Sarcoidosis showing a livedo reticularis-like eruption

ARTICLE

Sarcoidosis is known to show various types of skin eruptions. We report a case of sarcoidosis showing a livedo reticularis-like eruption which has rarely been reported and as such is difficult to classify using ordinary sarcoidosis classification parameters.

Case report

A month before his first visit to our hospital, mistiness of the left eye and an eruption on the lower legs had developed in a 22-year-old man. Chest X-ray examination revealed bilateral, hilar lymphadenopathy (BHL). The eruption on his lower legs then enlarged and coalesced. He began to cough sputum, but he had neither dyspnea, nor fever. He was then seen by the Department of Internal Medicine of the Cancer Institute Hospital and ocular sarcoidosis was diagnosed because of iritis at Department of Ophthalmology. He was then referred to our Department for the cutaneous eruption.

His mother had also been diagnosed as suffering from sarcoidosis. According to our patient's description of her symptoms, her sarcoidosis was probably of the nodular type; not the same as our patient.

On examination there were many nail-sized erythematous areas on both lower legs, partly showing a reticular appearance because of coalescence, leaving islands of normal skin (Fig. 1). Slight induration was felt. He suffered neither itching nor pain. Cervical lymph nodes were not palpable.

A biopsy specimen from the left lower leg showed relatively small nodules scattered from the dermis (Fig. 2A) to the subcutaneous fat (Fig. 2B). The nodules were round, oval or irregular in shape and scattered mainly around the sweat glands or vessels. They were well-demarcated and consisted of epithelioid cells, partly containing foreign body or Langerhans type giant cells or with lymphoid cell infiltration around them, but without caseation necrosis (Fig. 3).

Laboratory examinations revealed a normal peripheral blood cell count. Erythrocyte sedimentation rate was 6 mm/h. Blood chemistry including serum calcium level was within normal limits apart from a slight increase in the serum level of lactate dehydrogenase, 371 IU/l and uric acid, 78 mg/dl. Hypergamma-globulinemia was not seen. Serologic tests showed that both C-reactive protein and the RA (rheumatoid arthritis) test were negative and the serum angiotensin converting enzyme (ACE) level was slightly increased 24.8 IU/l. Immunological tests revealed a serum IgM level of 367 mg/dl. Skin test reaction to purified protein derivative of tuberculin was
0 x 0/7 x 7. Chest X-ray showed BHL.

After initiating treatment with oral prednisolone, 20 mg/day and topical betamethasone valerate, the eruption disappeared within a week. The BHL did not change but serum ACE level decreased gradually. Prednisolone was tapered to 5 mg/day, but was increased to 40 mg/day later because of misting of the right eye, iritis of the both eyes, an increase of intraocular pressure, bleeding and vasculitis. After that, the condition of the eyes improved and prednisolone was tapered to 20 mg and 15 mg, on alternate days.

Discussion

Sarcoidosis is usually classified as follows: erythema nodosun, erythematous and erythematopapular, "scar sarcoidosis", papular ("small nodular"); including the (Boeck) lichenoid variety, erythrodermic (Schaumann), nodular; including annular (or circinate), angiolupoid (Brocq-Pautrier) and subcutaneous plaque; including lupus pernio and miscellaneous; including ulcerative, psoriasiform, palmoplantar, ungual, mucosal, etc., and sarcoidosis of the American negro [1].

We use most commonly the Fukushiro's classification [2] for classifying sarcoidosis of the skin in Japan. This classifies sarcoidosis of the skin into three major categories: (1) erythema nodosum; (2) infiltration of the scar; (3) sarcoid of the skin. Sarcoid of the skin includes the nodular type, plaque type, diffuse infiltration type, subcutaneous type and the following as non-specific: erythema nodosum-like eruption, lichenoid type, etc.

Cases such as icthyosiform eruption [3, 4], a case showing poikiloderma [5] and morphea-like eruptions [4] are reported as rare cases.

If we try to classify our case according to the usual classification, it may be the diffuse infiltration type or an erythema nodosum-like eruption although neither of these seems to exactly describe the eruption. We therefore thought of the expression "livedo reticularis-like eruption".

In Japan we could find only two other cases showing livedo reticularis [6, 7] and one case partly showing it [8]. There are no similar cases in other countries to our knowledge, and we cannot give a suitable explanation as to whether or not such cases are seen only in Japan. These three cases have shown sarcoidosis nodules on cutaneous biopsy, and were classified as sarcoidosis showing erythema nodosum-like eruption by Fukushiro [9]. In contrast, the clinical finding of our case differed from that of erythema nodosum because the eruption was erythema without induration. Komine et al. [10] reported a case which resembled our case showing indurated erythema of the lower legs, abdomen and back as sarcoidosis showing an atypical eruption which is impossible to classify (Table I). Our case of sarcoidosis was a relatively rare, familial one involving a mother and son. Of all the familial cases of sarcoidosis reported in Japan up to December 1992 (80 cases), most of them were brothers and sisters, then parents and children, and the combinations including females are more frequent than male [11] (Table II). About half of the familial cases of sarcoidosis were discovered in patients over 40 years old and showed lesions of sarcoidosis on chest X-ray, extrapulmonary lesions and the long-duration, high serum level of ACE [11].

Our case was thought to be a relatively typical familial case because the combination mother/son, both having pulmonary and extrapulmonary lesions although the details of the mother were not well-known and the serum ACE levels decreased to within normal limits after therapy.

CONCLUSION

REFERENCES

1. Savin JA. Sarcoidosis. In: Champion RH, Burton JL, Ebling FJG, eds. Textbook of Dermatology. 5th ed., Vol. 3. Oxford: Blackwell Scientific Publications, 1992: 2383-406.

2. Fukushiro R, Niki F. Sarcoidosis as viewed from dermatology. Rinsho Dermatol 1960; 14: 730-41.

3. Matarasso SL, Bruce S. Ichthyosiform sarcoidosis: report of a case. Cutis 1991; 47: 405-8.

4. Hess SP, Agudelo CA, White WL, Jorizzo JL. Ichthyosiform and morpheaform sarcoidosis. Clin Exp Rheumatol 1990; 8: 171-5.

5. Watanabe K, Suzuki K. A case of sarcoidosis showing poikiloderma. Nippon Hifuka Gakkai Zasshi 1983; 93: 232.

6. Masaki H, Shinkai H. Three cases of sarcoidosis showing atypical symptoms. Hifu 1972; 14: 253-65.

7. Yoshie H, Hatayama M. A case of sarcoidosis. Jpn J Clin Dermatol 1973; 27: 587-94.

8. Hashimoto T, Uehara Y, Kuramochi M, et al. A case of sarcoidosis with bilateral facial palsy. Rinsyo Dermatol 1981; 35: 131-4.

9. Fukushiro R. Erythema nodosum like eruption. In: Fukushiro R, Kukita A, Sano E, Kiyotera M, eds. Gendai Fifukagaku Taikei. 1st ed., Vol. 18. Tokyo: Nakayama Shoten, 1984: 318-21.

10. Komine M, Ogawa K, Harada S. Sarcoidosis showing atypical eruption. Rinsyo Dermatol 1994; 36: 1739-43.

11. Tachibana T, Hiraga H, Yamaguchi E, et al. Prospective study of familial cases of sarcoidosis. Year's report of the diffuse pulmonary lung disease by the research congress of the Ministry of Health and Welfare 1992: 40-1.