmented and the patient currently remains clear of any new lesions, followed up regularly as an outpatient.


   Discussion Top


Hypopigmented lesions are an uncommonly reported presenting manifestation of MF, usually observed in dark skinned individuals, and exceedingly rare in Caucasians and others with fair skin types.[2],[5],[6],[7],[8]

Most cases of the hypopigmented variant of MF occur at an earlier age compared to classical MF and the response to PUVA is excellent as exemplified by our patient. It is often recurrent, so follow up is very important, given also that very occasionally the biological behaviour may become uncharacteristically aggressive.[2],[10],[11],[12],[13]

The correct identification of hypopigmented MF can be delayed for many years because it is easily misdiagnosed as pitryasis alba, leprosy, pitryasis versicolor, sarcoidosis, post inflammatory hypopigmentation or idiopathic guttate hypomelanosis[14],[15] and consequently treated with inappropriate medication. the rarity of this variant may be secondary to underreporting or incorrect diagnosis or both.

The mechanism of the hypopigmentation in this MF variant is still unclear. It has been postulated that atypical lymphoid cells infiltrating the epidermis cause melanocyte degeneration and abnormal melanogenesis as a result of non-specific cell injury.[16] In contrast, other workers propose that the loss of pigment results from a defect in melanosome transfer from melanocyte to keratinocyte.[17]

Immunophenotypic analysis of the infiltrating lymphoid cells in hypopigmented MF demonstrates a predominance of CD 4 + T cells.[2],[4],[14] Some authors describe CD 8 + positive lymphocytes in the epidermal infiltrate[3] and in these cases most of the patients reported were dark skinned individuals or children.[18]

Hypopigmented MF should be included in the differential diagnosis of any persistent hypopigmented macule or patch that is resistant to treatment. It is emphasized that this variant usually presents at a younger age than the classical variant; sequential biopsies are recommended if the diagnosis remains in doubt; and follow up of the patient is clearly indicated since recurrence is common and occasional cases behave aggressively.[2],[5],[11],[12],[13]

 
   References Top

1.Cerroni L, Keri H, Gatter K. An illustrated guide to skin lymphomas. Blackwell Science: Oxford; 1998.  Back to cited text no. 1    
2.Ardigo 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;49:267-70.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Ei Shabrawi-Caelen L, Cerroni L, Medeiros LJ, McCalmont TH. Hypopigmented mycosis fungoides. Frequent expression of a CD8+ T-cell phenotype. Am J Surg Pathol 2002;26:450-7.   Back to cited text no. 3    
4.Sigal M, Grossin M, Laroche L, Basset F, Aitken G, Haziza JL, et al . Hypopigmented mycosis fungoides. Clin Exp Dermatol 1987;12:453-4.  Back to cited text no. 4    
5.Di Landro A, Marchesi L, Naldi L, Motta T, Cainelli T. A case of hypopigmented mycosis fungoides in a young Caucasian boy. Pediatr Dermatol 1997;14:449-52.   Back to cited text no. 5  [PUBMED]  
6.Grunwald MH, Amichai B. Localized hypopigmented mycosis fungoides in a 12 year-old Caucasian boy. J Dermatol 1999;26:70-1.   Back to cited text no. 6  [PUBMED]  
7.Amichai B, Grunwald MH, Avinoach I, Halevy S. Hypopigmented mycosis fungoides in a white female. J Dermatol 1996;23:425-6.  Back to cited text no. 7  [PUBMED]  
8.Robert C, Moulonguet I, Baudot N, Flageul B, Dubertret L. Hypopigmented mucosis fungoides in a light-skinned women. Br J Dermatol 1998;139:341-3.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Ryan EA, Sanderson KV, Bartak P, Samman PD. Can mycosis fungoides begin in the epidermis? A hypothesis. Br J Dermatol 1973;88:419-29.   Back to cited text no. 9  [PUBMED]  
10.Stone ML, Styles AR, Cockerell CJ, Pandya AG. Hypopigmented mycosis fungoides: A report of 7 cases and review of the literature. Cutis 2001;67:133-8.   Back to cited text no. 10  [PUBMED]  
11.CME examination for volume 41 Include entire volume with the exception of the CME articles. J Am Acad Dermatol 2000;42:33-6.   Back to cited text no. 11    
12.Lambroza E, Stevan R, Cohen R, Phelps R, Lebwohl M, Braverman I, et al . Hypopigmented variant of mycosis fungoides: Dermatography, histopathology and treatment of seven cases. J Am Acad Dermatol 1995;32:987-93.   Back to cited text no. 12    
13.Handfield-Jones S, Smith N, Breathnach S. Hypopigmented mycosis fungoides. Clin Exp Dermatol 1992;17:374-5.   Back to cited text no. 13    
14.Whitmore E, O'Brien ES, Rotter FS. Hypopigmented mycosis fungoides. Arch Dermatol 1994;130:476-80.  Back to cited text no. 14    
15.Neuhaus IM, Ramos-Caro FA, Hassanein AM. Hypopigmented mycosis fungoides in childhood and adolescence. Pediatr Dermatol 2000;17:403-6.  Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Breathnach SM, Mckee PH, Smith NP. Hypopigmented mycosis fungoides: Report of five cases with ultrastructural observations. Br J Dermatol 1982;106:643-9.   Back to cited text no. 16  [PUBMED]  
17.Goldberg DJ, Schinella RA, Kechijian P. Hypopigmented mycosis fungoides: Speculations about the mechanism of hypopigmentation. Am J Dermatopathol 1986;8:326-30.  Back to cited text no. 17    
18.Whittam LR, Calonjie E, Orchard G, Fraser-Andrews EA, Woolford A, Russel-Jones R. CD8 positive juvenile onset mycosis fungoides:an immunohistichemical and genotypic analysis of six cases. Br J Dermatol 2000;143:1199-204.  Back to cited text no. 18    


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]

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    Abstract
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    Case Report
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