DOI: 10.1097/RHU.0000000000000270

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PMID: 27219314

Issn Print: 1076-1608

Publication Date: 2016/06/01


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Nailfold Capillary Patterns in a Patient With Multicentric Reticulohistiocytosis and Raynaud Phenomenon

Hèctor Corominas; Johan Villareal; Paula Estrada; Daniel Roig-Vilaseca; Vicenç Torrente-Segarra; Delia Reina; Dacia Cerdà-Gabaroi; Silvia García-Diaz; David Vidal

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A 72-year-old woman has been followed up in our rheumatology clinic since 2012. Her medical history included breast cancer 17 years before with mastectomy, lymphadenectomy, surgical intervention postradiotherapy, and treatment with tamoxifen for 7 years and uterus cancer with hysterectomy 5 years before. She reported mild joint pain in her hands, without arthritis, together with cutaneous papules on both hands and in the nasal septum as well as severe Raynaud phenomenon (RP). Laboratory studies showed cholesterol 6.20 mmol/L, low-density lipoprotein 4.16 mmol/L, C-reactive protein 4.5 mg/L, and β2-microglobulin 2.59 mcg/ml. Hematologic parameters and acute phase reactants were within reference ranges. The immunological study disclosed a speckled pattern of antinuclear antibody 1/320, whereas tests for SCL-70, DNA, and rheumatoid factor were all negative. Radiographic studies of the hands revealed joint space narrowing in both wrists and, in distal interphalangeal joints, mutilating arthritis, telescoping shortening of digits, “doigts en lorgnette,” and opera-glass fingers, somewhat mimicking a seronegative erosive arthritis. Skin biopsies were taken from the papulonodular lesions at the back of the hands and nostrils (Fig. 1), which confirmed the suspicion of multicentric reticulohistiocytosis (MRH).1,2 A computed tomography scan of the lung was obtained and showed irregular increased density in the left breast. Multiple pulmonary micronodules suspected of metastases were also observed.
Our patient presented severe RP and showed nailfold capillaroscopic3–5 findings that included decreased capillary density, abundance of bushy capillaries, pericapillary hemorrhages, and neoangiogenesis suggesting proliferation phenomena, without visibility of the subpapillary venular plexus, tortuosities, or megacapillaries (Fig. 2). There was no consistent relation of the nailfold changes to the skin lesions.
To our knowledge, this is the first description of the nailfold capillaroscopic findings in a patient with MRH and RP. Further observations are needed to see if capillaroscopic changes occur in MRH without RP.
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