A 61-year-old man presented with a 4-month history of a nonpruritic rash, which started on his forearm and face after sun exposure and subsequently spread to the ear, scalp and upper chest, after which asymptomatic skin lesions appeared on the lateral margin of his finger. The patient denied any history of photosensitivity but complained of low-grade fever and painful swelling of the joints in his wrist, knee and ankle, accompanied by restricted activity for 1 month. He had a sensation of a foreign body in his pharynx with difficulty in swallowing for about 1 month.
The patient also had a history of hepatitis B, which was diagnosed in the 1970's, and severe knee joint pain with bony spurs, which were confirmed by X-ray, for at least 2 years. There was no history of drug allergy, operation, trauma or inherited disease. Treatment with hydroxychloroquine and thalidomide for solar dermatitis in other hospitals showed no therapeutic effect.
Physical examination showed a confluent erythematous eruption affecting his scalp, face, neck and the extensor aspect of his forearm, which was characterized by ruby-red, translucent and grouped papules (fig. ) that were 0.5 mm to 1 cm in diameter. Red nodules could also be found on the dorsum of the nose, lateral area of the fingers and tongue tip (fig. ). Musculoskeletal examination revealed a painful swelling of the wrist and knee joints. The results of the clinical examination were otherwise unremarkable.
a Multiple fusion ruby-red papulonodular eruption in the neck, extensor aspect of the forearm and chest (sharply demarcated). b Light pink and shiny nodule on philtrum and tongue tip.
An extensive laboratory evaluation, including white cell count, urinalysis, stool studies, renal function, electrolytes, cholesterol and triglycerides, erythrocyte sedimentation rate, antistreptolysin O level, rheumatoid factor and antinuclear antibody were normal. Liver function tests including AST 34 U/l (<30 U/l), ALT 125 U/l (<50 U/l), ALP 159 U/l (35–104 U/l), GGT 100 U/l (3–45 U/l), total bilirubin 30 µmol/l (3.4–20.4 µmol/l), TBA 21 µmol/l (<6.8 µmol/l) and total protein, albumin and globulin were normal. Hepatitis index studies showed HBsAg(+), HBsAb <0.2 IU/l (>10 IU/l), HBeAb(+), HBsAb < HBeAg(–), HBeAb(+), HBcAb(+), HBcIgM(–), HAVIgM(–) and HCV(–). The AFP level was significantly elevated (6,820 µg/l, normal range <10 µg/l). Type-B ultrasonic examination revealed a solid tumor (91 × 69 mm) on the right lobe of the liver, which was suspected to be a small hepatocellular carcinoma.
Treatment with hydroxychloroquine and thalidomide were ineffective. The patient then received hepatic arterial chemoembolization followed by percutaneous transhepatic cholangiography drainage. Five-FU, Oxaliplatin and THP were used as anticancer drugs. The AFP level after the interventional treatment was 1,027.5 µg/l, and the tumor mass decreased to 67 × 66 mm. The cutaneous symptoms improved significantly within 1 month (several large nodules disappeared immediately; later, the erythematous rash and grouped papules resolved and the patient's arthralgia and swelling of joints gradually improved).
Skin biopsy on the right forearm showed infiltration of the dermis with polymorphic histiocytes and giant cells with ‘ground glass’ cytoplasm lying between collagen bundles (fig. ). The histiocytic marker CD68 was positive, as were leukocyte common antigen, HLA-DR, lysozyme and vimentin (fig. ). Langerhans cell markers (S-100 and CD1a) were negative and the histological appearances were consistent with MRH.
Fig. 2 a H&E section showing a prominent dermal histocytic infiltrate with typical ‘ground glass’ cytoplasm (×200). Skin samples obtained were studied by immunohistochemical staining. CD68 staining (b) and also Vim positive cells (more ...)