Clinieal and Experimental Dermatology 1992; 17: 374-375.
Hypopigmented mycosis fungoides S.E.HANDFIELD-JONES, N.P.SMITH AND S.M.BREATHNACH St John's Dermatology Centre, St Thomas' Hospital, London SEI 7EH, UK Accepted for puhlication 14 October 1991
Summary We report the case of a 25-year-old Jamaican woman with hypopigmented mycosis fungoides. She first developed a hypopigmented patch on her arm at the age of 11 years. Further lesions developed on the trunk and hmbs over a period of 10 years. The lesions were completely impalpable. Skin biopsy showed an infiltrate of atypical lymphocytes, some with cerebriform nuclei, suggesting a diagnosis of mycosis fungoides. The lesions cleared with PUVA therapy.
Case report A 25-year-old British-born woman of Jamaican parents first noticed a pale patch of skin on her arm when she was 11 years old. Gradually lesions developed on trunk and limbs until there was extensive, patchy hypopigmentation. The lesions were not pruritic or painful but the patient was very concerned by the appearance. On examination, the patient was in good health. There were multiple circular and oval lesions on the trunk and Figure 1. Oval and circular hypopigmented patches on back and limbs showing varying degrees of hypopigmentation (Fig. buttocks. 1). There was no suggestion of thickening or palpability in any ofthe lesions including those that had been present for many years. There was no cutaneous sensory deficit. There was no lymphadenopathy or hepatosplenomegaly. Discussion Investigations including full blood count and liver Mycosis fungoides can show many clinical patterns. In function tests were normal. Antibodies to HTLVl were addition to the well recognized plaques erythema and not detected. A skin biopsy from the trunk showed an poikiloderma, atypical manifestations include pustular, infiltrate of lymphocytes in the upper dermis with patchy bullous and verrucous forms.' Hypopigmented patches colonization of the overlying epidermis by mononuclear are a rare presenting feature. There have been 13 cases cells, some of which showed nuclear atypia. There was previously reported in the literature.^ ' All of these cases pigmentary incontinence (Fig. 2). have been in patients of African or Asian origin. The only In view ofthe patient's distress at the appearance ofthe Caucasian reported had papules and plaques in addition to lesions, PUVA therapy was started. After 8 weeks hypopigmented lesions.** Common features in all these treatment with a total cumulative dose of less 400 J the cases have been the early age of onset and the slow lesions cleared completely. Six months following comple- progression with good prognosis. As distinct from our tion of therapy she remains clear of skin lesions. patient in whom the lesions were totally impalpable, all previously reported patients eventually developed some degree of scahng or lichenification. However, none had The patient has been shown at a St. John's Meeting. 374
HYPOPIGMENTED MYCOSIS FUNGOIDES
375
includes sarcoidosis, leprosy, pityriasis lichenoides chronica and most commonly post inflammatory hypopigmentation. In our case response to PUVA therapy was excellent. However even when untreated the disease appears to run a benign course. References
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.
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*
Figure 2. Histology of skin hiopsy showing mononuclear cell infiltrate in the upper dermis and atypical lymphocytes in tbe epidermis. (H & E X 38)
developed plaque or tumour stage mycosis fungoides at the time of reporting. The cause ofthe hypopigmentation is unknown. There is no erythema or other evidence of inflammation at any stage in the disease. Ultrastructural studies have shown degeneration of melanocytes within some*^'^' but not all of the lesions in the eight cases studied. In one study, immunocytochemistry has shown a preponderance of Tcells ofthe suppressor/cytotoxic subset in contrast to the usual excess of T-helper cells,' but a further study has not confirmed this.'' The differential diagnosis of hypopigmented macules
1. Mackie RM. Mycosis fungoides. In: Rook A, Wilkinson DS, Ebling FJG, Champion RH, Burton JL, eds. Textbook of Dermatology, 4th edn. Oxford: Blackwell Scientific Publications, 2. Ryan F.A, Sanderson KV, Bartak P, Samman PD. Can mycosis fungoides begin in the epidermis? A hypothesis. British Journal of Dermatology 1973; 88: 419-429. 3. Smith NP, Samman PD, Mycosis fugoides presenting with areas of cutaneous hypopigmentation. Clinical and Experimental Dermatotogy 1978; 3: 213-216. 4. Zackheim D, Epstein EH, Grekin MD, McNutt NS, Mycosis fungoides presenting as areas of bypopigmentation. Journat ofthe American Academy of Dermatotogy 1982; 6: 340-345, 5. Breatbnach SM, McKee PH, Smith NP. Hypopigmented mycosis fungoides: report of five cases with ultrastructural observations. British Journal of Dermatology 1982; 106: 643-649. 6. Rustin MHA, Griffiths M, Ridley CM. The immunopathology of hypopigmented mycosis fungoides. Clinical and Experimental Dermatotogy 1986; 11: 332-339. 7. Goldberg DJ, Schinella RS, Kecbijian P. Hypopigmented mycosis fungoides, American Journat of Dermatotogy 1986; 8: 326-330. 8. Sigal M, Grossin M, Laroche L, Bassett F, Aitken G, Haziza JL and Belaich S. Hypopigmented mycosis fungoides. Ctinicat and Experimentat Dermatology 1987; 12: 453-454, 9. Misch KJ, MacLennan K, Marsden RA. Hypopigmented mycosis fungoides. Cltmcat and Experimental Dermatology 1987; 12: 5355.
Hypopigmented mycosis fungoides S.E.HANDFIELD-JONES, N.P.SMITH AND S.M.BREATHNACH St John's Dermatology Centre, St Thomas' Hospital, London SEI 7EH, UK Accepted for puhlication 14 October 1991
Summary We report the case of a 25-year-old Jamaican woman with hypopigmented mycosis fungoides. She first developed a hypopigmented patch on her arm at the age of 11 years. Further lesions developed on the trunk and hmbs over a period of 10 years. The lesions were completely impalpable. Skin biopsy showed an infiltrate of atypical lymphocytes, some with cerebriform nuclei, suggesting a diagnosis of mycosis fungoides. The lesions cleared with PUVA therapy.
Case report A 25-year-old British-born woman of Jamaican parents first noticed a pale patch of skin on her arm when she was 11 years old. Gradually lesions developed on trunk and limbs until there was extensive, patchy hypopigmentation. The lesions were not pruritic or painful but the patient was very concerned by the appearance. On examination, the patient was in good health. There were multiple circular and oval lesions on the trunk and Figure 1. Oval and circular hypopigmented patches on back and limbs showing varying degrees of hypopigmentation (Fig. buttocks. 1). There was no suggestion of thickening or palpability in any ofthe lesions including those that had been present for many years. There was no cutaneous sensory deficit. There was no lymphadenopathy or hepatosplenomegaly. Discussion Investigations including full blood count and liver Mycosis fungoides can show many clinical patterns. In function tests were normal. Antibodies to HTLVl were addition to the well recognized plaques erythema and not detected. A skin biopsy from the trunk showed an poikiloderma, atypical manifestations include pustular, infiltrate of lymphocytes in the upper dermis with patchy bullous and verrucous forms.' Hypopigmented patches colonization of the overlying epidermis by mononuclear are a rare presenting feature. There have been 13 cases cells, some of which showed nuclear atypia. There was previously reported in the literature.^ ' All of these cases pigmentary incontinence (Fig. 2). have been in patients of African or Asian origin. The only In view ofthe patient's distress at the appearance ofthe Caucasian reported had papules and plaques in addition to lesions, PUVA therapy was started. After 8 weeks hypopigmented lesions.** Common features in all these treatment with a total cumulative dose of less 400 J the cases have been the early age of onset and the slow lesions cleared completely. Six months following comple- progression with good prognosis. As distinct from our tion of therapy she remains clear of skin lesions. patient in whom the lesions were totally impalpable, all previously reported patients eventually developed some degree of scahng or lichenification. However, none had The patient has been shown at a St. John's Meeting. 374
HYPOPIGMENTED MYCOSIS FUNGOIDES
375
includes sarcoidosis, leprosy, pityriasis lichenoides chronica and most commonly post inflammatory hypopigmentation. In our case response to PUVA therapy was excellent. However even when untreated the disease appears to run a benign course. References
-'••.-
.
•-' I ' v l i ' ' ^ . * "
*
Figure 2. Histology of skin hiopsy showing mononuclear cell infiltrate in the upper dermis and atypical lymphocytes in tbe epidermis. (H & E X 38)
developed plaque or tumour stage mycosis fungoides at the time of reporting. The cause ofthe hypopigmentation is unknown. There is no erythema or other evidence of inflammation at any stage in the disease. Ultrastructural studies have shown degeneration of melanocytes within some*^'^' but not all of the lesions in the eight cases studied. In one study, immunocytochemistry has shown a preponderance of Tcells ofthe suppressor/cytotoxic subset in contrast to the usual excess of T-helper cells,' but a further study has not confirmed this.'' The differential diagnosis of hypopigmented macules
1. Mackie RM. Mycosis fungoides. In: Rook A, Wilkinson DS, Ebling FJG, Champion RH, Burton JL, eds. Textbook of Dermatology, 4th edn. Oxford: Blackwell Scientific Publications, 2. Ryan F.A, Sanderson KV, Bartak P, Samman PD. Can mycosis fungoides begin in the epidermis? A hypothesis. British Journal of Dermatology 1973; 88: 419-429. 3. Smith NP, Samman PD, Mycosis fugoides presenting with areas of cutaneous hypopigmentation. Clinical and Experimental Dermatotogy 1978; 3: 213-216. 4. Zackheim D, Epstein EH, Grekin MD, McNutt NS, Mycosis fungoides presenting as areas of bypopigmentation. Journat ofthe American Academy of Dermatotogy 1982; 6: 340-345, 5. Breatbnach SM, McKee PH, Smith NP. Hypopigmented mycosis fungoides: report of five cases with ultrastructural observations. British Journal of Dermatology 1982; 106: 643-649. 6. Rustin MHA, Griffiths M, Ridley CM. The immunopathology of hypopigmented mycosis fungoides. Clinical and Experimental Dermatotogy 1986; 11: 332-339. 7. Goldberg DJ, Schinella RS, Kecbijian P. Hypopigmented mycosis fungoides, American Journat of Dermatotogy 1986; 8: 326-330. 8. Sigal M, Grossin M, Laroche L, Bassett F, Aitken G, Haziza JL and Belaich S. Hypopigmented mycosis fungoides. Ctinicat and Experimentat Dermatology 1987; 12: 453-454, 9. Misch KJ, MacLennan K, Marsden RA. Hypopigmented mycosis fungoides. Cltmcat and Experimental Dermatology 1987; 12: 5355.
