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Pityriasis lichenoides in children: Therapeutic response to erythromycin
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Pityriasis lichenoides in children: Therapeutic response to erythromycin Andrew P. Truhan, M.D., Adelaide A. Hebert, M.D., and Nancy B. Esterly, M.D.
Chicago, 1L Fifteen of twenty-two children with pityriasis lichenoides were treated with oral erythromycin. Eleven (73%) had a remission, usually within 2 months. Two others showed partial improvement, and two were unimproved. Seven of the children who experienced a remission were off erythromycin and free of lesions after 2 to 5 months of therapy. A trial of erythromycin as described herein should be considered in children with pityriasis lichenoides before other, possibly more toxic, measures are instituted. (J AM ACAD DERMATOL 15:66-70, 1986.)
Pityriasis lichenoides is an uncommon cutaneous disorder consisting of two variants, acute (pityriasis lichenoides et varioliformis acuta, Mucha-Habermann disease) and chronic (pityriasis lichenoides chronica, Juliusberg type). Both are characterized by generalized, erythematous or tan, scaly papules and macules primarily occurring over the trunk and flexural surfaces of the extremities. The eruption persists for many months or recurs in periodic, abrupt exacerbations. In acute pityriasis lichenoides, the lesions at a single point in time are i~a various stages of evolution and may include hemorrhagic, vesicular, pustular, and even necrotic (varioliform) lesions. Scarring and pigmentary changes are common sequelae, and the entire process lasts weeks to months. Chronic pityriasis lichenoides is characterized by lesions that are less severe and less variable and usually more scaly than those of the acute variant. Chronic pityriasis lichenoides persists for months to years and exacerbations and remissions are the rule. The distincfion between acute and chronic pityriasis liFrom the Departments of Pediatrics and Dermatology, Northwestern University Medical School. Accepted for publication March 20, 1986. Reprint requests to: Dr. Nancy B, Esterly, Division of Dermatology, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614.
66
chenoides is not always clear because there may be transitional lesions forming a continuous spectrum. 1.2 Although various types of therapy have been proposed, for pityriasis lichenoides, including systemic steroids, 3 methotrexate, 4 sulfones, 5 and ultraviolet radiation, 6 most of the documented therapeutic trials have involved adults w.ith the disease. There are few studies of appropriate, efficacious therapy in children. We wish to report our experience with oral erythromycin in the management of fifteen pediatric patients with pityriasis lichenoides. PATIENTS AND METHODS
We reviewed twenty-two cases of pityriasis lichenoides seen at Children's Memorial Hospital since i980. Sixteen patients were male and six were female. Their ages at diagnosis ranged from 2 to 15 years. Seventeen patients were 10 years of age or younger at the time of diagnosis. Thirteen patients were white, three were black, five were Hispanic, and one was of East Indian descent. The diagnosis of pityriasis lichenoides was based, in all but one case, on the combined history, clinical appearance, and histopathologic examination of biopsy material. Skin biopsies were done in twenty-one of the cases; no other laboratory studies were routinely performed. Nine patients were refe~ed to us with the di-
Volume 15 Number 1 July, 1986
agnosis of pityriasis lichenoides, in which we concurred. All of the cases included in this study were typical in that they had a persistent cutaneous disorder characterized by scaly, papular lesions that were occasionally hemorrhagic and that tended to occur in crops. Fourteen patients had acute pityriasis lichenoides, and eight patients had the chronic form of pityriasis lichenoides. The duration of pityriasis lichenoides varied from 6 weeks to 1Y2 years at the time of presentation to our clinic. Most patients had received topically applied corticostero!ds and systemic antihistamines with a generally poor response. One patient had also been treated with oral tetracycline for 2 weeks without significant effect. Another patient had not responded to an 8-week course of ultraviolet B phototherapy three times weekly. After the self-limited nature of pityriasis lichenoides wa s explained, treatment with oral erythromycin was offered to all patients. No other systemic therapy was .used. Nineteen patients were contacted for follow-up, and most of these had follow-up of greater than 1 year at the time of this writing. Follow-up in thirteen patients included evaluation in the dermatology clinic by one of us and information obtained via questionnaire and telephone interview. Follow-up data in six patients were obtained via questionnaire and telephone interview only. In three patients, no follow-up data were available because the patient could not be traced. The response tO therapy was assessed on the basis of a quantitative resolution of lesions, with remission of disease defined as return of skin to the predisease state or clearing with only occasional development of new lesions. RESULTS
Of the nineteen patients with pityriasis lichenoides on whom follow-up evaluations were obtained, fifteen received treatment with erythromycin (Table I). Remission was achieved in eleven patients (Cases 1-7, 9, 12-14), generally within 2 months of initiation of therapy. Seven of these patients (Cases 1-6, ~13) discontinued erythromycin after 2 to 5 months of therapy and remained free of lesions in follow-up periods ranging from 6 weeks (Case 4) to 3 years (Case 6). Three patients (Cases 7, 12, 14) were nearly clear of lesions but still on erythromycin at the time of writing. The other patient ,(Case 9) had been in remission for 5 months while on medication but experienced a recrudescence of disease when erythromycin was suddenly discontinued.
Pityriasis lichenoides in children
67
The remaining four patients (Cases 8, 10, 11, 15) who were treated with erythromycin were evenly divided in response. Two patients (Cases 8 and 10) noted some improvement with erythromycin, but, despite significant dosage increases, the responses were insufficient to be classified as remissions. The two other patients (Cases 11 and 15) showed no response to erythromycin, although one patient (Case 15) might have benefited from a higher dosage. Of the four patients who never received erythromycin, three had shown spontaneous improvement, according to the parents, prompting them to withhold therapy. However, their rate of clearance was considerably slower than that observed in the majority of patients who received erythromycin. Two of these patients were diagnosed as having pityriasis lichenoides et varioliformis acuta and one, pityriasis lichenoides chronica. All three patients were free or nearly free o f lesions 1 to 2 years later. The fourth patient, seen in consultation, was not given erythromycin by the referring physician. His eruption (pityriasis lichenoides chronica) had continued unchanged after 9 months. DISCUSSION Many therapeutic modalities have been employed in the treatment of pityriasis lichenoides and, although some have been beneficial, no one agent has attained wide clinical acceptance. The most commonly used treatments are topical corticosteroids and systemic antihistamines, which may relieve pruritus but have little influence on the course of the disease. Systemically administered steroids occasionally clear the lesions, ~'7 and improvement has been reported after methot r e x a t e 4,8,9 and dapsone? Lesions of pityriasis lichenoides occur less frequently and in milder forms on sun-exposed skin, and sunlight has, therefore, been thought to be useful. 1'7 Accordingly, patients with pityriasis lichenoides have been treated with artificial ultraviolet radiation, often with good results.68 Oral antibiotics, including penicillin, 1° high-dose tetracycline,11,12 and erythromycin,9a3 have also been used in clearing pityriasis lichenoides. A number of these .therapeutic maneuvers, however, are unsuitable for use
68
Journal of the American Academy of Dermatology
Truhan et al
T a b l e I, Response to treatment with oral erythromycin
-C e
, ..
[
Diagnosis
i Duration
Treatment .
Erythromycin, 200 mg tid (40 mg/ kg/day) Erythromycin, 200 mg qid (35 mg/ kg/day) Erythromycin, 200 mg tid (40 mg/ kg/day) Erythromycin, 200 mg tid (40 rag/ kg/day) Erythromycin, 200 mg tid (23 mg/ kg/day) Erythromycin (1 gm/day)
1
M
2
PLEVA
2 mo
2
M
4
PLEVA
11/2yr
3
M
3
PLEVA
2 mo
4
M
2
PLEVA
6 wk
5
M
10
PLEVA
2 mo
6*
F
11
PLEVA
6 wk
7
M
4
PLEVA
1 yr
8
M
2
PLEVA
4 mo
.
Result
Improvement within 1 mo; nearly dear within 2 mo Improvement within ! too; nearly clear within 2 mo Improvement within 3 wk; nearly clear within 2 mo Improvement within 1 too; nearly clear within 2 mo Improvement within 1 too; nearly clear within 2 mo Improvement within 3 wk, but recurrence when drug tapered at this time; nearly clear 2 mo later on initial dosage Erythromycin, 200 Improvement within 2 wk; nearly clear mg tid (44 mg/ within 2 mo kg/day) Erythromycin, 200 Improvement within 1 mo; flare when mg qid (50 rag/ drug tapered at 6 kg/day) wk; dosage increased 400 nag tid with good response
I
Follow-up
Drug tapered and stopped after 3 mo; 6 mo later, no recurrence Drug tapered and stopped after 4 too; 272 yr later, no recurrence Drug tapered and stopped after 3 ruo; 11 mo later, no recurrence Drug stopped after 2 too; 6 wk later, no recurrence Drug tapered and stopped after 2 too; 1V2 yr later, no recurrence Drug tapered and stopped after 5 too; 3 yr later, no recurrence
Minimal disease activity 7 mo later on medication Much improved 4 mo later on medication
PLC: Pityriasislichenoideschronica~PLEVA:pityriasislichenoideset varioliformisacuta;qd: everyday; qid: four times daily;rid: three times daily. *Previousunsuccessfultreatment with tetracyclinefor 2 wk. ?Previousunsuccessfultreatment with ultravioletB phototherapyfor 8 wk. in pediatric patients. Since 20% of patients with pityriasis lichenoides may be less than 10 years old,' the choice of an appropriate therapeutic agent in this self-limited disease may be a dilemma. To date, there have been only a few reports in the literature of patients with pityriasis lichenoides treated with erythromycin. 9:3 Shavin et al ~3 described three children whose Mucha-Habermann disease resolved with erythromycin. Rasmussen, 9 however, noted no consistently beneficial response in eight pediatric patients treated with erythromycin for a minimum o f 6 weeks. Two of his patients apparently improved, while four patients
remained unchanged, and two worsened. Our overall response to treatment with erythromycin has been more favorable, with thirteen of fifteen patients (87%) in our series experiencing remission or partial improvement. However, it frequently took as long as 2 months before a significant therapeutic effect was noted, and we had to push the dosage level to its upper limit in several cases. Clearing with oral erythromycin was seen in most cases at doses of 30 to 50 mg/kg/day. We then slowly tapered the dosage of the drug over the course of several months, depending on the response. In our experience, the diseaseusually re-
Volume 15 Number 1 July, 1986
Pityriasis lichenoides in children
69
Table I. Cont'd
Case Sex I Age (yr, Diagnus2.s Duration I 9
F
9
PLEVA
11/2yr
10t
M
7
PLEVA
10 mo
11
F
11
PLEVA
1 yr
12
M
10
PLC
6 mo
13
F
14
PLC
7 mo
14
M
4
PLC
2 mo
15
M
8
PLC
1 yr
Treatment
Result ....
Erythromycin (1 gm/day)
Improvement within 6 wk; nearly clear within 3 mo Erythromycin, 200 No response after 6 wk; dosage inmg qid (30 mg/ creased 1 gin/day kg/day) with improvement within 3 wk No response after 1 Erythromycin (1 mo; dosage ingm/day) creased 500 mg tid also without significant response; drug stopped Erythromycin, 200 Improvement within 1 too; nearly clear mg qid (25 rag/ within 3 mo kg/day) Improvement within 1 Erythromycin (1 mo; clear within 2 gm/day)
mo
Erythromycin, 400 No response after 3 mo; dosage inmg qd (15 mg/ creased 400 mg tid kg/day) with improvement within 2 wk Erythromycin, 200 No response after 6 mo; drug stopped mg qid (23 mg/ kg/day)
curred if erythromycin therapy was tapered too rapidly. We prefer erythromycin over tetracycline in all patients under 12 years of age to avoid possible adverse effects on dentition. We observed no significant side effects from treatment with erythromycin. Evaluation of clinical improvement with erythromycin is difficult in pityriasis lichenoides because of the cyclic and self-limited nature of the disease. We believe that cessation in the development of new lesions, together with recurrences that were temporally related to the tapering or discontinuance of erythromycin, speak for therapeutic effect. In addition, spontaneous remissions were not seen in any of our patients prior to the time erythromycin therapy was begun. The etiology of pityriasis lichenoides remains obscure. Serologic, epidemiologic, and therapeu-
]
Follow-up
5 n-to later, recurrence after drug stopped Moderate disease activity after 1 Vz yr on medication; drug stopped
3 yr later, only minimal disease activity remains
Drug tapered; 1 yr later, no recurrence on medication Drug tapered and stopped after 5 mo; 4 mo later, n o recurrence Minimal disease activity 7 wk later on medication
5 yr later, clear
tic evidence suggests that Mucha-Habermann disease may be the result of a hypersensitivity reaction to an infectious agent. ~1,~2No such agent has been identified, though, and transmission of the disease from one individual to another has never been documented. Circulating immune complexes may be involved in the pathogenesis of the disorder. 14 When the pathogenesis o f pityriasis lichenoides is finally elucidated, it may be easier to explain the therapeutic mechanism of erythromycin. It has been speculated, however, that an antiinflammatory effect related to inhibition of monocyte chemotaxis by erythromycin might explain the improvement seen in this condition.13 Pityriasis lichenoides can be a severe, progressive, scarring disease with significant emotional, social, and physical consequences. This retrospective study demonstrates the efficacy of eryth-
70
Journal of the American Academy of Dermatology
Truhan et al
r o m y c i n in t h e c l e a r a n c e o f this disease in children if u s e d for at least 2 m o n t h s at the u p p e r r a n g e o f therapeutic d o s a g e . It is our o p i n i o n that such a trial o f e r y t h r o m y c i n is indicated in all pediatric patients w i t h pityriasis l i c h e n o i d e s b e f o r e treatm e n t with other modalities is initiated.
REFERENCES 1. Marks R, Black M, Wilson Jones E: Pityriasis lichenoides: A reappraisal. Br J Dermatol 86:215-225, 1972. 2. Lambert WC, Everett MA: The nosology of parapsoriasis. J AM ACADDERMATOL5:373-395, 1981. 3. Winkelmann RK, Lorenc E: Treatment of acute parapsoriasis with corticotropin (ACTH): Report of a case. Arch Dermatol 79:512-515, 1959. 4. Lynch PJ, Saied NK: Methotrexate treatment ofpityriasis lichenoides and lymphomatoid papulosis. Cutis 23:634636, 1979. 5. McMfllan EM, Everett MA: Pityriasis lichenoides et varioliforrnis acuta, in Maddin S, Carruthers A, editors: Current dermatologic therapy. Philadelphia, 1982, W. B. Saunders Co., pp. 362-364.
6. LeVine MJ: Phototherapy of pityfiasis lichenoides. Arch Dermatol 119:378-380, 1983, 7. Powell FC, Muller SA: Psoralens and ultraviolet A therapy of pityrJasis lichenoides. J AM Ac.Ao DERMATOL I0:59-64, 1984. 8. Tham SN: UVB phototherapy for pityriasis lichenoides. Australas J Dermatol 26:9-13, 1985. 9. Rasmussen JE: Mucha-Habermann's disease. Arch Dermatol 115:676-677, 1979. (Letter to Editor.) 10. Verallo VM, Haserick JR: Mucha-Habermann's disease simulating lymphoma cutis. Report of two cases. Arch Dermatot 94:295-299, 1966. 11. Piamphongsant T: Tetracycline for the treatment of pityriasis lichenoides. Br J Dermatol 91:319-322, 1974. 12. Shelley WB, Griffith RF: Pityriasis lichenoides et varioliforrnis acuta. A report of a case controlled by a high dosage of tetracycline. Arch Dermatol 100:596-597, 1969. 13. Shavin JS, Jones TM, Aton JK, et al: Mucha-Habermann's disease in children. Treatment with erythromycin. Arch Dermatol 114:1679-1680, 1978. 14. Clayton R, Haffenden G: An immunofluorescence study of pityriasis lichenoides. Br J Dermatol 99:491-493, 1978.
ABSTRACTS
Small intestinal permeability in dermatological disease Hamilton I, Fairris GM, Rothwell J, et al: Q J Med 56:559-567, 1985 Impaired permeability of the small bowel in persons with psoriasis or atopic dermatitis often has been mentioned but studied little. In this study of 62 patients no such abnormality was found. Abnormal permeability is not likely a nonspecifie effect of skin disease, but a specific part of diseases such as dermatitis herpetiformis. Philip C. Anderson, M.D.
Tick-borne Borrelia infection in Sweden Stiemstedt G: Scand J Infect Dis 45:1-70, 1985
Treatment of varicella-zoster virus infection in severely immunocompromised patients: A randomized comparison of acyclovir and vidarabine Shepp DH, Dandliker PS, Meyers JD: N Engl J Med 314:208-212, 1986 Cutaneous dissemination of infection did not occur in any of the 10 acyclovir recipients but was present in 5 of 10 vidarabine recipients presenting with localized dermatomal disease. Acylovir was more effective than vidarabine in decreasing the median interval to the first decrease in pain, pustulation of lesions, crusting of lesions, and complete healing of lesions. d. Graham Smith, Jr., M.D.
Swedish ticks contain the spirochetes of Lyme d~sease in this study, leading on to the conclusion that erythema chronicum migrans is a manifestation of infection by Borrelia. Philip C. Anderson, M.D.
Dermatomyositis-like syndrome caused by trichinae. Report of two cases
Erythema nodosum following typhoid vaccination
Trichinosis, we all recall, can mimic dermatomyositis. The proof of the diagnosis still depends on finding the larva in muscle. Philip C. Anderson, M.D.
Thomson BJ, Nuki G: Scott Med J 30:173, 1985 Other vaccinations also cause erythema nodosum. Philip C. Anderson, M.D.
Herrera R, Varela E, Morales G, e t a [ : J Rheumatol 12:782-784, 1985
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Pityriasis lichenoides in children: Therapeutic response to erythromycin Andrew P. Truhan, M.D., Adelaide A. Hebert, M.D., and Nancy B. Esterly, M.D.
Chicago, 1L Fifteen of twenty-two children with pityriasis lichenoides were treated with oral erythromycin. Eleven (73%) had a remission, usually within 2 months. Two others showed partial improvement, and two were unimproved. Seven of the children who experienced a remission were off erythromycin and free of lesions after 2 to 5 months of therapy. A trial of erythromycin as described herein should be considered in children with pityriasis lichenoides before other, possibly more toxic, measures are instituted. (J AM ACAD DERMATOL 15:66-70, 1986.)
Pityriasis lichenoides is an uncommon cutaneous disorder consisting of two variants, acute (pityriasis lichenoides et varioliformis acuta, Mucha-Habermann disease) and chronic (pityriasis lichenoides chronica, Juliusberg type). Both are characterized by generalized, erythematous or tan, scaly papules and macules primarily occurring over the trunk and flexural surfaces of the extremities. The eruption persists for many months or recurs in periodic, abrupt exacerbations. In acute pityriasis lichenoides, the lesions at a single point in time are i~a various stages of evolution and may include hemorrhagic, vesicular, pustular, and even necrotic (varioliform) lesions. Scarring and pigmentary changes are common sequelae, and the entire process lasts weeks to months. Chronic pityriasis lichenoides is characterized by lesions that are less severe and less variable and usually more scaly than those of the acute variant. Chronic pityriasis lichenoides persists for months to years and exacerbations and remissions are the rule. The distincfion between acute and chronic pityriasis liFrom the Departments of Pediatrics and Dermatology, Northwestern University Medical School. Accepted for publication March 20, 1986. Reprint requests to: Dr. Nancy B, Esterly, Division of Dermatology, Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614.
66
chenoides is not always clear because there may be transitional lesions forming a continuous spectrum. 1.2 Although various types of therapy have been proposed, for pityriasis lichenoides, including systemic steroids, 3 methotrexate, 4 sulfones, 5 and ultraviolet radiation, 6 most of the documented therapeutic trials have involved adults w.ith the disease. There are few studies of appropriate, efficacious therapy in children. We wish to report our experience with oral erythromycin in the management of fifteen pediatric patients with pityriasis lichenoides. PATIENTS AND METHODS
We reviewed twenty-two cases of pityriasis lichenoides seen at Children's Memorial Hospital since i980. Sixteen patients were male and six were female. Their ages at diagnosis ranged from 2 to 15 years. Seventeen patients were 10 years of age or younger at the time of diagnosis. Thirteen patients were white, three were black, five were Hispanic, and one was of East Indian descent. The diagnosis of pityriasis lichenoides was based, in all but one case, on the combined history, clinical appearance, and histopathologic examination of biopsy material. Skin biopsies were done in twenty-one of the cases; no other laboratory studies were routinely performed. Nine patients were refe~ed to us with the di-
Volume 15 Number 1 July, 1986
agnosis of pityriasis lichenoides, in which we concurred. All of the cases included in this study were typical in that they had a persistent cutaneous disorder characterized by scaly, papular lesions that were occasionally hemorrhagic and that tended to occur in crops. Fourteen patients had acute pityriasis lichenoides, and eight patients had the chronic form of pityriasis lichenoides. The duration of pityriasis lichenoides varied from 6 weeks to 1Y2 years at the time of presentation to our clinic. Most patients had received topically applied corticostero!ds and systemic antihistamines with a generally poor response. One patient had also been treated with oral tetracycline for 2 weeks without significant effect. Another patient had not responded to an 8-week course of ultraviolet B phototherapy three times weekly. After the self-limited nature of pityriasis lichenoides wa s explained, treatment with oral erythromycin was offered to all patients. No other systemic therapy was .used. Nineteen patients were contacted for follow-up, and most of these had follow-up of greater than 1 year at the time of this writing. Follow-up in thirteen patients included evaluation in the dermatology clinic by one of us and information obtained via questionnaire and telephone interview. Follow-up data in six patients were obtained via questionnaire and telephone interview only. In three patients, no follow-up data were available because the patient could not be traced. The response tO therapy was assessed on the basis of a quantitative resolution of lesions, with remission of disease defined as return of skin to the predisease state or clearing with only occasional development of new lesions. RESULTS
Of the nineteen patients with pityriasis lichenoides on whom follow-up evaluations were obtained, fifteen received treatment with erythromycin (Table I). Remission was achieved in eleven patients (Cases 1-7, 9, 12-14), generally within 2 months of initiation of therapy. Seven of these patients (Cases 1-6, ~13) discontinued erythromycin after 2 to 5 months of therapy and remained free of lesions in follow-up periods ranging from 6 weeks (Case 4) to 3 years (Case 6). Three patients (Cases 7, 12, 14) were nearly clear of lesions but still on erythromycin at the time of writing. The other patient ,(Case 9) had been in remission for 5 months while on medication but experienced a recrudescence of disease when erythromycin was suddenly discontinued.
Pityriasis lichenoides in children
67
The remaining four patients (Cases 8, 10, 11, 15) who were treated with erythromycin were evenly divided in response. Two patients (Cases 8 and 10) noted some improvement with erythromycin, but, despite significant dosage increases, the responses were insufficient to be classified as remissions. The two other patients (Cases 11 and 15) showed no response to erythromycin, although one patient (Case 15) might have benefited from a higher dosage. Of the four patients who never received erythromycin, three had shown spontaneous improvement, according to the parents, prompting them to withhold therapy. However, their rate of clearance was considerably slower than that observed in the majority of patients who received erythromycin. Two of these patients were diagnosed as having pityriasis lichenoides et varioliformis acuta and one, pityriasis lichenoides chronica. All three patients were free or nearly free o f lesions 1 to 2 years later. The fourth patient, seen in consultation, was not given erythromycin by the referring physician. His eruption (pityriasis lichenoides chronica) had continued unchanged after 9 months. DISCUSSION Many therapeutic modalities have been employed in the treatment of pityriasis lichenoides and, although some have been beneficial, no one agent has attained wide clinical acceptance. The most commonly used treatments are topical corticosteroids and systemic antihistamines, which may relieve pruritus but have little influence on the course of the disease. Systemically administered steroids occasionally clear the lesions, ~'7 and improvement has been reported after methot r e x a t e 4,8,9 and dapsone? Lesions of pityriasis lichenoides occur less frequently and in milder forms on sun-exposed skin, and sunlight has, therefore, been thought to be useful. 1'7 Accordingly, patients with pityriasis lichenoides have been treated with artificial ultraviolet radiation, often with good results.68 Oral antibiotics, including penicillin, 1° high-dose tetracycline,11,12 and erythromycin,9a3 have also been used in clearing pityriasis lichenoides. A number of these .therapeutic maneuvers, however, are unsuitable for use
68
Journal of the American Academy of Dermatology
Truhan et al
T a b l e I, Response to treatment with oral erythromycin
-C e
, ..
[
Diagnosis
i Duration
Treatment .
Erythromycin, 200 mg tid (40 mg/ kg/day) Erythromycin, 200 mg qid (35 mg/ kg/day) Erythromycin, 200 mg tid (40 mg/ kg/day) Erythromycin, 200 mg tid (40 rag/ kg/day) Erythromycin, 200 mg tid (23 mg/ kg/day) Erythromycin (1 gm/day)
1
M
2
PLEVA
2 mo
2
M
4
PLEVA
11/2yr
3
M
3
PLEVA
2 mo
4
M
2
PLEVA
6 wk
5
M
10
PLEVA
2 mo
6*
F
11
PLEVA
6 wk
7
M
4
PLEVA
1 yr
8
M
2
PLEVA
4 mo
.
Result
Improvement within 1 mo; nearly dear within 2 mo Improvement within ! too; nearly clear within 2 mo Improvement within 3 wk; nearly clear within 2 mo Improvement within 1 too; nearly clear within 2 mo Improvement within 1 too; nearly clear within 2 mo Improvement within 3 wk, but recurrence when drug tapered at this time; nearly clear 2 mo later on initial dosage Erythromycin, 200 Improvement within 2 wk; nearly clear mg tid (44 mg/ within 2 mo kg/day) Erythromycin, 200 Improvement within 1 mo; flare when mg qid (50 rag/ drug tapered at 6 kg/day) wk; dosage increased 400 nag tid with good response
I
Follow-up
Drug tapered and stopped after 3 mo; 6 mo later, no recurrence Drug tapered and stopped after 4 too; 272 yr later, no recurrence Drug tapered and stopped after 3 ruo; 11 mo later, no recurrence Drug stopped after 2 too; 6 wk later, no recurrence Drug tapered and stopped after 2 too; 1V2 yr later, no recurrence Drug tapered and stopped after 5 too; 3 yr later, no recurrence
Minimal disease activity 7 mo later on medication Much improved 4 mo later on medication
PLC: Pityriasislichenoideschronica~PLEVA:pityriasislichenoideset varioliformisacuta;qd: everyday; qid: four times daily;rid: three times daily. *Previousunsuccessfultreatment with tetracyclinefor 2 wk. ?Previousunsuccessfultreatment with ultravioletB phototherapyfor 8 wk. in pediatric patients. Since 20% of patients with pityriasis lichenoides may be less than 10 years old,' the choice of an appropriate therapeutic agent in this self-limited disease may be a dilemma. To date, there have been only a few reports in the literature of patients with pityriasis lichenoides treated with erythromycin. 9:3 Shavin et al ~3 described three children whose Mucha-Habermann disease resolved with erythromycin. Rasmussen, 9 however, noted no consistently beneficial response in eight pediatric patients treated with erythromycin for a minimum o f 6 weeks. Two of his patients apparently improved, while four patients
remained unchanged, and two worsened. Our overall response to treatment with erythromycin has been more favorable, with thirteen of fifteen patients (87%) in our series experiencing remission or partial improvement. However, it frequently took as long as 2 months before a significant therapeutic effect was noted, and we had to push the dosage level to its upper limit in several cases. Clearing with oral erythromycin was seen in most cases at doses of 30 to 50 mg/kg/day. We then slowly tapered the dosage of the drug over the course of several months, depending on the response. In our experience, the diseaseusually re-
Volume 15 Number 1 July, 1986
Pityriasis lichenoides in children
69
Table I. Cont'd
Case Sex I Age (yr, Diagnus2.s Duration I 9
F
9
PLEVA
11/2yr
10t
M
7
PLEVA
10 mo
11
F
11
PLEVA
1 yr
12
M
10
PLC
6 mo
13
F
14
PLC
7 mo
14
M
4
PLC
2 mo
15
M
8
PLC
1 yr
Treatment
Result ....
Erythromycin (1 gm/day)
Improvement within 6 wk; nearly clear within 3 mo Erythromycin, 200 No response after 6 wk; dosage inmg qid (30 mg/ creased 1 gin/day kg/day) with improvement within 3 wk No response after 1 Erythromycin (1 mo; dosage ingm/day) creased 500 mg tid also without significant response; drug stopped Erythromycin, 200 Improvement within 1 too; nearly clear mg qid (25 rag/ within 3 mo kg/day) Improvement within 1 Erythromycin (1 mo; clear within 2 gm/day)
mo
Erythromycin, 400 No response after 3 mo; dosage inmg qd (15 mg/ creased 400 mg tid kg/day) with improvement within 2 wk Erythromycin, 200 No response after 6 mo; drug stopped mg qid (23 mg/ kg/day)
curred if erythromycin therapy was tapered too rapidly. We prefer erythromycin over tetracycline in all patients under 12 years of age to avoid possible adverse effects on dentition. We observed no significant side effects from treatment with erythromycin. Evaluation of clinical improvement with erythromycin is difficult in pityriasis lichenoides because of the cyclic and self-limited nature of the disease. We believe that cessation in the development of new lesions, together with recurrences that were temporally related to the tapering or discontinuance of erythromycin, speak for therapeutic effect. In addition, spontaneous remissions were not seen in any of our patients prior to the time erythromycin therapy was begun. The etiology of pityriasis lichenoides remains obscure. Serologic, epidemiologic, and therapeu-
]
Follow-up
5 n-to later, recurrence after drug stopped Moderate disease activity after 1 Vz yr on medication; drug stopped
3 yr later, only minimal disease activity remains
Drug tapered; 1 yr later, no recurrence on medication Drug tapered and stopped after 5 mo; 4 mo later, n o recurrence Minimal disease activity 7 wk later on medication
5 yr later, clear
tic evidence suggests that Mucha-Habermann disease may be the result of a hypersensitivity reaction to an infectious agent. ~1,~2No such agent has been identified, though, and transmission of the disease from one individual to another has never been documented. Circulating immune complexes may be involved in the pathogenesis of the disorder. 14 When the pathogenesis o f pityriasis lichenoides is finally elucidated, it may be easier to explain the therapeutic mechanism of erythromycin. It has been speculated, however, that an antiinflammatory effect related to inhibition of monocyte chemotaxis by erythromycin might explain the improvement seen in this condition.13 Pityriasis lichenoides can be a severe, progressive, scarring disease with significant emotional, social, and physical consequences. This retrospective study demonstrates the efficacy of eryth-
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Truhan et al
r o m y c i n in t h e c l e a r a n c e o f this disease in children if u s e d for at least 2 m o n t h s at the u p p e r r a n g e o f therapeutic d o s a g e . It is our o p i n i o n that such a trial o f e r y t h r o m y c i n is indicated in all pediatric patients w i t h pityriasis l i c h e n o i d e s b e f o r e treatm e n t with other modalities is initiated.
REFERENCES 1. Marks R, Black M, Wilson Jones E: Pityriasis lichenoides: A reappraisal. Br J Dermatol 86:215-225, 1972. 2. Lambert WC, Everett MA: The nosology of parapsoriasis. J AM ACADDERMATOL5:373-395, 1981. 3. Winkelmann RK, Lorenc E: Treatment of acute parapsoriasis with corticotropin (ACTH): Report of a case. Arch Dermatol 79:512-515, 1959. 4. Lynch PJ, Saied NK: Methotrexate treatment ofpityriasis lichenoides and lymphomatoid papulosis. Cutis 23:634636, 1979. 5. McMfllan EM, Everett MA: Pityriasis lichenoides et varioliforrnis acuta, in Maddin S, Carruthers A, editors: Current dermatologic therapy. Philadelphia, 1982, W. B. Saunders Co., pp. 362-364.
6. LeVine MJ: Phototherapy of pityfiasis lichenoides. Arch Dermatol 119:378-380, 1983, 7. Powell FC, Muller SA: Psoralens and ultraviolet A therapy of pityrJasis lichenoides. J AM Ac.Ao DERMATOL I0:59-64, 1984. 8. Tham SN: UVB phototherapy for pityriasis lichenoides. Australas J Dermatol 26:9-13, 1985. 9. Rasmussen JE: Mucha-Habermann's disease. Arch Dermatol 115:676-677, 1979. (Letter to Editor.) 10. Verallo VM, Haserick JR: Mucha-Habermann's disease simulating lymphoma cutis. Report of two cases. Arch Dermatot 94:295-299, 1966. 11. Piamphongsant T: Tetracycline for the treatment of pityriasis lichenoides. Br J Dermatol 91:319-322, 1974. 12. Shelley WB, Griffith RF: Pityriasis lichenoides et varioliforrnis acuta. A report of a case controlled by a high dosage of tetracycline. Arch Dermatol 100:596-597, 1969. 13. Shavin JS, Jones TM, Aton JK, et al: Mucha-Habermann's disease in children. Treatment with erythromycin. Arch Dermatol 114:1679-1680, 1978. 14. Clayton R, Haffenden G: An immunofluorescence study of pityriasis lichenoides. Br J Dermatol 99:491-493, 1978.
ABSTRACTS
Small intestinal permeability in dermatological disease Hamilton I, Fairris GM, Rothwell J, et al: Q J Med 56:559-567, 1985 Impaired permeability of the small bowel in persons with psoriasis or atopic dermatitis often has been mentioned but studied little. In this study of 62 patients no such abnormality was found. Abnormal permeability is not likely a nonspecifie effect of skin disease, but a specific part of diseases such as dermatitis herpetiformis. Philip C. Anderson, M.D.
Tick-borne Borrelia infection in Sweden Stiemstedt G: Scand J Infect Dis 45:1-70, 1985
Treatment of varicella-zoster virus infection in severely immunocompromised patients: A randomized comparison of acyclovir and vidarabine Shepp DH, Dandliker PS, Meyers JD: N Engl J Med 314:208-212, 1986 Cutaneous dissemination of infection did not occur in any of the 10 acyclovir recipients but was present in 5 of 10 vidarabine recipients presenting with localized dermatomal disease. Acylovir was more effective than vidarabine in decreasing the median interval to the first decrease in pain, pustulation of lesions, crusting of lesions, and complete healing of lesions. d. Graham Smith, Jr., M.D.
Swedish ticks contain the spirochetes of Lyme d~sease in this study, leading on to the conclusion that erythema chronicum migrans is a manifestation of infection by Borrelia. Philip C. Anderson, M.D.
Dermatomyositis-like syndrome caused by trichinae. Report of two cases
Erythema nodosum following typhoid vaccination
Trichinosis, we all recall, can mimic dermatomyositis. The proof of the diagnosis still depends on finding the larva in muscle. Philip C. Anderson, M.D.
Thomson BJ, Nuki G: Scott Med J 30:173, 1985 Other vaccinations also cause erythema nodosum. Philip C. Anderson, M.D.
Herrera R, Varela E, Morales G, e t a [ : J Rheumatol 12:782-784, 1985