Cutaneous Involvement in Hodgkin Lymphoma: A Rare Case Report.

Sir,

Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy,[1] clinically characterized by nodal infiltration, and typically involves the cervical, mediastinal, and axillary regions. While the disease most commonly involves contiguous lymph node groups, in rare cases, it may also affect extranodal tissues. Cutaneous involvement in HL is extremely rare.[12] It may present as specific or nonspecific manifestations of the disease.[3] Here, we present a peculiar clinical case that illustrates the infrequent coexistence of both specific and nonspecific skin lesions of Hodgkin disease in an uncommon localization.

A 52-year-old previously healthy woman presented with a 3-month history of fever, malaise, fatigue, night sweats, and weight loss.

Physical examination revealed bilateral inguinal lymphadenopathy and severe xerosis cutis, with thick brown scales predominantly on the extensor surfaces.

Computed tomography scans revealed several enlarged lymph nodes in the supra and infradiaphragmatic regions. Biopsy of the left inguinal adenopathy showed morphological and immunohistochemical results compatible with classical HL of the nodular sclerosis subtype. Bone marrow biopsy did not demonstrate lymphoma infiltration.

The patient was diagnosed with advanced HL (Stage IIIB) and received the first adriamycin, bleomycin, vinblastine, and dacarbazine (AVBD) chemotherapy cycle. Later, the patient evolved to erythroderma and multiple painless papules, nodules, and infiltrated rounded plaques with slightly raised borders, ranging from 3 mm to 1.5 cm in diameter, distributed over the pubis [Figure 1].

Figure 1

Multiple papules, nodules, and plaques with yellowish centers located over the pubis, vulva, and proximal left thigh area

Punch biopsies performed on the pubic lesions and the erythrodermic skin showed a dermal mixed inflammatory cell infiltrate combined with atypical large lymphoid cells with large uni- or bi-nucleated “mirror image” nuclei and prominent eosinophilic nucleoli [Figure 2a and b]. The large neoplastic cells were positive for CD30 (with membrane positivity and paranuclear dot staining pattern) and PAX-5, which weakly stained the nuclei of the atypical cells, but were negative for CD20 and CD15.

Figure 2

(a) Histopathologic examination of a punch biopsy specimen from a pubic lesion showing mixed inflammatory cell infiltrate, including numerous reactive lymphocytes, histiocytes, and scattered eosinophils, that are mixed with large atypical lymphoid cells in the dermis (hematoxylin and eosin stain, ×10). (b) High-power view, demonstrating the presence of Reed–Sternberg cells (continuous arrow) and Hodgkin cells (dotted arrow) (hematoxylin and eosin stain, ×40)

The clinical suspicion of cutaneous involvement of HL was confirmed and the patient was reclassified as stage IV BD but declined further treatments and died 3 months after the diagnosis.

The cutaneous manifestations of HL have been divided into two groups[4]: specific lesions, reported to occur in 0.5%–3.4% of HL patients,[3] in which direct infiltration of the skin by malignant cells is observed; and nonspecific/paraneoplastic lesions, which are much more frequent, but do not reflect cutaneous infiltration of the tumor.[4] The nonspecific cutaneous involvement has many presentations, including hyperpigmentation, pruritus, prurigo, alopecia, and acquired ichthyosis.[3]

Specific cutaneous manifestations of HL typically tend to affect the trunk and most frequently occur secondary to retrograde lymphatic spread from a proximal nodal focus.[35] These lesions may present as 1) papules, 2) plaques, 3) nodules or tumors, 4) ulcerative lesions, 5) combinations of these, and/or 6) erythrodermia.[45]

In the present case, our patient presented with acquired ichthyosis and later evolved to a combination of lesions associated with erythroderma, exhibiting the rare coexistence of both groups of manifestations at a very uncommon localization.

Cutaneous involvement in HL usually represents Stage IV Hodgkin's disease and often portends an ominous prognosis.[45]

Timely recognition of these lesions represents a clinical challenge owing to the wide variety of presentations.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.