Papules, Arthralgia, Dry Mouth and Dry Eyes: A Quiz
Hee Dam Jung, Jong Ho Lim, Hei Sung Kim, Jun Young Lee, Hyung Ok Kim and Young Min Park*
Department of Dermatology, College of Medicine, Seoul St Mary’s Hospital, The Catholic University of Korea, 505 Banpo-dong, Seocho-ku, Seoul 137-040, South Korea. *E-mail: firstname.lastname@example.org
A 59-year-old Korean woman presented with asymptomatic multiple papules on the superior aspect of her ears, the posterior neck and proximal nail-folds of her right thumb and right 3rd and 4th fingers for one month (Fig. 1). One month earlier, she visited her GP with a 2-month history of symmetrical polyarthralgia involving the wrists, fingers, shoulders and knees. She was treated with prednisolone, gabapentin and non-steroidal anti-inflammatory drugs for one week with no improvement. She also developed symptoms of dry mouth and dry eyes. She was transferred to the Department of Rheumatology for further evaluation. The results of laboratory examination, systemic lupus erythematosus (SLE) profile studies, radiological examination, ultrasound-guided arthrocentesis, Schirmer’s test, salivary gland scan and lip biopsy were not compatible with rheumatoid arthritis or Sjögren’s syndrome.
The patient was transferred to our clinic for the evaluation and we performed a skin biopsy of a yellow-red papule on the right ear (Fig. 1C).
Fig. 1. Asymptomatic, multiple, 2–3-mm diameter, yellow-to-red papules on: (A) the superior aspect of the ears, and posterior neck (inset), (B) proximal nail folds of the right 3rd and 4th. (C) The biopsy specimen revealed a diffuse infiltration of numerous large, mononucleated or multinucleated histiocytes in the dermis (haematoxylin and eosin ×100).
What is your diagnosis? See next page for answer.
Papules, Arthralgia, Dry Mouth and Dry Eyes: Comment
Acta Derm Venereol 2012; 92: XX–XX (contd.)
Diagnosis: Multicentric reticulohistocytosis
Multicentric reticulohistiocytosis (MRH) is a well-documented disease of unknown aetiology, which belongs to the group of non-Langerhans’ cell histiocytosis. It is characterized by cutaneous or mucosal papular lesions with severe polyarthritis and arthralgias. Although the commonly affected sites are the skin and joints, involvement of the mucosa, muscles, cardiopulmonary system, eyes, gastrointestinal system, thyroid and salivary glands have been reported (1). In addition, a number of conditions, such as rheumatological disorders, diabetes, thyroid disease, tuberculosis and malignancies, have been reported to be associated with MRH (2–6). Cutaneous lesions of MRH usually present as diffusely scattered, asymptomatic, firm, smooth, yellow-to-red papules or nodules on the face, head, elbows and hands. However, the initial clinical findings of MRH can be diffuse symmetrical polyarthritis with arthralgia, and can therefore be misdiagnosed as rheumatoid arthritis (7). In addition, some extraordinary symptoms of MRH, such as xerostomia and dry eyes, may cause confusion with Sjögren’s syndrome, as shown in our case. Therefore, careful history-taking, physical examination and histopathological evaluation of the cutaneous lesions are required for a definite diagnosis of MRH.
Treatment of MRH is not established, but reports of remission with immune-suppressants have been published. Tumour necrosis factor-α inhibitors, including etanercept, infliximab and leflunomide, have shown good results in recalcitrant cases of MRH (8–10). Our patient has been well managed with a combination therapy of prednisolone (starting with 8 mg/day, and gradually tapering to 2.5 mg/day), COX-2 inhibitor (400 mg/day), hydroxychloroquine (400 mg/day) and methotrexate (7.5 mg, once a week). The patient’s arthralgia disappeared 2 months after treatment, but there was only a slight improvement in the cutaneous lesions. Extensive investigation did not reveal any underlying malignancy.