Hypopigmented Mycosis Fungoides Present on the Face Without Oral (HMF) [2, 5]

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Diffuse Hypopigmented Rash on an Eight-Year Old Girl Sheena Nguyen, DO and Navid Nami, DO Western University of Health Sciences/Chino Valley Medical Center

CASE PRESENTATION DISCUSSION

Chief Complaint: White spots all over body Figure 1: Many scattered Mycosis fungoides (MF) is the most common primary cutaneous T cell lymphoma that usually presents in the stages of patches, plaques, tumors or asymptomatic, hypopigmented erythrodermca [1-4]. There are several distinct variants of MF that have Signs and symptoms: Patient is an eight-year-old girl who presented to macules coalescing into patches their own clinical behavior and outcome. They include pustular, bullous, clinic with a three-year history of asymptomatic, hypopigmented macules purpuric, follicular, icthyosiform, veurrucous, unilesional, invisible, spread diffusely throughout her body. She denied any preceding illnesses, granulomatous, hyperkeratotic, and hypopigmented mycosis fungoides present on the face without oral (HMF) [2, 5]. or systemic symptoms. involvement. HMF is an indolent cutaneous T cell lymphoma that represents a rare Past Medical History: None clinical variant of the early patch stage of MF [1, 3, 6, 7]. It is most Figures 2 and 3: Diffusely scattered commonly seen in dark skinned and Asian individuals, it has infrequently been reported in Caucasians as well [1-8]. The average age of onset is Medications: None yet discrete hypopigmented usually seen between the first and third decades as compared to classic MF, macules and patches present here which manifests around the sixth decade [1, 2, 6, 7, 9]. Family History: Non-contributory on the trunk and bilateral upper Patients will most commonly present with multiple, ill-defined, hypopigmented patches of varying sizes on the trunk, back and proximal Social History: Lives at home with parents, attends elementary school, extremities without areas of extremities [2, 4-6, 8]. Cutaneous sensation will be intact with a variable denies alcohol/tobacco/illicit drug use, denies recent travel Fig. 1 sparing. degree of scaling, atrophy, infiltration, and pruritus [2, 4-6, 8]. The differential diagnosis for HMF includes all possible etiologies for Surgical History: None hypopigmented patches on the skin, such as vitiligo, leprosy, progressive macular hypomelanosis, sarcoid, pitryiasis lichenoides chronica, tinea versicolor, pitryiasis alba, morphea, post inflammatory hypopigmentation, Allergies: NKDA hypomelanosis of ito, syphilis, among many others [1-6, 8].

Previous Treatment: Patient’s mother reported having been prescribed and Histopathological examination usually shows an intense disproportionate using Triamcinolone 0.1% ointment on affected areas twice daily for the epidermotropism of large haloed lymphocytes with convoluted nuclei individually or in small clusters (pautrier microabscesses), basilar tagging patient for one month without improvement. of lymphocytes, lymphocytic infiltration in the dermis with mild atypia, papillary dermal fibrosis and wiry collagen [1-9]. Occasionally pigment Physical Exam: Patient is a well-nourished, well-appearing female who incontinence, melanophages, and folliculotropsim may also be seen [5, 6, 9]. HMF lesions characteristically demonstrate CD8+ T cells with a low presented in no acute distress. She had a diffuse eruption of scattered CD4+: CD8+ ratio, unlike classic MF that presents with a CD4+ hypopigmented macules without other surface changes ranging in size from predominant infiltrate [1-9]. 2mm - 12mm throughout her face and body. There was no oral involvement. Proposed mechanisms for the cutaneous hypopigmentation include the cytotoxic effect of CD8+ T cells on CD117 causing dysfunction and/or loss of melanocytes, a defect in the transfer of melanosomes from melanocytes Laboratory Tests: Complete blood count and blood chemistries within to keratinocytes, and a nonspecific result of inflammation [2, 4, 8, 9]. normal limits. The presence of a T-cell receptor gene arrangement is seen in a large Quantitative T cell subset and T cell receptor rearrangement in blood are percentage of patients with HMF, however the absence of clonality does negative. not rule out the disease [1, 6-9]. Initial evaluation may include CBC with differential, quantification of T and B cell lymphocytes using flow cytometery, physical exam of the peripheral lymph nodes, and diagnostic Quantitative immunoglobulins are normal. imaging if suspecting any systemic involvement [2, 7]. Fig. 2 Fig. 3 Psoralen + UVA (PUVA) or narrow band UVB with/without topical Dermatohistopathology: A shave biopsy was performed on the left upper steroids is the mainstay of treatment [1-10]. Topical nitrogen mustard, back that demonstrated mild psoriasiform hyperplasia of the epidermis with topical carmustine, electron beam therapy, bexarotene, methotrexate, and a paucicellular atypical epidermotropic lymphoid infiltrate. Rare foci pralatrexate have also showed effectiveness [2, 4, 5, 7, 8, 10]. Most demonstrating “lining up” of atypical lymphoid cells along the patients require repetitive cycles of phototherapy or maintenance therapy dermoepidermal junction and haphazardly infiltrating the epidermis. In REFERENCES due the high recurrence rates associated with HMF [1, 2]. addition, there is superficial perivascular lymphohistiocytic inflammatory 1. Castano E, Glick S, Wolgast L, Naeem R, Sunkara J, Elston D, Jacobson M: Hypopigmented mycosis fungoides in childhood and adolescence: a long-term infiltrate with abundant incontinent melanin pigment. PAS stain is negative HMF has an indolent course with an overall survival of 98% at 20 years, a retrospective study. J Cutan Pathol 2013, 40(11):924-934. significantly better prognosis than classic MF [3, 5]. However there have for fungal organisms. been cases of progression to tumor, systemic involvement, and even death 2. Furlan FC, Sanches JA: Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology. An Bras Dermatol 2013, 88(6):954-960. [3, 4, 9]. It has been postulated that the predominance of CD8+ T cells 3. Gameiro A, Gouveia M, Tellechea O, Moreno A: Childhood hypopigmented mycosis fungoides: a commonly delayed diagnosis. BMJ Case Rep 2014, 2014. Immunologic staining performed reveals a predominance of CD3+ T-cells prevents the evolution of HMF lesions to MF plaques and tumors by 4. Stone ML, Styles AR, Cockerell CJ, Pandya AG: Hypopigmented mycosis fungoides: a report of 7 cases and review of the literature. Cutis 2001, 67(2):133- participating in the protective Th1 response [2, 3]. Hypopigmentation may in the atypical lymphoid cells. The CD8:CD4 ratio appears markedly be considered as a marker of good prognosis. increased, although the total number of atypical cells is small. There is 138. some loss of staining with pan T-cell marker CD7. There is retention of 5. Hassab-El-Naby HM, El-Khalawany MA: Hypopigmented mycosis fungoides in Egyptian patients. J Cutan Pathol 2013, 40(4):397-404. The diagnosis is usually rendered by clinicopathological correlation staining with pan T-cell marker CD5. Both CD20 and CD30 immunostains 6. Koorse S, Tirumalae R, Yeliur IK, Jayaseelan E: Clinicopathologic profile of hypopigmented mycosis fungoides in India. Am J Dermatopathol 2012, supplemented by immunohistochemistry and ancillary studies such as highlight rare lymphoid cells. Mart-1 demonstrates a normal distribution 34(2):161-164. TCR-gene rearrangement, blood tests, and diagnostic imaging. Occasionally you may need several skin biopsies to confirm the disease. and number of melanocytes arguing against vitiligo and most cases of 7. Tolkachjov SN, Comfere NI: Hypopigmented mycosis fungoides: a clinical mimicker of vitiligo. J Drugs Dermatol 2015, 14(2):193-194. pityriasis alba. 8. Khopkar U, Doshi BR, Dongre AM, Gujral S: A study of clinicopathologic profile of 15 cases of hypopigmented mycosis fungoides. Indian J Dermatol Due to the increasing frequency of HMF it is important to be aware of this Venereol Leprol 2011, 77(2):167-173. entity and perform a skin biopsy especially in patients with persisting or Patient Course/Treatment: Once the diagnosis of hypopigmented mycosis 9. El-Shabrawi-Caelen L, Cerroni L, Medeiros LJ, McCalmont TH: Hypopigmented mycosis fungoides: frequent expression of a CD8+ T-cell phenotype. Am J progressing hypopigmentation despite therapy. HMF is a malignant fungoides was made, patient was referred to oncology for close follow-up Surg Pathol 2002, 26(4):450-457. neoplastic disease with a lethal potential and should be treated as such with and started on a regimen NB-UVB therapy. proper work up and mandatory follow up. 10. Galper SL, Smith BD, Wilson LD: Diagnosis and management of mycosis fungoides. Oncology (Williston Park) 2010, 24(6):491-501.