778
Journal o[ the American Academy of Dermatol%y
Brief communications
i
Fig. 2. Melanocytic hyperplasia in basal cell layer. (Melanin stain; x400.) It is probable that histopathologicaUy this entity may actually be a spectrum; at one end there is hypermelanosis of the epidermis without melanocytic hyperplasia, whereas at the opposite end there is atypical melanocytic hyperproliferation. 6 V M / L appears to have a benign course with no reports of malignant degeneration. The sparsity of case reports and the lack of long-term follow-up, however, make it impossible to predict its natural history. Biopsies should be performed whenever pigmented spots are noted in the genital area. Regular follow-up and repeat biopsies of changing lesions are suggested until this condition is understood more completely.
2. Maize JC. Mucosal melanosls. Dermatol Clin 1988;6:28393. 3. Rudolph RL Vulvar melanosis. J AM ACAD DERMATOL 1990;23:982-4. 4. Barnhill RL, Albert LS, Sharna SK, et al. Genital lentiginosis: a clinical and histopathologic study. J AM ACAD DERMATOL 1990;22:453-60. 5. Kopf AW, Bart RS. Penile lentigo. J Dermatol Surg Oncol 1982;8:637-9. 6. Leicht S, Youngberg G, Diaz-Miranda C. Atypical pigmented penile maeules. Arch Derrnatol 1988;124:1267-70. 7. Sison-Torre EQ, Ackerman AB. Melanosis of the vulva: a clinical simulator of malignant melanoma. Am J Dermatopatho/1985;7(suppl):51-60. 8. Revuz J, Clerici T. Penile melanosis. J AM ACAD DERMA. TO1. 1989;20:567-70. 9. Lever WF, Schamburg-Lever G. Bowen's disease. In: Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1990:549-51. 10. Bhawan J, Cahn TM. Atypical penile lentigo. J Dermatol Surg Oncol 1984;10:99-[00.
REFERENCES 1, Jackson R. Melanosis of the vulva..I Dermatol Surg Oneol 1984; 10:119-21. I
I
I
PUVA treatment of pigmented purpuric lichenoid dermatitis (Gougerot-Blum) Judit Krizsa, MD, J~mos Hunyadi, PhD, and Attila Dobozy, DSc Szeged, Hungary The term pigmented purpuric dermatosis incorporates several diseases characterized by orange or brown pigmentation interspersed with From the Department of Dermatology, Albert Szent-Gy6rgylMedical University. Reprint request.s: Judit Krizsa, MD, P.O. Box 480, H-6701 Szeged, Hungary. 16/54/38765
petechiae. One of these is the pigmented purpuric lichenoid dermatitis (PPLD) described by Gougerot and Blum. The disease most often occurs on the legs. t Pigmented purpuric dermatoses can be resistant to treatment. Simon and Hunyadi 2 reported the PUVA therapy to be effective in eczematid-like purpura; this was recently confirmed by Wong and Ratnam. 3
Volume 27 Number 5, Part I November 1992
Brief communications 779
Fig. 1. Patient 3. Before PUVA therapy.
Fig. 2. Patient 3. After pLrVA therapy.
Table I. S u m m a r y of clinical data and efficacy of PUVA therapy Cumula five Patient No.
Sex
Age (yr)
Duration of disease (too)
I
F
2 3 4
F M M
43 53 32 60
84 96 18 12
5 6 7
F F F
63 48 54
12 9 1
No. of PUVA treatments
dose of UVA
(J/era 2)
Efficacy of PUVA (mo)*
7
16 43 18 42 46 35 35 35 49
76 64 34 It 15 16 12 it 7
20 8 12 15 14 14 12 15
*Duration of symptom-free period after PUVA therapy. q'After 1 month symptom-free period a relapse was observed and the patients were treated again.
W e report seven cases of P P L D treated successfully with PUVA. PATIENTS AND METHODS Seven patients with clinical and histologic findings characteristic of PPLD were treated with PUVA (Table I). In the papillary vessels, depositions of C3 with or without immunoglobulins and/or fibrin were demon-
strated by direct immunofluorescence studies in all patients. PUVA therapy was carried out as recommended by the European PUVA Study Group. 4 RESULTS All patients' lesions cleared after 7 to 20 P U V A treatments (Figs. i and 2). Five patients remained clear but in two (patients 4 and 7) recurrence was
780
Brief communications
seen after 1 m o n t h ( T a b l e I). After a second series of treatments, these patients cleared and have remained disease free. DISCUSSION P P L D is characterized histologically by inflammation and h e m o r r h a g e without fibrinoid necrosis of vessels. A f t e r extravasation, yellow-brown hemosiderin deposits a c c u m u l a t e in the dermis and are ingested by macrophages. E p i d e r m a l involvement leads to the formation o f lichenoid papules and plaques, l, 5 T h e origin o f the p i g m e n t e d purpuric dermatoses is obscure. T h e s e diseases have been reported to result from a sensitivity to wool oil, 6 carbutamide, 7 or carbromal. 8 I n the papillary vessels, i m m u n e complex deposition has been observed. 9, ~0 T h e lesional keratinocytes express surface antigens believed to be characteristic of cytokine-mediated dermatoses.t 1 These findings suggest the involvement of a delayedtype hypersensitivity reaction in the pathogenesis of pigmented p u r p u r i c dermatoses. U V radiation can alter the distribution and function of the lymphocytesl2; a single dose of P U V A transiently decreased the n u m b e r of circulating T cells, 13 and P U V A t r e a t m e n t alters the distribution of the l y m p h o c y t e subpopulations.14 Transient impairment of t h e response o f lymphocytes to phytohemagglutinin also occurs. 15 Most patients undergoing P U V A t r e a t m e n t show a diminished responsiveness to dinit rochloroben zene. 16P U V A treatment can inhibit t h e m i g r a t o r y activity of T lymphocytes I 7 and c a n suppress interleuldn 2 production, t 8 Furthermore, it leads to the disappearance of epidermal L a n g e r h a n s cells. 19 W e speculate that the immunosuppressive effects of p h o t o c h e m o t h e r a p y played a role in the excellent clinical response to P U V A in these patients. REFERENCES
1, Braun-Falco O, Plewig G, Wolff HH, et a[. Dermatology. Berlin: Springer Verlag, 1984:661. 2. Simon M Jr, Hunyadi J. PUVA-Therapie der Ekzematidartigen Purpura. Aktuel Dermatol 1986;12:100-2.
Journal of the American Academy of Dermatology
3. Wong WK, Ratnam KV. A report of two cases of pigmented purpuric dermatoses treated with PUVA therapy. Acta Derm Venereol (Stockh) 1991;71:68-70. 4. Henseler T, Wolff K, H6nigsmann H, et al. Oral 8-methoxypsoralen photochemotherapy of psoriasis. Lancet 1981; 1:853-7. 5. McKee PH. Pathology of the skin with clinical correlations. London: Gower Medical Publishing, 1989:5-22. 6. Greenwood K. Dermatitis with capillary fragility. Arch Dermatol 1960;81:113-7. 7. Dobozy A, Hunyadi J, Husz S. Dutch Carhutamid-Uberempfindlichkeit verursachter Morbus Gougerot-Blum. Z Hautkr 1974;49:1009-12. 8. Rosenthal D, Burnham TK. Nonthrombocytic purpura due to carbromal ingestion. Arch Dermatol 1964;89:200-4. 9. Iwatsuki K, Aoshima T, Tagami H. Immunofluorescence study in purpura pigmentosa chronica. Acta Derm Venereol (Stockh) 1980;60:341-70. 10. Ratnam KV, Su WPD, Peters MS. Purpura simplex (inflammatory purpura without vasculitis): a clinicopathologic study of 174 eases. J AM ACAD DERMATOL1991;25: 642-7. 11. Simon M Jr, Heese A, Gftz A. Immunopathological investigations in purpura pigmentosa chronica. Aeta Derm Venereol (Stockh) 1989;69:10I-4. 12. Morison WL, Parrish JA, Epstein JH. Photoimmunology. Arch Dermatol 1979;115:350-5. 13. Morison WL, Parrish JA, Bloch KJ. In vivo effects of PUVA on lymphocyte function. Br J Dermatol 1981;104: 405-13. 14. Moscicki RA, Morison WL, Parrish JA, et al. Reduction of the fraction of circulating helper-inducer T cells identified by monoclonal antibodies in psoriatic patients treated with long-term psoralen/ultraviolet-A radiation (PUVA). J Invest Dermatol 1982;79:205-8. 15. Scherer R, Kern B, Braun-Falco O. UVA-induced inhibition of proliferation of PHA-stimulated lymphocytes from humans treated with 8-methoxypsoralen. Br J Dermatol 1977;97:519-28. 16. Strauss GH, Bridges BA, Greaves M. Inhibition of delayed hypersensitivity reaction in skin (DNCB test) by 8-methoxypsoralen photochemotherapy. Lancet 1980;2:556-9. 17. Okamoto H, Takigawa M, Horio T. Alteration of lymphocyte functions by 8-methoxypsoralen and long-wave ultraviolet radiation. 1. Suppressiveeffects of PUVA on T lymphocyte migration in vitro. J Invest Dermatol 1985;84: 203-5. 18. Okamoto H, Horio T, Maeda M. Alteration &lymphocyte functions by 8-methoxypsoralen and long-wave ultraviolet radiation. II. The effect of in vivo PUVA on IL-2 production. J Invest Dermatol 1987;1:24-6. 19. Friedmann PS. Disappearance of epidermal Langerhans ceils during PUVA therapy. Br J Dermatol 198 I; 105:21921.
Journal o[ the American Academy of Dermatol%y
Brief communications
i
Fig. 2. Melanocytic hyperplasia in basal cell layer. (Melanin stain; x400.) It is probable that histopathologicaUy this entity may actually be a spectrum; at one end there is hypermelanosis of the epidermis without melanocytic hyperplasia, whereas at the opposite end there is atypical melanocytic hyperproliferation. 6 V M / L appears to have a benign course with no reports of malignant degeneration. The sparsity of case reports and the lack of long-term follow-up, however, make it impossible to predict its natural history. Biopsies should be performed whenever pigmented spots are noted in the genital area. Regular follow-up and repeat biopsies of changing lesions are suggested until this condition is understood more completely.
2. Maize JC. Mucosal melanosls. Dermatol Clin 1988;6:28393. 3. Rudolph RL Vulvar melanosis. J AM ACAD DERMATOL 1990;23:982-4. 4. Barnhill RL, Albert LS, Sharna SK, et al. Genital lentiginosis: a clinical and histopathologic study. J AM ACAD DERMATOL 1990;22:453-60. 5. Kopf AW, Bart RS. Penile lentigo. J Dermatol Surg Oncol 1982;8:637-9. 6. Leicht S, Youngberg G, Diaz-Miranda C. Atypical pigmented penile maeules. Arch Derrnatol 1988;124:1267-70. 7. Sison-Torre EQ, Ackerman AB. Melanosis of the vulva: a clinical simulator of malignant melanoma. Am J Dermatopatho/1985;7(suppl):51-60. 8. Revuz J, Clerici T. Penile melanosis. J AM ACAD DERMA. TO1. 1989;20:567-70. 9. Lever WF, Schamburg-Lever G. Bowen's disease. In: Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1990:549-51. 10. Bhawan J, Cahn TM. Atypical penile lentigo. J Dermatol Surg Oncol 1984;10:99-[00.
REFERENCES 1, Jackson R. Melanosis of the vulva..I Dermatol Surg Oneol 1984; 10:119-21. I
I
I
PUVA treatment of pigmented purpuric lichenoid dermatitis (Gougerot-Blum) Judit Krizsa, MD, J~mos Hunyadi, PhD, and Attila Dobozy, DSc Szeged, Hungary The term pigmented purpuric dermatosis incorporates several diseases characterized by orange or brown pigmentation interspersed with From the Department of Dermatology, Albert Szent-Gy6rgylMedical University. Reprint request.s: Judit Krizsa, MD, P.O. Box 480, H-6701 Szeged, Hungary. 16/54/38765
petechiae. One of these is the pigmented purpuric lichenoid dermatitis (PPLD) described by Gougerot and Blum. The disease most often occurs on the legs. t Pigmented purpuric dermatoses can be resistant to treatment. Simon and Hunyadi 2 reported the PUVA therapy to be effective in eczematid-like purpura; this was recently confirmed by Wong and Ratnam. 3
Volume 27 Number 5, Part I November 1992
Brief communications 779
Fig. 1. Patient 3. Before PUVA therapy.
Fig. 2. Patient 3. After pLrVA therapy.
Table I. S u m m a r y of clinical data and efficacy of PUVA therapy Cumula five Patient No.
Sex
Age (yr)
Duration of disease (too)
I
F
2 3 4
F M M
43 53 32 60
84 96 18 12
5 6 7
F F F
63 48 54
12 9 1
No. of PUVA treatments
dose of UVA
(J/era 2)
Efficacy of PUVA (mo)*
7
16 43 18 42 46 35 35 35 49
76 64 34 It 15 16 12 it 7
20 8 12 15 14 14 12 15
*Duration of symptom-free period after PUVA therapy. q'After 1 month symptom-free period a relapse was observed and the patients were treated again.
W e report seven cases of P P L D treated successfully with PUVA. PATIENTS AND METHODS Seven patients with clinical and histologic findings characteristic of PPLD were treated with PUVA (Table I). In the papillary vessels, depositions of C3 with or without immunoglobulins and/or fibrin were demon-
strated by direct immunofluorescence studies in all patients. PUVA therapy was carried out as recommended by the European PUVA Study Group. 4 RESULTS All patients' lesions cleared after 7 to 20 P U V A treatments (Figs. i and 2). Five patients remained clear but in two (patients 4 and 7) recurrence was
780
Brief communications
seen after 1 m o n t h ( T a b l e I). After a second series of treatments, these patients cleared and have remained disease free. DISCUSSION P P L D is characterized histologically by inflammation and h e m o r r h a g e without fibrinoid necrosis of vessels. A f t e r extravasation, yellow-brown hemosiderin deposits a c c u m u l a t e in the dermis and are ingested by macrophages. E p i d e r m a l involvement leads to the formation o f lichenoid papules and plaques, l, 5 T h e origin o f the p i g m e n t e d purpuric dermatoses is obscure. T h e s e diseases have been reported to result from a sensitivity to wool oil, 6 carbutamide, 7 or carbromal. 8 I n the papillary vessels, i m m u n e complex deposition has been observed. 9, ~0 T h e lesional keratinocytes express surface antigens believed to be characteristic of cytokine-mediated dermatoses.t 1 These findings suggest the involvement of a delayedtype hypersensitivity reaction in the pathogenesis of pigmented p u r p u r i c dermatoses. U V radiation can alter the distribution and function of the lymphocytesl2; a single dose of P U V A transiently decreased the n u m b e r of circulating T cells, 13 and P U V A t r e a t m e n t alters the distribution of the l y m p h o c y t e subpopulations.14 Transient impairment of t h e response o f lymphocytes to phytohemagglutinin also occurs. 15 Most patients undergoing P U V A t r e a t m e n t show a diminished responsiveness to dinit rochloroben zene. 16P U V A treatment can inhibit t h e m i g r a t o r y activity of T lymphocytes I 7 and c a n suppress interleuldn 2 production, t 8 Furthermore, it leads to the disappearance of epidermal L a n g e r h a n s cells. 19 W e speculate that the immunosuppressive effects of p h o t o c h e m o t h e r a p y played a role in the excellent clinical response to P U V A in these patients. REFERENCES
1, Braun-Falco O, Plewig G, Wolff HH, et a[. Dermatology. Berlin: Springer Verlag, 1984:661. 2. Simon M Jr, Hunyadi J. PUVA-Therapie der Ekzematidartigen Purpura. Aktuel Dermatol 1986;12:100-2.
Journal of the American Academy of Dermatology
3. Wong WK, Ratnam KV. A report of two cases of pigmented purpuric dermatoses treated with PUVA therapy. Acta Derm Venereol (Stockh) 1991;71:68-70. 4. Henseler T, Wolff K, H6nigsmann H, et al. Oral 8-methoxypsoralen photochemotherapy of psoriasis. Lancet 1981; 1:853-7. 5. McKee PH. Pathology of the skin with clinical correlations. London: Gower Medical Publishing, 1989:5-22. 6. Greenwood K. Dermatitis with capillary fragility. Arch Dermatol 1960;81:113-7. 7. Dobozy A, Hunyadi J, Husz S. Dutch Carhutamid-Uberempfindlichkeit verursachter Morbus Gougerot-Blum. Z Hautkr 1974;49:1009-12. 8. Rosenthal D, Burnham TK. Nonthrombocytic purpura due to carbromal ingestion. Arch Dermatol 1964;89:200-4. 9. Iwatsuki K, Aoshima T, Tagami H. Immunofluorescence study in purpura pigmentosa chronica. Acta Derm Venereol (Stockh) 1980;60:341-70. 10. Ratnam KV, Su WPD, Peters MS. Purpura simplex (inflammatory purpura without vasculitis): a clinicopathologic study of 174 eases. J AM ACAD DERMATOL1991;25: 642-7. 11. Simon M Jr, Heese A, Gftz A. Immunopathological investigations in purpura pigmentosa chronica. Aeta Derm Venereol (Stockh) 1989;69:10I-4. 12. Morison WL, Parrish JA, Epstein JH. Photoimmunology. Arch Dermatol 1979;115:350-5. 13. Morison WL, Parrish JA, Bloch KJ. In vivo effects of PUVA on lymphocyte function. Br J Dermatol 1981;104: 405-13. 14. Moscicki RA, Morison WL, Parrish JA, et al. Reduction of the fraction of circulating helper-inducer T cells identified by monoclonal antibodies in psoriatic patients treated with long-term psoralen/ultraviolet-A radiation (PUVA). J Invest Dermatol 1982;79:205-8. 15. Scherer R, Kern B, Braun-Falco O. UVA-induced inhibition of proliferation of PHA-stimulated lymphocytes from humans treated with 8-methoxypsoralen. Br J Dermatol 1977;97:519-28. 16. Strauss GH, Bridges BA, Greaves M. Inhibition of delayed hypersensitivity reaction in skin (DNCB test) by 8-methoxypsoralen photochemotherapy. Lancet 1980;2:556-9. 17. Okamoto H, Takigawa M, Horio T. Alteration of lymphocyte functions by 8-methoxypsoralen and long-wave ultraviolet radiation. 1. Suppressiveeffects of PUVA on T lymphocyte migration in vitro. J Invest Dermatol 1985;84: 203-5. 18. Okamoto H, Horio T, Maeda M. Alteration &lymphocyte functions by 8-methoxypsoralen and long-wave ultraviolet radiation. II. The effect of in vivo PUVA on IL-2 production. J Invest Dermatol 1987;1:24-6. 19. Friedmann PS. Disappearance of epidermal Langerhans ceils during PUVA therapy. Br J Dermatol 198 I; 105:21921.
