Unusual Periungual Nodulocystic Lesions Leading to the Diagnosis of Extranodal Marginal Zone Lymphoma.

Sir,

We report a 91-year-old female who was examined for a 6 month lasting periungual nodulocystic lesions with dystrophic nails affecting several digits of the upper extremities [Figure 1a]. Lesions were painful. Patient was on oral antidiabetics and antihypertensive medication, otherwise she was healthy. She had been treated by another dermatologist with systemic itraconazole with the suspicion of candidal paronychia with no effect. General blood count was normal. Biochemistry examination revealed slightly elevated uric acid. We consulted a rheumatologist who excluded gout and periungual lesions as tophi. General physical examinations including chest X-ray and abdomen ultrasound were normal. We performed a punch biopsy with the result of extranodal marginal zone B-cell lymphoma [Figure 2a and b]. The patient had no systemic symptoms of lymphoma. She was immediately examined by a hematologist. Lymphoma cells were found in microscopy of peripheral count examination by the hematologist and we concluded that the skin lesions were secondary cutaneous manifestations of systemic B-lymphoma. Because of the age of patient, no further tests were performed to examine if any other organs were affected (gastric mucosa, colon, etc). The serology for Helicobacter pylori and Borrelia burgdorferi were negative. The patient declined a bone-marrow examination. The systemic therapy with repeated cycles of dexamethasone and chlorambucil was initiated with excellent effect to the skin lesions. After 2 months, almost complete regression was observed [Figure 1b].

Figure 1

(a) Nodulocystic periungual lesions of the digits of the upper extremities. (b) Regression of the lesions after systemic therapy

Figure 2

Histopathological findings. Diffuse dermal infiltrate composed of small CD20+ B lymphocytes. (a) (H and E, x40); (b) (Anti CD20 immunohistochemistry, x200)

Extranodal marginal zone lymphoma (EMZL) is a sub-entity of marginal zone lymphoma (MZL), which is a mature B-cell lymphoma and represents a distinct clinicopathological entity of non-Hodgkin's lymphoma (NHL).[1] It was first described by Isaacson and Wright in 1983.[2] EMZL is most frequently found in the stomach, lung, thyroid, skin, lacrimal, and salivary glands. It represents approximately 5% of NHL.[3] This type of marginal lymphoma usually has an indolent course, and patients may remain long-term asymptomatic as was the case of our patient. When skin lesions are present, it is very important to distinguish if the patient has primary cutaneous marginal zone lymphoma (PCMZL) or secondary cutaneous marginal zone lymphoma (SCMZL). PCMZL usually has a very indolent course in contrast to other marginal zone lymphomas, such as the nodal one. Patients with SCMZL tend to be older than patients with PCMZL, and in addition, there is a different localization of the lesions. SMZL was found more often in the area of head and neck.[4] In general, secondary skin lesions are not so common. The case of our patient is interesting because of the localization of the skin lesions, which were observed in periungual region. A case with similar clinical picture has been described in the literature, but the hematological diagnosis was small lymphocytic lymphoma, and it was known before the skin lesions appeared.[5] Our case is important because the skin lesions were the primary symptom of the systemic lymphoma. The therapeutic options of EMZL depend on the stage, localization, and symptoms of the disease. Asymptomatic patients may be followed with no treatment, otherwise radiotherapy or systemic treatment may be required.[4]

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

This work was supported by PROGRES Q28 (Oncology), research program awarded by the Charles University.

Conflicts of interest

There are no conflicts of interest.