|LETTER TO THE EDITOR
|Year : 2017 | Volume
| Issue : 1 | Page : 100-102
A case of multiple skin nodules: Cutaneous T-cell lymphoma
Anita Basavaraj1, Rahul S Kulkarni2
1 Department of Medicine, Byramjee-Jeejeebhoy Government Medical College, Pune, Maharashtra, India
2 GCRI, Ahmedabad, Gujarat, India
|Date of Web Publication||9-Jan-2017|
Dr. Rahul S Kulkarni
F-6, Shalimar Flats, Shahibaug, Ahmedabad - 380 004, Gujarat
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Basavaraj A, Kulkarni RS. A case of multiple skin nodules: Cutaneous T-cell lymphoma. Med J DY Patil Univ 2017;10:100-2
A 51-year-old married male farmer, diabetic for 5 years, presented with recurrent vomiting and generalized weakness for 4 days. His random blood sugar was grossly deranged (496 mg%) and was admitted for blood sugar control. However, on examination, he had multiple nodules all over the body. These skin eruptions began 10 years back and developed slowly from excoriations into nodular lesions which initially appeared over the face and slowly developed all over the body during the last 1-year. These lesions were diagnosed as neurofibromatosis during his previous hospital visits and were not investigated further. However, on inquiry, the patient admitted recent rapid increase in the size of these nodules over last 3-4 months.
There was no history of oral or genital ulcerations, palm and sole involvement, decreased visual acuity, fever, night sweats, weight loss, abdominal pain, and loss of sensations. Personal and family history was not contributory. General examination was normal except cervical lymphadenopathy. Systemic examination was within normal limits. Dermatological examination showed multiple nodules of varying sizes ranging from 0.5 to 6 cm in diameter over the scalp, face, both ears, neck, trunk, and extremities. The Large exophytic growth of 7.5 cm was seen over the forehead with ulceration. Single nodule of 6 cm over the back with hemorrhagic crust was seen. Multiple ulcerated nodules and plaques with blood and pus discharge and hyperpigmentation over face, trunk, and extremities were seen [Figure 1]a,[Figure 1]b,[Figure 1]c. Nails and oral and ocular mucosa was normal.
|Figure 1: (a-c) Multiple nodules of varying sizes ranging from over the scalp, face, neck, trunk, and extremities. (d-f) Dramatic response and significant resolution of skin lesions after chemotherapy|
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At this stage, a differential diagnosis of neurofibromatosis, cutaneous T-cell lymphoma (CTCL), multiple reticulohistiocytosis, sarcoidosis, Hansen's disease, and cutaneous leishmaniasis was made. A routine investigation including hemogram, renal and liver function tests, HIV, anti-leishmania antibody, venereal disease research laboratory was also negative. Chest X-ray was within normal limits. Skin biopsy showed flattening of rete ridges and mild focal intraepithelial lymphocytic infiltrate. Dermis shows nodular infiltrate composed of histiocytes with a variable number of lymphocytes, and few multinucleated giant cells, suggestive of diffuse cutaneous reticulohistiocytosis [Figure 2]g. However, CD 68 marker was found to be negative thus excluding the diagnosis. No parasite/fungal element was seen.
|Figure 2: (g) Skin biopsy showing flattening of rete ridges and mild focal intraepithelial lymphocytic infiltrate. (h and i) Immunohistochemistry showing intraepidermal lymphocytic infiltrate, dense monotonous population of atypical lymphoid cells with prominent nucleoli which uniformly expressed CD 3 and negative for CD 20|
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Further, immunohistochemistry panel showed intraepidermal lymphocytic infiltrate, dense monotonous population of atypical lymphoid cells with prominent nucleoli which uniformly expressed CD 3 and were negative for CD 20. No Hodgkin's Reed-Sternberg cells More Details were seen, suggesting cutaneous involvement by non-Hodgkin lymphoma — T-cell type [Figure 2]h and [Figure 2]i.
Complete blood count revealed no abnormal sezary cells in circulation. Bone marrow biopsy showed no nodular or diffuse infiltrate. USG abdomen was normal. Computed tomography (CT) thorax revealed bilateral axillary lymphadenopathy. Fine needle aspiration cytology cervical and axillar lymph node showed reactive lymphadenitis with no evidence of malignancy. Positron emission tomography/CT scan was not done due to financial constraints. Considering the widespread skin involvement of around 90% body surface with surrounding erythema around few lesions without lymph node and visceral involvement, the final diagnosis according to tumor-node-metastasis classification was stage IIIA (T4N0M0B0) CTCL.
The patient was initially treated with intravenous (IV) fluids, antibiotics, and IV insulin therapy for control of blood sugar level and ketoacidosis. After stabilization, the patient was started on CVP regimen(cyclophosphamide 1500 mg, vincristine 2 mg IV push, and Prednisolone 100 mg OD for 5 days). The patient received 6 cycles of chemotherapy at 3 weekly intervals, after completion of 6 cycles of chemotherapy, there was the dramatic resolution of skin lesions [Figure 1]d,[Figure 1]e,[Figure 1]f. The patient is currently under follow-up.
Mycosis fungoides is the most common variant of CTCL and is characterized by indolent course with subsequent evolution of patches plaques and tumors and histologically by infiltration of the epidermis by atypical T-cells with cerebriform nuclei. CTCL usually develops slowly over many years. In early stages, skin becomes itchy and dry which turn into dark patches. Later, on tumors may form on the skin. This stage is called mycosis fungoides. The term “mycosis fungoides” was initially used by Alibert in 1806 to describe a skin eruption that developed into tumors shaped like mushrooms. Mycosis fungoides is a misnomer because there is no association with a fungus. As the disease progresses, skin becomes involved and become infected, and disease spreads to lymph nodes, spleen, lungs, and liver. In this advanced stage, a large number of tumor cells are found in blood, and this stage is called sezary syndrome. It may persist in this stage for years or sometimes decades and slowly progress to another stage (from patches to thicker plaques and eventually to tumors).
CTCL may mimic other common skin disorders such as eczema, psoriasis, dermatophytosis, sarcoidosis or neurofibromatosis in widespread disease thus delaying the diagnosis. Various treatment options are available such as topical steroids, nitrogen mustards, PUVA therapy, extracorporeal photopheresis, and immunotherapy. Systemic chemotherapy such as CVP, cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP), etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin (EPOCH) regimens are generally reserved for advanced disease. Alemtuzumab is a monoclonal antibody which attaches to cancer cells and triggers the immune system to attack and destroy the cancer cells. It is usually given in recurrent CTCL or failure of other modalities. Certain newer drugs such as forodesine (inhibits nucleotide phosphorylase) and panobinostat are currently under trial.
Thus, authors suggest that lymphoma should be included in the differential diagnosis of any chronic skin condition resistant to treatment. Early diagnosis and prompt institution of treatment can prevent widespread involvement and improve the chances of cure.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Grover S, Verma R, Mani NS, Grewal RS, Singh GK. Primary cutaneous T cell lymphoma: Two rare presentations. MJAFI 2010;66:73-5.
Alibert JL. Description of skin diseases: Observed at St. Louis Hospital and exhibition of best methods used in their treatment. 1806. p. 413-46.
Bagot M. Introduction: Cutaneous T-cell lymphoma (CTCL) — classification, staging, and treatment options. Dermatol Clin 2008;26 Suppl 1:3-12.
Rosen ST, Querfeld C. Primary cutaneous T cell lymphomas. Hematolgy 2006;1:323-9.
[Figure 1], [Figure 2]