Folliculitis, follicular mucinosis, and papular mucinosis as a presentation of chronic myelomonocytic leukemia
Published Web Locationhttps://doi.org/10.5070/D30sw9f7xz
Folliculitis, follicular mucinosis, and papular mucinosis as a presentation of chronic myelomonocytic leukemiaDepartment of Dermatology, MD Anderson Cancer Center, UT Houston, Houston, Texas. Rrashid@mdanderson.org
Rashid Rashid MD PhD, Sharon Hymes MD
Dermatology Online Journal 15 (5): 16
Leukemias and Lymphomas can present in indolent and surprisingly unusual manners. Although uncommon, follicular lesions such as eosinophilic folliculitis have been reported in association with leukemia. However, follicular and papular mucinosis are novel associations for chronic myelomonocytic leukemia.
Leukemias and Lymphomas can present in indolent and surprisingly unusual manners. Although uncommon, follicular lesions such as eosinophilic folliculitis have been reported in association with leukemia . However, follicular and papular mucinosis are novel associations for chronic myelomonocytic leukemia.
A 74-year-old male presented to our clinic with a history of a rash that developed on his trunk and his chest. He noted pink, pruritic, small bumps, and flat areas that started to develop slowly over two to three months prior to his presentation at his primary care physician's office (one year prior to presentation in the dermatology clinic). He had not started any new mediations. Past medical history was noncontributory. He had a negative review of systems including weight loss, night sweats, and fevers. He originally went to see his primary care physician and had an initial work up and physical examination. The initial evaluation included a CBC and CMP. Abnormalities in his differential cell count led to further studies by a hematologist and the eventual diagnosis of chronic myelomonocytic leukemia as confirmed by bone marrow flow cytometry showing elevated myeloid cell lines with unremarkable B and T cell line numbers. Lymph node biopsy was negative. The eruption did not change with his initiation of chemotherapy (Fig. 1). During his chemotherapy, he also had a course of prednisone orally to control the pruritic papules, but this did little to change the appearance of the skin lesions. He was then referred to our clinic to determine if the skin changes were leukemia cutis. On exam, the lesions were on the trunk and neck and consisted of pink inter- and peri- follicular papules without pustules. They were slightly infiltrated and did not blanch. Biopsies were performed on an interfollicular and a perifollicular papule.
Biopsy results of the interfollicular papule showed increased dermal mucin, confirmed by a colloidal iron stain. There was minimal inflammatory infiltrate. The second biopsy showed folliculitis with follicular mucinosis. Furthermore, perivascular and interface dermatitis with eosinophils and increased dermal mucin were noted (Fig. 2). Colloidal iron staining showed an increase in dermal mucin and follicular mucinosis. Immunohistochemical studies performed on paraffin sections revealed that the majority of the cells of the inflammatory infiltrate were CD3 positive. CD4:CD8 ratio was 3:1 with no detection of T-cell receptor rearrangement. Myeloperoxidase (MPO) did not demonstrate involvement by leukemia.
Serum thyroid-stimulating hormone (TSH), ANA, and electrophoresis studies were unremarkable.
Follicular mucinosis can have varied clinical presentations. The classic description involves alopecia and dermal infiltration. An additional feature is occasional redness. Plaques typically occur on the head and neck, but they have been reported to affect all regions of the body. Follicular mucinosis is classified as a more common primary idiopathic disease and rare secondary forms are associated with lymphoma. In most presentations, the associated lymphoma is mycosis fungoides .
A literature search produced one case of follicular mucniosis as the presenting/associated sign of acute myeloblastic leukemia. However, this brief report highlights the novel association of folliculitis, follicular mucinosis, and papular mucinosis in association with chronic myelomonocytic leukemia . With such associations now in the literature, it is important to remain highly vigilant when managing such seemingly benign and non-specific skin changes. This is particularly true for perceived inflammatory lesions that are recalcitrant to standard treatments such as topical corticosteroids and prednisone. Fortunately, this patient was diagnosed with leukemia early due to his cutaneous presentation that precipitated the initial systemic and thorough evaluation by his primary care physician. Finally, we emphasize that this association may have been coincidental and one pathologic process may not actually have triggered the other. However, both are unusual diseases, and we must be especially vigilant in these situations.
References1. Patrizi A, Chieregato C, Visani G, Morrone P, Patrone P. Leukaemia-associated eosinophilic folliculitis (Ofuji's disease). J Eur Acad Dermatol Venereol. 2004;18(5):596-8. [PubMed]
2. Sumner WT, Grichnik JM, Shea CR, Moore JO, Miller WS, Burton CS. Follicular mucinosis as a presenting sign of acute myeloblastic leukemia. J Am Acad Dermatol. 1998;38:803-5. [PubMed]
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