|SPOT THE DIAGNOSIS
|Year : 2016 | Volume
| Issue : 1 | Page : 52-53
Asymptomatic hypopigmented macules on the trunk and limbs of a young male
Soumya Jagadeesan1, Malini Eapen2, Jacob Thomas1
1 Department of Dermatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
2 Department of Pathology, Amrita Institute of Medical Sciences, Kochi, Kerala, India
|Date of Web Publication||17-Jun-2016|
Dr. Soumya Jagadeesan
Department of Dermatology, Amrita Institute of Medical Sciences, Kochi, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jagadeesan S, Eapen M, Thomas J. Asymptomatic hypopigmented macules on the trunk and limbs of a young male. Pigment Int 2016;3:52-3
A 35-year-old man presented with asymptomatic hypopigmented macules on the chest, back, arms, and thighs for the past 4 years, with no preceding inflammatory lesions. He was otherwise healthy with unremarkable medical and family histories. Physical examination revealed multiple hypopigmented macules on the chest, abdomen, back, and legs [Figure 1], with no scaling, atrophy/telangiectasias. Mucosal surfaces were spared with no sensory loss or peripheral nerve thickening. No significant lymphadenopathy/hepatosplenomegaly was seen.
|Figure 1: Well to ill-defined hypopigmented macules with smooth surface on the trunk of a dark skinned individual|
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Histopathological examination of the hypopigmented lesion on the thigh showed lentiginous arrangement of lymphoid cells along the basal layer with focal collections intraepidermally; some of which appeared larger and mildly irregular [Figure 2]. Papillary dermis was edematous and was infiltrated with lymphoid cells and a few histiocytes. Immunohistochemical studies demonstrated most of the lymphoid cells to be T-cells (CD3+ and 20−) with all of them staining positive with CD8 [Figure 3].
|Figure 2: Lentiginous arrangement of large, irregular looking lymphoid cells in the epidermis. (H and E, ×400)|
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|Figure 3: Immunohistochemistry with CD8 marker highlighting the intraepidermal lymphocytes to be uniformly CD8+ (×400)|
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| Diagnosis|| |
Hypopigmented mycosis fungoides (HMF).
| Discussion|| |
MF is the most common form of cutaneous T-cell lymphoma. It usually presents as patches, plaques, nodules, or tumors although several atypical variants of MF have been described. HMF is considered to be a unique variant, first described by Ryan et al. in 1973, distinct from poikiloderma or pigmentary changes that occur in conventional MF. Breathnach et al. postulated that atypical lymphoid cells infiltrating the epidermis cause melanocyte degeneration and abnormal melanogenesis as a result of nonspecific cell injury. In contrast, other workers have proposed hypopigmentation to be secondary to a defect in melanosome transfer. Recently, Furlan et al. demonstrated fewer melanocytes and CD117+ melanocytes in HMF and postulated that cytotoxic CD8+ cell action may determine CD117/MiTF dysfunction, causing hypopigmentation.
The clinical presentation is with asymptomatic hypopigmented to depigmented macular lesions, some of which may mimic vitiligo. The lesions are not sharply circumscribed and may be slightly dry. The usual sites of involvement are the trunk and the proximal aspect of extremities. The presentation is often delayed as the initial lesions may be subtle and the progression very gradual as was the case in our patient.
HMF differs from classical MF in certain ways. In contrast to classical MF which is commonly found in the fifth–sixth decades of life, HMF preferentially involves young adults and children. A predilection for the involvement of dark-skinned individuals and Asians is also noted. The prognosis for HMF is considered much better than the classical type; the lesions tend to retain their macular character for many years, and usually do not progress beyond stage 1B (International Society of Cutaneous Lymphoma staging), although some authors have reported aggressive forms.
The histological features of HMF may not be much different from the classical type of MF. A review of the histopathological features of HMF in Indian patients found psoriasiform/lichenoid epidermal pattern, disproportionate epidermotropism, basilar tagging of lymphocytes, haloed lymphocytes, and wiry dermal collagen to be present in most of the patients. Pautrier microabscess More Detailses are also uncommon. In spite of these, single histological examination may not be conclusive, and repeated biopsies may be required to confirm the diagnosis.
Immunohistochemistry can be helpful in such cases. In contrast to classical MF where the epidermotropism is characterized by neoplastic lymphocytes with a predominant CD4+ phenotype and CD8+ phenotype restricted to the reactive lymphocytes in the dermal papillae, the neoplastic lymphocytes exhibiting epidermotropism are usually CD8+ in HMF.,
Differential diagnosis should include pityriasis alba, progressive macular hypomelanosis, leprosy, vitiligo, idiopathic guttate hypomelanosis, postinflammatory hypopigmentation, treponematoses, sarcoidosis, pityriasis lichenoides chronica, and lichen sclerosus. Inflammatory vitiligo can be a close differential, both clinically and histopathologically. Soro et al. have listed certain features that may help in differentiating both the conditions. HMF should be considered as a differential in any hypopigmented patch/macule resistant to treatment. Diagnosis involves clinicopathologic correlation as the changes can be subtle in the early stages.,
Treatment options include narrow-band ultraviolet-B therapy, photochemotherapy, topical steroids, carmustine, nitrogen mustard, and total skin electron beam therapy. Given the indolent nature of the disease and the high probability of recurrence, aggressive treatment measures are generally not favored, and long-term follow-up is essential.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Ryan EA, Sanderson KV, Barták P, Samman PD. Can mycosis fungoides begin in the epidermis? A hypothesis. Br J Dermatol 1973;88:419-29.
Furlan FC, de Paula Pereira BA, da Silva LF, Sanches JA. Loss of melanocytes in hypopigmented mycosis fungoides: A study of 18 patients. J Cutan Pathol 2014;41:101-7.
Khopkar U, Doshi BR, Dongre AM, Gujral S. A study of clinicopathologic profile of 15 cases of hypopigmented mycosis fungoides. Indian J Dermatol Venereol Leprol 2011;77:167-73.
Furlan FC, Sanches JA. Hypopigmented mycosis fungoides: A review of its clinical features and pathophysiology. An Bras Dermatol 2013;88:954-60.
Koorse S, Tirumalae R, Yeliur IK, Jayaseelan E. Clinicopathologic profile of hypopigmented mycosis fungoides in India. Am J Dermatopathol 2012;34:161-4.
Soro LA, Gust AJ, Purcell SM. Inflammatory vitiligo versus hypopigmented mycosis fungoides in a 58-year-old Indian female. Indian Dermatol Online J 2013;4:321-5.
[Figure 1], [Figure 2], [Figure 3]