176 Journal of the Royal Society of Medicine Volume 86 March 1993

Erosive in a patient with mycosis fungoides

D Schapira MD DSC1 H Kerner MD2 Y Scharf MD1 'The B Shine Department of and 2Department of Pathology, Rambam Medical Center, and The Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel

Keywords: mycosis fungoides; lymphoma; arthritis

Mycosis fungoides is a cutaneous T-cell lymphoma with Figure 2. Showing swelling of the left wrist and proximal possible lymph nodes and visceral organs involvement. interphalangeal joints and swan neck deformities of the fingers Synovitis is a very uncommon manifestation of this con- dition. The case ofa mycosis fungoides patient with erosive pruritus and an eosinophilia of 50%. The joint X-rays is described, and the pathogenesis of this revealed erosive destruction ofthe left carpus and the distal condition is discussed. interphalangeal joints (Figure 1). Total skin electron beam therapy resulted in temporary Case report disappearance ofthe skin rash and in partial alleviation of A previously healthy 64-year-old woman presented in the joint complaints. Since then, sporadic exacerbations of October 1979 with a 3-month history of shoulders and hips the cutaneous disease are controlled by steroid treatment. , morning stiffness and arthritis of the right In spite of specific treatments (imuran, methotrexate, . The general examination and the X-rays were not endoxan), deformities ofthe fingers (Figure 2) and functional contributory. Forty millilitres of cell-rich sterile fluid was disability progressively appeared. evacuated from the inflamed knee joint. The erythrocyte sedimentation rate (ESR) was 60 mm/h (Westergren). Discussion Rheumatoid and antinuclear factors were negative. Steroid Mycosis fungoides is an uncommon T-cell lymphoma ofthe treatment brought a rapid improvement of the symptoms. skin which may affect lymph nodes and visceral organs1. In August 1981 the patient was hospitalized for diffuse Generalized erythroderma, parapsoriasis, eczematous arthralgia, exacerbation of the right knee arthritis, severe plaques, and intense pruritus are the main features of morning stiffness, a weight loss of 7 kg and continuous cutaneous involvement. At the beginning ofthe disease the during the last 5 months. Extensive investigations failed to histology of non-specific and definite diagnosis may be reveal malignancy, infection or a specific inflammatory joint achieved only after several years. disease. One month later diffuse maculopapular skin rash The extent of the skin involvement and the presence of together with intense pruritus appeared. Skin biopsies are important prognostic factors. Anaemia, showed non specific dermatitis, and bone biopsy disclosed leucocytosis and eosinophilia are frequent laboratory numerous eosinophils in the bone marrow. Treatment with features. Abnormal liver function discloses hepatic involve- 40 mg meticorten a day was commenced. The cutaneous and ment'. the articular symptoms and signs withdrew, but they Arthritis is a rare manifestation of mycosis fungoides, intensified each time the storage dosage was lowered. as shown by the few documented cases in the relevant In 1983 the histological examination of the skin was literature25. In most cases it is seronegative and involves consistent with mycosis fungoides, plaque stage. At that time the appendicular skeleton, but associated clinical sacroiliitis , swelling and progressive limitation of the wrists and was also described4. The chronic, sometimes deforming of the small joints of the hands and feet were reported symmetric polyarthritis2, the eventual subcutaneous together with the exacerbation of erythroderma and nodules5 and keratoconjunctivitis sicca4 and the morning stiffness may mimic . However, in the presence of a definite diagnosis of mycosis fungoides, the correlation between the exacerbation ofthejoint symptoms and the flares of skin disease2, the presence ofT helper cells in the synovial fluid4 and the histological incompatibility of the nodular tissue with that of rheumatoid nodules6 associate the synovitis to the T-cell lymphoma. The character of this connection has yet to be elucidated. Malignant infiltration of the synovium was reported in T-cell chronic lymphocytic leukaemia, a condition in which circulating lymphocytes have unclear irregularities similar to those of the proliferating T cells of Sezary syndrome and mycosis fungoides6. Nevertheless, there is no evidence of malignant infiltration ofthe synovial tissue in mycosis fungoides, the synovitis being considered as a reactive phenomenon to the involvement of the adjacent bone marrow or skin5. This hypothesis is supported by the prolonged remission of both dermatitis and synovitis following electron beam therapy. Figure 1. Roentgenogram ofthe hands showing destruction ofthe left Besides having a direct effect on the superflcial structures, carpus, basal erosion of the fourth right metacarpal bone (arrow), this treatment induces lymphocytopenia leading to a systemic immunosuppressive effect2. Correspondence to: Dr D Schapira, The B Shine Department of Osteolytic lesions have been rarely reported in mycosis Rheumatology, Rambam Medical Center, POB 9602, 31096 Haifa, fungoides7-12. They are caused by the marrow infiltration or Israel by direct invasion of the bone tissue by tumour cells. Journal of the Royal Society of Medicine Volume 86 March 1993 177

To our knowledge this is the first report of erosive arthritis 6 Van Soesbergen RM, Feltkamp-Vroom TM, Feltkamp CA, in mycosis fungoides. Somers R, Van Beek WP. T-cell leukemia presenting as chronic polyarthritis. Arthritis Rheum 1982;25:87-91 7 Greer KE, Legum LL, Hess CE. Multiple osteolytic lesions in References a patient with mycosis fungoides. Arch Dermatol 1977;113: 1 Rappaport H, Thomas LB. Mycosis fungoides: the pathology of 1242-4 extracutaneous involvement. Cancer 1974;34:1198-229 8 McCormick CC. Case report 20. Skel Radiol 1977;1:183-4 2 Gottlieb M, Hoppe RT, Colin A, Strober S. Arthritis in a patient 9 O'Reilly GV, Clark TM, Crum CP. Skeletal involvement in with mycosis fungoides. Complete remission after radiotherapy. mycosis fungoides. Am J Roentgenol 1977;129:741-3 Arthritis Rheum 1979;22:424-5 10 Carney DH, Bunn PA Jr. Manifestation of cutaneous T-cell 3 Vallina E, Navia JM, Carton JA, Diaz J, Arribas JM. Arthritis lymphoma. J Dermatol Surg Oncol 1980;6:369-405 y mycosis fungoide, una asociacion exceptional. Communication 11 Matsumoto K, Takigawa M, Oku T, Iwatzuki K, Imaizumi S, y comentaries sobre un caso. Rev Clin Espanola 1984;172:235-6 Yasmara M. Giant ulcerated tumor and bone destruction in a 4 Berger RG. Mycosis fungoides with polyarthritis. Arthritis Rheum patient with mycosis fungoides. Arch Dermatol 1986;122:135-6 1988;31:1335-6 12 Metzger Von H, Kurtz B, Ahlemann L. Osteolytische knochen- 5 Seleznick M, Aguilar JL, Rayhack J, Fenske N, Espinosa L. manifestationen bei mycosis fungoides. RoFo 1980;133:331-3 Polyarthritis associated with cutaneous T cell lymphoma. J Rheumatol 1989;16:1379-82 (Accepted 5 November 1991)

Linear sebaceous naevus syndrome LwJ with oncogenic rickets and diffuse C leltrlol L _ _ _ Phophate pulmonary angiomatosis 1.Sg/d.

SERUM 1.5

1.0 E M O'Neill MD FRCP Department ofPaediatrics, District General Hospital, Barnsley S75 2BR 3.0 SERUM Ca 2.5 I Keywords: oncogenic rickets; linear sebaceous naevus syndrome; 2.0 diffuse pulmonary angiomatosis SERUM ALK. R

PHOSPH4ATASE~ \ ~ I I Oncogenic osteomalacia is recognized in adults. Oncogenic rickets is less well Dn.~ Dec. Dec. known. This case also had diffuse 1977 1078 1079 1980 1981 pulmonary angiomatosis and the linear sebaceous naevus syndrome (LSNS), all of which were probably interrelated. Figure 2. Results of tests for calcium, phosphorus and alkaline phosphatase (R, rickets; Hg, healing; H, healed rickets) Case report The patient, a girl was born on 5 March 1974. The family Vitamin D prophylaxis had been regular but she walked history was negative and pregnancy and delivery normal. late and rickets was diagnosed at 3 years when 2,300 IU Birth weight 3.43 kg. There were pigmented and verrucous vitamin D per day for 9 weeks failed to assist healing. naevi of the face, head and neck with a limbal dermoid All further tests then were normal apart from calcium, (Figure 1) but no other anomalies. phosphorus and alkaline phosphatase (Figure 2). Family screening for rickets was negative. On vitamin D 50,000 IU per day with phosphate 1.5 G per day there was good healing in 4 weeks and complete healing in 5 months. After an episode of hypercalcaemia calcitriol (1,25(OH)2D3) was introduced then maintained at 0.25 Ag per day. At 4½ years there was dyspnoea with a pericardial effusion. Diffuse pulmonary haemangiomatosis' was diagnosed and lung histology showed capillary haemangiomata. The peri- cardium was drained of brown fluid followed by partial pericardectomy. Later a left pleural effusion was drained of pink fluid with separating white supernatant and talc - m --~~~~ ~ ~~~~~~~~~~~~~~~~~..was insufflated. Six months later she was no longer dyspnoeic but had left pleural thickening and cardiomegaly. She continued well until 6 years when dyspnoea returned with a pleural effusion and mediastinal pain. The effusion was drained with pain relief and slight residual dyspnoea. At 7% years Figure 1. Extensive pigmented and verrucous naevi at birth pneumonia and heart failure caused death. present There was never chronic cough or haemoptysis. During investigation of the generalized rickets bone cysts throughout the left upper limb and tenth rib were Correspondence to: Dr E M O'Neill, 5 Amberley Gardens, Bedford found. These remained static and were angiomata radio- MK40 3BT logically.