|Year : 2014 | Volume
| Issue : 2 | Page : 79-82
Different faces of cutaneous Hodgkin's disease
Shikha Goyal1, Ahitagni Biswas1, Tarun Puri1, Ruchika Gupta2, Sandeep Muzumder1, Pramod Kumar Julka1
1 Department of Radiotherapy, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||18-Dec-2014|
Department of Radiotherapy and Oncology, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Hodgkin's disease with skin involvement is a rare entity with a reported incidence of 0.5-3.5%. Various routes of skin involvement may include retrograde lymphatic spread, direct extension from underlying nodes, or hematogenous spread. Prognosis is usually determined by the degree of visceral involvement and the stage of disease. We report our experience with four cases of Hodgkin's disease with varying presentations, mimicking other malignant or inflammatory conditions.
Keywords: Cutaneous infiltration, Hodgkin′s lymphoma, systemic chemotherapy
|How to cite this article:|
Goyal S, Biswas A, Puri T, Gupta R, Muzumder S, Julka PK. Different faces of cutaneous Hodgkin's disease. Indian J Dermatopathol Diagn Dermatol 2014;1:79-82
|How to cite this URL:|
Goyal S, Biswas A, Puri T, Gupta R, Muzumder S, Julka PK. Different faces of cutaneous Hodgkin's disease. Indian J Dermatopathol Diagn Dermatol [serial online] 2014 [cited 2019 Oct 17];1:79-82. Available from: http://www.ijdpdd.com/text.asp?2014/1/2/79/147310
| Introduction|| |
Skin involvement is a clearly recognized phenomenon in non-Hodgkin's lymphomas (NHL), but it is extremely uncommon in Hodgkin's lymphoma.  The first description of specific cutaneous lesions of Hodgkin's disease (HD) was given by Grosz in 1906. Subsequent literature reports less than 50 cases with cutaneous involvement in various patterns.  We describe the clinical characteristics of four patients with specific cutaneous involvement.
| Case Reports|| |
A 50-year-old woman presented with bilateral neck masses and mild to moderate fever of 1 month duration. There were no symptoms referable to the aerodigestive tract. The patient did not give any history of exposure to Koch's. There was no significant personal or family history. On examination, she had bilateral cervical lymph nodes, level III-IV, each 4 × 4 cm, with extension to overlying skin causing fungation and necrotic areas [Figure 1]a and 1b]. Other lymph node stations, oral examination, triple endoscopy, and systemic examination were normal. Her complete blood counts, liver and renal function tests were normal. Wound swab cultures from the ulcerating nodes were negative for bacteria and acid-fast bacilli. Abdominal imaging showed splenomegaly with multiple focal splenic deposits. Fine needle aspiration cytology from the cervical lymph nodes was equivocal. Trucut biopsy revealed a diagnosis of HD with a polymorphous population of lymphoid cells, occasional eosinophils, and atypical large mononuclear as well as binucleate cells. Characteristic Reed-Sternberg cells could be identified [Figure 1]c]. Bone marrow aspiration and biopsy returned negative. She was diagnosed to have HD, stage IVB, and was planned for chemotherapy with ABVD regimen (Adriamycin 40 mg/m 2 D1, D15; Bleomycin 10 IU/m 2 D1, D15; Vinblastine 6 mg/m 2 D1, D15; and Dacarbazine 375 mg/m 2 D1, D15) every 4 weeks for a total of six cycles. Follow-up whole body positron emission tomography-contrast-enhanced computed tomography (PET-CECT) showed complete regression of neck nodes as well as splenic lesions. She received involved field radiotherapy to bilateral neck (30.6 Gy in 17 fractions) and is free of recurrence at a follow-up of 3 years.
A 21-year-old man presented with fever and an ulcerated sternal swelling for 6 months [Figure 2]a]. He had received a trial of anti-tubercular therapy with a presumptive diagnosis of osseous Koch's considering the prevalence of tuberculosis in our country, but did not improve. Imaging showed soft tissue swelling involving the sternum and presternal skin [Figure 2]b]. Multiple focal hypodense lesions were seen in liver and spleen. Sternal mass biopsy showed fibrosis, numerous eosinophils, and large atypical cells and occasional binucleate cells, with the large atypical cells showing abundant cytoplasm, large nucleus, prominent eosinophilic nucleolus, and perinuclear positivity for CD30. Overall features suggested HD [Figure 2]c and 2d]. Bone marrow was uninvolved. He received six cycles of ABVD regimen with a diagnosis of HD stage IVB, with partial response. Consolidation radiotherapy to the sternal lesion resulted in complete resolution; however, there was recurrence in cervical region within 6 months of treatment, for which second-line chemotherapy was planned.
A 25-year-old lady was evaluated for complaints of anterior chest wall swelling, fever, and night sweats for 1 year. Clinical examination showed left parasternal swelling (5 × 5 cm) extending to left breast skin and left supraclavicular lymphadenopathy (2 × 1 cm) [Figure 3]a]. Two consecutive biopsies from sternal lesion showed sheets of eosinophils, strongly suggesting eosinophilic granuloma though immunohistochemistry (IHC) for S-100 was noncontributory [Figure 1]. Left cervical lymph node biopsy showed similar morphology and a diagnosis of Langerhans cell histiocytosis (LCH) was given. She received low-dose radiotherapy and three cycles of vinblastine. She initially showed good response, but soon developed bulky cervical and axillary lymphadenopathy. CECT chest and abdomen showed sternal body thickening with lytic lesion, multiple bilateral axillary, left supraclavicular, prevascular, and anterior diaphragmatic lymph nodes, and ill-defined focal splenic lesions with few splenic hilar lymph nodes. Axillary lymph node biopsy showed HD nodular sclerosis, British National Lymphoma Investigation (BNLI) type II [Figure 3]b and 3c]. IHC was positive for both CD30 and CD15, and CD30 alone in the biopsy specimens from cervical and axillary lymph nodes, respectively. Previous biopsy specimens were reevaluated and showed atypical cells. IHC was performed on these specimens as well, corroborating the diagnosis of HD masquerading as eosinophilic granuloma. Repeat bone marrow examination was normal. Previous biopsies were reviewed and the initial diagnosis revised. She received eight cycles of ABVD with complete clinical and radiological remission.
A 28-year-old male presented to our clinic with bilateral cervical swellings, moderate fever, weight loss > 10% over 4 months, and skin rash. There was a diffuse folliculitis-like centrifugal rash over arms and legs [Figure 4]a]. Dermatology consultation was sought and the patient was given a course of antibiotic therapy, but the rash persisted. Cutaneous biopsy taken twice was inconclusive. The skin biopsy findings included wedge-shaped, superficial, and deep mixed infiltrate of small lymphocytes, atypical lymphocytes, and eosinophils, but no CD30 positivity in the atypical cells. Cervical node biopsy showed classic HD, with atypical mononuclear and binucleate cells, immunopositive for CD30 [Figure 4]b]. Systemic workup showed hepatosplenomegaly without any focal lesions and lymphoma deposits in bone marrow biopsy. He was diagnosed to be a case of HD stage IVB, and started on ABVD regimen. Following two cycles of chemotherapy, he showed clinical complete remission and complete disappearance of the skin rash. He was given ABVD chemotherapy for a total of eight cycles, and is now disease-free after a follow-up of 2.5 years.
| Discussion|| |
Skin involvement in HD can be either in the form of non-specific problems such as pigmentation, pruritus, or ichthyosis, or in the form of specific cutaneous lesions, which may take a variety of forms such as papules, nodules, infiltrative plaques, ulcerative lesions, a combination of these, or erythroderma.  White and Patterson reported a series of 16 patients with cutaneous involvement, comprising 3.4% of patients with HD seen at their institute over a 30-year period. They proposed three mechanisms for cutaneous involvement - retrograde lymphatic spread (11/16), direct extension from an underlying focus (3/16), or hematogenous dissemination (2/16).  Extensive skin involvement may suggest advanced disease with poor prognosis, while limited involvement may respond well to institution of early therapy. , Isolated cutaneous involvement in the absence of systemic disease has also been reported and has a particularly indolent course.  Occasionally, HD may relapse with cutaneous lesions.  Cutaneous vasculitis as a paraneoplastic syndrome, that responds well to antineoplastic therapy, is another phenomenon. 
Morphologically, the cutaneous lesions are indistinguishable from nodal involvement, with constant CD30 and CD15 positivity and CD45 negativity. 
Three of our cases had skin involvement secondary to contiguous spread from underlying node or soft tissue, representing the commonest form of specific cutaneous involvement, while the etiology in the fourth, whether hematogenous or paraneoplastic, could not be discerned. Despite being diagnosed as stage III or IV disease, all four patients responded well to ABVD, though long-term follow-up is unavailable to conclusively comment on eventual prognosis.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]