1018 Case reports
J AM ACAD DERMATOL DECEMBER 2004
Eruptive disseminated superficial actinic porokeratosis in an immunocompetent host: Is this associated with herpes simplex virus or bacterial infection? Yong-Hyun Jang, MD,a Sei-Jung Chun, MD,b Won Hyoung Kang, MD,c and Eun-So Lee, MDa Suwon and Gwangmyeong, Korea
orokeratosis, a specific disorder of keratinization, generally progresses slowly over years.1 The etiology of different variants of porokeratosis is unknown. However, there are increasing numbers of reports of disseminated superficial actinic porokearatosis (DSAP) in connection with impaired immunocompetence. Recently, we observed two cases of acute onset DSAP in immunocompetent hosts.
CASE 1 A 57-year-old woman presented with numerous 3 mm to 6 mm pruritic brown annular lesions on the face, arms, and legs that abruptly developed within 2 weeks. At the first visit, in addition to the DSAP lesions, she also presented with erythema multiforme (EM) lesions on both extremities and some grouped vesicles on the buttock shown to be herpes simplex virus (HSV) infections (Fig 1, A). The patient had experienced a recurrent HSV infection over 10 years. She said target lesions turned into DSAP lesions. A biopsy specimen revealed dermal edema and necrotic keratinocytes in the epidermis, which was compatible with EM, surrounded by parakeratotic column on the margin (Fig 2, A). The DSAP lesion was positive for HSV type 1 by polymerase chain reaction (Fig 2, B).
Fig 1. A, Some grouped vesicles showing HSV infection on the buttock and porokeratotic lesions directly associated with the target lesion. B, Multiple hyperpigmented macules with peripheral keratotic ridge on both the hands and forearms.
A 53-year-old woman visited our department for multiple pruritic 3 mm to 6 mm hyperpigmented macules on the dorsa of hands and forearms for 3
Fig 2. A, Porokeratotic column with the lesion of erythema multiforme showing dermal edema and dyskeratotic cells in the epidermis. B, Detection of HSV type 1 (271bp) by polymerase chain reaction in the DSAP lesion (Lane 1: 100bp DNA ladder; Lane 2: b-actin (539bp); Lane 3: positive control; Lane 4: DSAP lesion). (A, Hematoxylineosin stain; original magnification: A, 3100.)
From the Department of Dermatology, Ajou University School of Medicine,a Chun’s Skin Clinic,b and the Q Skin Clnic.c Funding sources: None. Conflicts of interest: None identified. Reprint requests: Eun-So Lee, MD, 5 Wonchon-dong, Yeongtong-ku, Suwon, 443-721, Korea (South). E-mail: [email protected]
J Am Acad Dermatol 2004;51:1018-9. 0190-9622/$30.00 ª 2004 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2004.07.006
days (Fig 1, B). The eruption started on her hand and rapidly extended to her forearm. She was otherwise in good health, but had suffered from a sore throat for 7 days. Histopathologic findings were compatible with DSAP. Several cases with rapid extension of DSAP have been reported in patients with chemotherapy,2 kidney transplantation,3 and bone marrow transplantation,4
Case reports 1019
J AM ACAD DERMATOL VOLUME 51, NUMBER 6
which are associated with immunosuppression. However, our patients showed pruritic DSAP as an acute flare-up in the immunocompetent state. Because of the acute and sudden appearance, we designated our cases as eruptive DSAP. The patient in case 1 had a recurrent HSV infection and EM simultaneously, which then turned into DSAP lesions. Kono et al5 reported 3 cases of DSAP in which the outbreak of DSAP was suspected to have occurred during the development of hepatocellular carcinoma in patients with hepatitis C virus-positive liver cirrhosis. But the association of HSV infection and DSAP has never been reported before. Unlike case 1, bacterial agents such as group A streptococcus may have contributed to the sudden appearance in case 2. We suspect that the abnormal clones of epidermal cells may be induced by a variety of triggering mechanisms, such as viral or bacterial infection, and is involved in the pathogenic mechanism of DSAP development. The presence of Langerhans cells in the epidermis in relation to early degenerating keratinocytes as well as the presence of helper T cells in the dermis are strong evidence for
immunologic mechanisms induced by antigen presentation. Whether the abnormal clone is triggered by infection allowing its proliferation and expression as a porokeratosis lesion, or the growth and differentiation of these abnormally susceptible cells are altered directly, is a question that remains to be answered.
REFERENCES 1. Chernosky ME, Freeman RG. Disseminated superficial actinic porokeratosis (DSAP). Arch Dermatol 1967;96:611-24. 2. Fields LL, White CR Jr, Maziarz RT. Rapid development of disseminated superficial porokeratosis after transplant induction therapy. Bone Marrow Transplant 1995;15:993-5. 3. Herranz P, Pizarro A, De Lucas R, Robayna MG, Rubio FA, Sanz A, et al. High incidence of porokeratosis in renal transplant recipients. Br J Dermatol 1997;136:176-9. 4. Rio B, Magana C, Le Tourneau A, Bachmeyer C, Levy V, Hamont N, et al. Disseminated superficial porokeratosis after autologous bone marrow transplantation. Bone Marrow Transplant 1997; 19:77-9. 5. Kono T, Kobayashi H, Ishii M, Nishiguchi S, Taniguchi S. Synchronous development of disseminated superficial porokeratosis and hepatitis C virus-related hepatocellular carcinoma. J Am Acad Dermatol 2000;43:966-8.
Recurrent hemolysis-associated pseudoerysipelas of the lower legs in a patient with congenital spherocytosis Martin Leverkus, MD,a Antonius Schwaaf, MD,a Eva-Bettina Bro¨cker, MD,a and Thomas M. Ru ¨ nger, MDb ¨ rzburg, Germany, and Boston, Massachusetts Wu A 29-year-old patient presented with recurrent erythematous eruptions on both lower legs of 15 years’ duration. Family history, along with clinical and laboratory examinations, revealed congenital hereditary spherocytosis and excluded other reasons for the erythematous eruptions of the lower legs. During two subsequent episodes, we detected increased hemolysis that disappeared concomittantly on spontanous resolution of the lesions. To our knowledge, this case is the first report showing a recurrent erythematous eruption on the lower legs in a patient with congenital hereditary spherocytosis. These eruptions might be caused by intermittent hemolysis-induced inflammation as a result of the increased osmotic fragility of the erythrocytes and may evolve to chronic leg ulcers later in life. ( J Am Acad Dermatol 2004;51:1019-23.)
From the Departments of Dermatology, University of Wu¨rzburg,a and School of Medicine, Boston University.b Funding sources: None. Conflicts of interest: None identified. Reprint requests: Martin Leverkus, MD, Department of Dermatology, University of Wu¨rzburg, Josef-Schneider-Str. 2, 97080 Wurzburg, Germany. E-mail: [email protected]
0190-9622/$30.00 ª 2004 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2004.08.010
e describe a patient with hereditary spherocytosis who presented with recurrent erythematous swelling of both lower legs. During these episodes, but not in symptom-free intervals, we found laboratory signs of increased hemolysis. Recurrent pseudoerysipelas might be the primary lesion leading to sclerosis and finally ulcer formation, the most common dermatologic complication in patients with hereditary spherocytosis.