Histiocytosis X in an adult: Treatment of lesions with Grenz rays Bernt Lindel6f , M.D. , Ph.D. Stockholm, Sweden

One adult woman patient with skin lesions typical of histiocytosis X was treated with grenz rays (ultrasoft X rays). She had no clear systemic involvement of the disease. During a period of 2 years she has received five courses of grenz rays, which have had a marked effect on the skin lesions, especially of the scalp. (J AM ACAD DERMA'roL 1988;19:426-7.)

skin

Histiocytosis X is a proliferative disorder of spe- cialized cells containing distinctive cytoplasmic structures characteristic of epidermal Langerhans cells.t Cutaneous lesions often occur.

The new information of the effect o f grenz rays (ultrasoft X rays) on Langerhans cells 2'3 and on certain skin disorders, 4-6 as well as the new aspects of safety o f these rays,7 have led me to treat with grenz rays the skin lesion of an adult patient with histiocytosis X. This article reports the results of such a therapy.

CASE REPORT

A 29-year-old woman had had diffuse redness, scal- ing, and crusting of the skin of the scalp, axillae, and inguinal regions since the age of 15 years. Since the age of 20 years, she also had had inflammation, swell- ing, and necrosis of gingival tissue in the mouth and had been followed up by several dermatologists. When she was 24 years old, a gingival biopsy specimen showed typical changes resulting from histiocytosis X. No systemic involvement has occurred, and roentgen- ographic examinations of the chest and osseous system have not given any clear evidence of extension of the disease to these sites. Neither have there been signs of lymph node enlargement, hepatosplenomegaly, or di- abetes insipidus.

Before the diagnosis of histiocytosis X, the patient had received treatment only with various topical prep-

From the Department of Dermatology, Karolinska Hospital, Stock- holm, Sweden.

Reprint requests to: Dr. Bernt Lindel6f, Department of Dermatology, Karolinska Hospital, S-104 01 Stockholm. Sweden.

426

arations containing corticosteroids and antibiotics. Af- ter the diagnosis, between the ages of 24 and 29 years, she had been treated with radiotherapy by oncologists and later with psoralens with ultraviolet A (PUVA) by dermatologists.

The patient received a total of 150 PUVA treatments on scalp, axillary, and inguinal areas. At the same sites she also had been treated with two courses of soft X rays consisting of 29 to 43 kV, half-value depth in tissue of 3 to 7 ram, and 2 Gy 5 times. During this period she also received seven courses of chemotherapy with vinblastine alkaloid (Velban).

METHOD

When the patient was 29 years old, I began grenz ray treatment because of the inadequacy of the PUVA therapy, which had moderately improved lesions in the axillae and inguinal region but not in the scalp. For 2 years the patient received a total of five courses of grenz ray therapy consisting of 10 kV, 10 mA, focus- skin distance of 10 cm, half-value layer of 0.03 mm A1 (half-value depth in tissue of 0.5 ram). The scalp has received 4 Gy, and the axillae and the inguinal region have received 1 to 2 Gy/wk for 6 to 10 weeks.

During these 2 years the patient has also received one course of chemotherapy with teniposid (Vumon) and cytarabine (Cytosar-U).

RESULTS

After the first course of grenz ray therapy, the improvement of the scalp lesions was impressive. The erythema, scaling, and crusts disappeared al- most totally. In the axillae and in the inguinal region the effect was marked but not as evident as in the scalp. In this regard the effect o f grenz rays

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Histioeytosis X treatment with grenz rays 427

in the axillae and in the inguinal region was es- sentially the same as the PUVA therapy previously given. However, the patient preferred grenz rays because of the convenience of administration of the grenz ray treatment. After that initial course of grenz rays, the skin symptoms slowly worsened and a new course of grenz rays was given after 6 months. The response was identical to that in the first course. The patient has now received a total of five courses of grenz rays over a period of 2 years, during which her scalp skin lesions have remained minimal. The lesions in the inguinal and axillary region have improved considerably, but the erythema persists and ulcers have occasionally developed.

DISCUSSION

Perhaps it is too early to accept categorically the proposition that histiocytosis X and Langer- hans cells share common lineage or indeed that histiocytosis X is due to a local proliferation and dissemination of Langerhans cells) The features linking histiocytosis X and Langerhans cells are, however, ultrastructurally well documented, even though there are differences. Both histiocytosis X and Langerhans cells contain Langerhans cells granules, but they are present in much greater numbers in histiocytosis X cells than they are in Langerhans cells.

The type of treatment used in histiocytosis X depends on the severity of the disease and the localization of lesions, and it consists either of systemic treatment with alkylating agents or plant alkaloids or of local treatment with surgery or ra- diotherapy. 9 When the disease is located on the skin, grenz ray therapy should be considered not only because of the results discussed here but be- cause it is very convenient to use.

The supersoft grenz rays (half-value layer in tissue, 0.5 mm) have proved to produce a pro- nounced reduction of Langerhans cells. A single dose of 4 Gy reduced the number of epidermal Langerhans cells to less than 30% of the pretreat- ment value. 2 This type of treatment seems to be of special value in dealing with histiocytosis X

skin lesions located in hair-bearing areas. Even though scales and hair absorb a large amount of the dose, unlike ultraviolet B and PUVA, the pen- etration of grenz rays is sufficient.

In a recent study 7 of the incidence of malignant skin tumors in 14,140 patients who received grenz ray treatment for benign skin disorders, it was concluded that even if grenz ray therapy cannot be excluded as a risk factor in the development of nonmelanoma skin tumors, this risk factor is small, if present at all, provided certain recom- mendations regarding the therapy are followed. This large-scale study has reassured us that grenz ray therapy is accompanied by little risk for cancer development. Other types of side effects of the treatment are also rare.* Thus grenz ray therapy appears to be a safe, effective, and convenient mode of treatment for skin lesions associated with histiocytosis X.

*Lindeltf B. Grenz ray therapy in dermatology: an experimental, clinical and epidemiological study [Dissertation]. Stockholm, Swe- den: Karolinska Institute, 1987.

REFERENCES 1. Wolff K, The Langerhans' ceil. Cutr Probl Dermatol

1972;4:79-145. 2. Lindel6f B, Lidtn S, Ros A-M. Effect of grenz rays on

Langerhans' ceils in human epidermis. Acta Derm Ve- taereol 1984;64:436-8.

3. LindeltfB,ForslindB. Electron microscopic observations of Langerhans' cells in human epidermis irradiated with grenz rays. Photodermatology 1985;2:367-71.

4. Lindeltf B, Lidtn S, Lagerholm B. The effect of grenz rays on the expression of allergic contact dermatitis in man. Scand J Immunol 1985;21:463-9.

5. Lindel6f B, Lindberg M. The effect of grenz rays on irritative skin reactions in man. Acta Derm Venereol 1987;67:128-32.

6. Johannesson A, Lindeltff B. The effect of grenz rays on psoriasis lesions of the scalp: a double blind trial. Pho- todermatology 1985;2:388-91.

7. Lindelff B, Eklund G. Incidence of malignant skin tu- rnouts in 14,140 patients after grenz ray treatment for benign skin disorders. Arch Dermatol 1986; 122:1391-5.

8. Nezelof C, Basset F, Rousseau M. Histiocytosis X: his- togenic arguments for the Langerhans' cell origin. Bio- medicine 1973;18:365-71.

9. Lever WF. Histiocytosis X. In: Demis J, ed. Clinical der- matology. New York: Harper and Row, 1986.