PUVA-treatment of urticaria pigmentosa

Britishjournal of Dermatology (1978) 98, 701.

PUVA-treatment of urticaria pigmentosa ENNO CHRISTOPHERS Department of Dermatology, University of Kiel, Germany, HERBERT HONIGSMANN and KLAUS WOLFF Department of Dermatology, University of Innsbruck, Austria, and ANDRZEJ LANGNER Department of Dermatology, University of Warsaw, Poland

Treatment of urticaria pigmentosa (UP) has been unsatisfactory. The patients' complaints of itching, swelling and increased skin sensitivity are well known and distressing and are unrelieved by local or systemic remedies. We have treated io patients suffering from UP with oral 8-methoxypsoralen (8-MOP) followed by long wave ultraviolet irradiation (UVA). The treatment schedule currently used for the treatment of psoriasis was adopted (Wolff ef al., 1975). All patients showed typical generalized cutaneous involvement of late onset (except patient 10). None of them were of the telangiectasia macularis eruptiva perstans type. All diagnoses were confirmed by biopsy (except patient 10). The patients received 05-0-8 mg/kg 8-MOP 2 h prior to UVA irradiation (Waldmann PUVA 4000). PUVA was given 4 times a week until no wealing could be produced by mechanical irradiation of the skin (Darier's sign). The data relevant to treatment are summarized in Table i. Treatment with PUVA resulted in generalized tanning and loss of Darier's sign. While the hyperpigmented macules overlying the mast cell infiltrates showed some persistence, wealing and dermographism following physical trauma could no longer be provoked. The padents unanimously noted rehef from itching and physical distress. TABLE I. Urticaria pigmentosa

Patients

Age/sex M

Age of onset (years) 37 35

KI

42

K2 K3

39 F 31 F

K4 I5

67

F F

60

16

48 F

38

I7 18 W9

27 40 40

WIO

51

M F M 7 F

20

36

25

36 23 I

Additional diagnosis Nephrolithiasis None Migraine, gastritis Cholelithiasis Thrombocythaemia IgG-K-paraproteinaemia. migraine None None Migraine None

Skin No of biopsy treatments

Total dose (J/cm*)

Total duration Free of sympof treatment toms after (months) (irradiations)

II

32

3

21

I

9 7

UP

9 49

417

10

14

UP UP

16

36

I

16

57

162

4

25

UP

28

157

2

14

UP UP UP

49

307

5

25

127

27 16

6 8

12

1-5 075

14

I

UP UP

*UP = urticaria pigmentosa; K = Kiel; I = Innsbruck; W = Warsaw. 0007-0963/78/0600-0701 $ 02.00 © 1978 British Association of Dermatologists 701

6 8

702

Brief communication

In patients 5-8 an area of 10 x 15 cm was shielded during the entire course of treatment. Here, the cutaneous symptoms persisted unchanged. Patients 3 and 6 suffered from recurrent attacks of migraine every 2-3 weeks. In patient 3, determination of serum histamine concentration revealed an increase to 10 /ig/ml. Re-examination of her histamine concentration after 4 months PUVA treatment revealed normal values (07-1-o ngjmX). No migraine attacks were noted. These reappeared, however, when the patient was taken off PUVA treatment for 8 weeks and also urtication of the skin could be demonstrated again. Five patients (Cases 3, 5, 6, 7, 8) were observed during post-treatment followup. A relapse occurred between 3 (patient 7) and 6 (patient 6) months. Also, in patient 6 this relapse coincided with the recurrence of migraine. Patient 8 remained clear for more than 6 months. Our observations indicate that PUVA is effective in the treatment of urticaria pigmentosa. It appears to be warranted in cases in which these cutaneous abnormalities are causing severe distress. Next to psoriasis (Parrish et al, 1974; Wolff et al, 1975) and mycosis fungoides (Gilchrest et al, 1976; Roenigk, 1977) urticaria pigmentosa can be recognized as a PUVA sensitive dermatosis. The spectrum of disorders known to be sensitive to PUVA treatment raises challenging questions as to the mode of action of this therapy. ACKNOWLEDGMENT

We thank Professor Parawesch, Department of Pathology, University of Kiel for spectrofluorometrical determination of blood histamine in patient 3. REFERENCES GILCHREST, B.A., PARRISH, J., TANENBAUM, L . HAYNES, H.A. & FITZPATRICK, T.B. (1976) Oral methoxsalen

photochemotherapy of mycosis fungoides. Cancer, 38, 683. PARRISH, J.A., FITZPATRICK, T.B., TANENBAUM, L . & PATHAK, M.A. (1974) Photochemotherapy of psoriasis with

oral methoxsalen and longwave ultraviolet light. New England Journal of Medicine, 291, 1207. ROENIGK, H.H. (1977) Photochemotherapy for mycosis fungoides. Archives of Dermatology, 113, 1047. WOLFF, K . , HONIGSMANN, H . , GSCHNAIT, F . & KONRAD, K . (1975) Photochemotherapie bei Psoriasis. Deutsche

medizinische Wochenschrift, 100, 2471. WOLFF, K . , FITZPATRICK, T.B., PARRISH, J.A., GSCHNAIT, F . , GILCHEST, B., HONIGSMANN, H . , PATHAK, M.A. &

TANENBAUM, L . (1976) Photochemotherapy for psoriasis with orally administered methoxsalen. Archives of Dermatology, 112, 943.