Sarcoidosis characterized as acquired ichthyosiform erythroderma

ARTICLE

Auteur(s) : Hong Zhang¹, Hui-Jun Ma², Wen Liu¹, Xiao-Ying Yuan¹

¹Department of Dermatology, The Airforce General Hospital of Chinese PLA, Beijing, China
²Department of Dermatology, The second affiliated Hospital of the Chinese PLA General Hospital, A 17 Heishanhu Road, Haidian District, Beijing, 100091, P.R. China

A 27-year-old female presented with flushing, swelling and dry scaling lesions over the whole body for 8 months and suffering from subcutaneous nodules in the neck, trunk and limbs for 3 months. Dermatological examination revealed some 2-3 cm soft subcutaneous nodules diffusely in the right neck, shoulder and back. Three to five solitary firm, mobile, non-tender bilateral axillary and inguinal lymph nodes were found. Flushing, swelling and dry scaling of the skin were seen throughout the body (figure 1) with ichthyotic skin lesions seen especially on the arms and legs (figure 1B). No abnormalities of the eyes or joints were found. The patient was diagnosed without biopsy in the local hospital 5 months previously as having ichthyosiform erythroderma. However, the condition did not improve after oral acitretin 30 mg/d for 1 month. No family history of similar clinical features was reported.

Laboratory findings were as follows: complete blood cell count, serum calcium, liver function test were within normal limits; 24 h urine protein (1184, normal 0-200 mg/24 h); serum creatine kinase (24, normal 26-140 U/L), blood urea nitrogen (8.9, normal 2.5~7.5 mmol/L), blood glucose (3.2, normal 3.6-6.1 mmol/L), erythrocyte sedimentation rate (27, normal 0-20 mm/h), carbohydrate antigen CA153 (33.02, normal 0-25 U/mL); negative results for hepatitis B and C viruses and microspironema pallidum; antistreptolysin-O, rheumatoid factor, C-reaction protein, tuberculin test, antinuclear antibody, thyroid functions and angiotensin-converting enzyme levels were normal; electrocardiography showed sinus arrhythmia with bradycardia; computer tomography scans of the chest and abdomen showed bilateral axillary fossa lymphadenectasis, increased numbers of lymph nodes in the mediastinum and splenomegaly. No neoplasm was found in trigger neoplasm scanning. Histopathological examination of an ichthyotic lesion and a subcutaneous nodule revealed multiple non-caseating epithelial cell-like granulomas in the deep dermis (figures 1C, D). Periodic acid-Schiff (for fungi), Ziehl-Nielsen (for acid fast bacilli), and Steiner (for spirochetes) stains were negative.

The skin lesions and most of subcutaneous nodules markedly improved in 2 weeks after oral prednisone (30 mg/d). Meanwhile, the urine protein (24 h) fell to a normal level. However, a relapse occurred within 3 weeks of a follow-up visit after discontinuation of oral prednisone.

Sarcoidosis is a systemic disease that can involve almost all organ systems and is characterised by non-caseating granuloma infiltrations [1]. Cutaneous manifestations occur in 25% of patients with systemic sarcoidosis [2]. As a “great imitator”, it may result in various clinical manifestations. The most common are papular, nodular, plaque lesions and involvement of scars. Rare cutaneous expressions of sarcoidosis are erythroderma and acquired ichthyosis [1, 2]. To our knowledge, only 5 cases of erythroderma with a silvery and lamellar scaling, characterized by non-caseating granulomas, has been described in the literature [1-5]. Our patient with generalized erythrodermic sarcoidosis had dry lamellar scaling, which was more ichthyosis vulgaris-like than cases previously reported.

Patients are diagnosed with sarcoidosis when a compatible clinical or radiological picture is present, along with histological evidence of non-caseating granuloma, and when other potential causes, such as infections, foreign objects, are excluded. Furthermore, Blau syndrome, a rare autosomal dominant, chronic granulomatous disease of skin, uveal tract and joints, must be distinguished from sarcoidosis. If possible, analysis on CARD15 gene is recommended [6]. In our case, no family history of similar conditions and no abnormalities in the eyes and joints helped us excluded the diagnosis of Blau syndrome. Other infections such as syphilis, tuberculosis and leprosy were all ruled out by laboratory examination.

The treatment of sarcoidosis is often frustrating, because lesions may be refractory to treatment or may recur following successful treatment. Systemic glucocorticoids with tapering dosages are the most effective agents [1, 2, 5]. Our case was sensitive to therapy with prednisone but recurred within 3 weeks without treatment.

Acknowledgements

References

2 Wirth FA, Gould WM, Kauffman CL. Erythroderma in a patient with arthralgias, uveitis, and dyspnea. Arch Dermatol 1999; 135: 1411-4.

3 Amal S, Khadir K, Agzour K, Nejjam F, Badre L, Sqalli S, Lakhdar H. Icthyosiform erythroderma: atypical manifestation of sarcoidosis. Ann Dermatol Venereol 2000; 127: 64-6.

4 Wigley JE, Musso LA. A case of sarcoidosis with erythrodermic lesions. Treatment with calciferol. Br J Dermatol 1951; 63: 398-406.

5 Morrison JG. Sarcoidosis in a child, presenting as an erythroderma with keratotic spines and palmar pits. Br J Dermatol 1976; 95: 93-7.

6 Masel G, Halbert A. Blau syndrome presenting with ichthyosis. Australas J Dermatol 2005; 46: 29-32.