Journal of Dr. NTR University of Health Sciences

: 2016  |  Volume : 5  |  Issue : 3  |  Page : 230--233

Multifocal primary cutaneous anaplastic lymphoma

Panthalla Vijaya Lakshmi, Indukuri Chandrasekhar Reddy, Mounika Ala 
 Department of Dermatology, Venereology and Leprology, Kurnool Medical College, Kurnool, Andhra Pradesh, India

Correspondence Address:
Panthalla Vijaya Lakshmi
Department of Dermatology, Venereology and Leprology, Kurnool Medical College, Flat No. 309, MS9 Priya Towers, Deva Nagar, Kurnool, Andhra Pradesh


Primary cutaneous anaplastic large cell lymphoma (CALCL) is a rare lymphoproliferative disorder characterized by proliferation of CD4 T helper cells that express CD30+ marker with anaplastic cytology. These lymphomas are seen in adults and present as solitary or multiple and often ulcerated nodules most often on the trunk. Multifocal lesions are seen in only 20% of patients. Careful staging with computed tomography scan is required to exclude systemic CD30+ anaplastic large cell lymphoma. Multifocal primary CALCL, not extensively reported is more likely to progress to extracutaneous involvement than localized disease. Herein, we report a case of an 83-year-old male with a 6 months history of minimally pruritic generalized ulcerated nodules.

How to cite this article:
Lakshmi PV, Reddy IC, Ala M. Multifocal primary cutaneous anaplastic lymphoma.J NTR Univ Health Sci 2016;5:230-233

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Lakshmi PV, Reddy IC, Ala M. Multifocal primary cutaneous anaplastic lymphoma. J NTR Univ Health Sci [serial online] 2016 [cited 2022 Sep 24 ];5:230-233
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As per the WHO-EORTC classification,[1] primary cutaneous lymphoproliferative disorders consist of a spectrum of conditions including lymphomatoid papulosis and CD30+ anaplastic large cell lymphoma (ALCL), which are defined on the basis of clinical and pathological features. Primary cutaneous ALCL (CALCL) is a rare disorder with an incidence of only 1.2-1.9 cases in 1,000,000. Multifocal lesions are seen in only 20% of these cases. Primary ALCL must be differentiated from systemic ALCL which requires aggressive therapy. We report a case of primary CALCL with multifocal lesions.

 Case Report

An 83-year-old man presented with multiple ulcerated plaques over face, trunk, both upper and lower extremities for 6 months. The lesions began as erythematous papules over upper extremities, which subsequently enlarged to form nodules, which eventually ulcerated. The lesions were mildly itchy and there was a history of serosanguinous discharge from the lesions. There were no constitutional symptoms.

He was initially treated with oral antibiotics and topical steroids by a local physician as the condition was presumed to be of infectious origin, but there was no improvement.

Clinically the plaques were indurated and tender with dusky erythematous rolled edges. There was a large ulcer of size 10 cm × 15 cm size over right forearm. Similar lesions were present over trunk and extremities. Multiple hyper-pigmented patches were present over the back, and a granulomatous plaque was present over the nose [Figure 1], [Figure 2], [Figure 3].{Figure 1}{Figure 2}{Figure 3}

There was no cervical, axillary or inguinal lymphadenopathy.

Routine blood investigations were normal.

Skin biopsy revealed a nodular interstitial infiltrate of predominantly large lymphocytes and plasmacytoid cells, involving the whole of reticular dermis and extending to the sub-cutis. The cells had pleomorphic nuclei and nucleoli, frequent mitosis was evident. The histopathological features were consistent with ALCL [Figure 4] and [Figure 5].{Figure 4}{Figure 5}

In order to rule out systemic involvement ultrasonogram of abdomen, computed tomography scan with contrast of thorax and abdomen, bone marrow aspiration studies were done, which revealed no abnormality.

Immunohistochemistry showed that the cell stained positive to CD30, CD4, CD8, and anaplastic lymphoma kinase (ALK) 1 was faintly positive [Figure 6], [Figure 7], [Figure 8].{Figure 6}{Figure 7}{Figure 8}

This patient was referred to the medical oncologist who started him on multiagent chemotherapy consisting of cyclophosphamide, adriamycin, vincristine, and prednisolone.

The lesions regressed in size, and there was decreased discharge from the lesions after starting chemotherapy. However, the patient developed severe cytopenia, fever, and altered sensorium 1 month after completion of the first cycle of chemotherapy and he died a week later due to side effects of chemotherapy.


Primary CALCL is a rare lymphoproliferative disorder that mainly affects adults with a male to female ratio of 1.47:1 and with a mean age of onset of 52 years.

The clinical presentations range from solitary or localized lesions to multifocal nodules or tumors, some of which can be ulcerated and some regress spontaneously.[2] Multifocal lesions are seen only in 20% of cases.

Primary CALCL must be distinguished from anaplastic transformation of other cutaneous lymphomas, especially mycosis fungoides and cutaneous infiltration of systemic ALCL.[3],[4]

Transformed mycosis fungoides can be distinguished by the presence of patches or plaques for years and the histological presence of epidermotropism.

Systemic ALCL should be suspected if any extracutaneous site other than regional lymph nodes is affected.

Anaplastic lymphoma kinase + primary systemic ALCL frequently affects younger patients and is more responsive to chemotherapy, whereas ALK -ve primary systemic ALCL carries a poorer prognosis.

Histological examination of a lesion of CALCL typically shows a diffuse infiltrate composed of large cells with an anaplastic, pleomorphic or immunoblastic cytomorphology and expression of CD30 antigen by more than 75% of the tumor cells. This was demonstrated in our patient. The tumor cells were CD30+ and ALK1 was faintly positive. The neoplastic cells showed an activated CD4+ T cell phenotype. Unlike systemic CD30+ lymphomas, most CALCL express the cutaneous lymphocyte antigen, but do not express epithelial membrane antigen and ALK which indicate the 2,5 chromosome translocation or its variants.

The choice of treatment in CALCL is based on the size, the extent and the clinical behavior of the skin lesion. Solitary or localized nodules or plaques can be treated with radiotherapy or surgical excision.[3],[5] Patients with multifocal skin lesions or regional lymph node involvement may respond to systemic therapies such as methotrexate, systemic retinoids, or interferon-alpha. Single or multi-agent chemotherapy may be required in resistant cases.[6]

The standard chemotherapy for this disease consisted of CHOP regimen.

Combination chemotherapy was thought to be the most appropriate first line treatment of multifocal primary cutaneous disease with or without evidence of extracutaneous disease.[7] However, a study from Dutch lymphoma group found that patients treated with CHOP chemotherapy regimen universally experienced one or more relapses in the skin.[8] On the basis of it, the authors recommended that traditional combination chemotherapy should not be used for multifocal ALCL involving only the skin. This is particularly applicable to elderly patients as in our case in whom chemotherapy induced side effects are fatal.

The prognosis being excellent for CD30+ primary CALCL, with a 5 years survival rate of 90%[9] it may be better to go for radiotherapy or low dose methotrexate as the initial modality of treatment in multifocal skin restricted disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


Our sincere thanks to Dr. Uday Khopkar, Dermatopathologist, Mumbai, India.


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