DermUtopia, Fairfax, Virginia
History and physical:
This is a 75-year-old man who was referred to our practice by his
allergist for a pruritic rash on the legs and arms. The patient reported that
he had first developed a rash on his trunk in July of 2021 following three
courses of amoxicillin. At that time, it was described as bright red to
purplish in color, with associated fluid-filled vesicles and pruritus. The rash
was treated with a topical steroid cream and resolved within 10 days.
In February 2022, the patient began to develop purpuric, pruritic patches and
urticarial wheals on his ankles, calves and forearms that progressively worsened
and seemed to be aggravated by heat and friction from clothing. The patient had
tried multiple courses of topical steroids without response. He was given
several short courses of oral prednisone by his primary care doctor and
although the rash resolved while he was on prednisone, it would return and
progressively worsen as soon as the course was complete. He denied any
constitutional symptoms such as fever, chills, cough, lymphadenopathy, night
sweats, abdominal pain, or weight loss.
Assessment:
The patient initially presented to our practice with a subtle erythematous eruption on the arms and legs and evidence of post-inflammatory pigmentation. Subsequently, he returned with a worsening violaeous, painful rash on his lower extremities and red indurated nodules on his arms just three days after his first visit:
The patient’s differential included drug reaction, vasculitis, hypereosinophilic syndrome, bullous pemphigoid, or lymphoproliferative syndrome. Biopsies were taken from active lesions, and lab work including CBC, CMP, SPEP, RF, ANA, and bullous pemphigoid antigen was ordered. He was also referred to rheumatology and oncology for further investigations.
Results:
Lab results were remarkable for eosinophilia (7.2%), elevated C-reactive protein (5.2), and a highly positive rheumatoid factor (>1000). The patient had a negative ANA, negative bullous pemphigoid antigen, and a normal SPEP.
Pathology:
4x. The histologic sections show a superficial and deep infiltrate that extends into subcutis and includes hemorrhage within the dermis and subcutis.
20x superficial dermis. Within the upper dermis, the infiltrate is seen to include numerous lymphocytes, neutrophils, and eosinophils. Hemorrhage is present. No leukocytoclastic vasculitis is identified.
Diagnosis and Treatment:
The patient’s primary
care doctor ordered a CT of the abdomen which showed retroperitoneal
lymphadenopathy in the upper pelvis. Subsequently, a PET scan showed widespread
low-grade adenopathy above and below the diaphragm.
The patient underwent a
bone marrow biopsy and inguinal lymph node biopsy, which were consistent with
CD5-negative mantle cell lymphoma (MCL). He had stage IV disease. He was
started on a course of rituxan for his lymphoma, and he was continued on
systemic corticosteroids to manage his rash.
Discussion:
This case is an example
of a patient with an indolent recurring skin eruption which required a
multidisciplinary effort to correctly diagnose and manage. He had a negative workup
by an allergist prior to being evaluated by dermatology and being referred to
oncology and rheumatology. It is also interesting to note that he had few to no
constitutional symptoms preceding his skin eruption and diagnosis with MCL.
One review of the
literature estimates that only 2-10% of patients with MCL have cutaneous
disease, most often caused by involvement of the lymphoma in the skin. This
case, where biopsies did not show involvement of the lymphoma in the skin but
rather suggested a paraneoplastic reaction, seems to be even more rare. We could
only find only one similar case report in PubMed. In that case, therapy with
rituximab was successful in treating the patient’s eruption.
References:
1. de Souza PK, Amorim RO, Sousa LS, Batista MD. Dermatological manifestations of hematologic neoplasms. Part I: secondary specific skin lesions. An Bras Dermatol. 2023;98(1):5-12. doi:10.1016/j.abd.2022.06.002
2. de Souza PK, Amorim RO, Sousa LS, Batista MD. Dermatological manifestations of hematologic neoplasms. Part II: nonspecific skin lesions/paraneoplastic diseases. An Bras Dermatol. 2023;98(2):141-158. doi:10.1016/j.abd.2022.08.005
3. Geropoulos G, Psarras K, Vlachaki E, et al. Cutaneous manifestations of mantle cell lymphoma: an extensive literature review. Acta Dermatovenerol Alp Pannonica Adriat. 2020;29(4):185-191.
4. Nemets A, Ronen M, Lugassy G. Chronic paraneoplastic cutaneous syndrome preceding an indolent variant of mantle cell lymphoma: favorable response to rituximab. Acta Haematol. 2006;115(1-2):113-116. doi:10.1159/000089477
