Angela Yen

EBV-associated Kikuchi's histiocytic necrotizing lymphadenitis with cutaneous manifestations

The clinical and pathologic findings of Kikuchis histiocytic necrotizing lymphadenitis may mimic those of malignant lymphoma. We describe a 6-year-old boy with generalized lymphadenopathy, spiking fever, chills, myalgias, malaise, and erythematous, crusted papules. Although cutaneous manifestations have been noted in 16% to 40% of patients with histiocytic necrotizing lymphadenitis, only three publications described skin lesions. The skin lesions and affected lymph nodes revealed histiocytic aggregates, atypical lymphoid cells, karyorrhectic debris, and patchy necrosis. Spontaneous resolution occurred in 2 months. Results of serologic studies, Epstein-Barr virus (EBV) latent membrane protein immunoperoxidase staining, EBER-1 RNA in-situ hybridization, and EBV EBNA-1 DNA polymerase chain reaction implicate EBV as the causative agent.

Detection of human herpesvirus-8 DNA in Kaposi’s sarcomas from iatrogenically immunosuppressed patients

BACKGROUND Kaposis sarcoma (KS) accounts for more than 5% of malignancies in immunosuppressed organ transplant patients (OKS). A new herpesvirus (HHV-8) was identified with high prevalence in biopsy specimens of AIDS-KS, endemic KS, and classic KS and in OKS. KS has also been associated with other underlying diseases in patients treated with corticosteroids, but this subset of KS has been reported to contain HHV-8 in only a few case reports. OBJECTIVE In this larger study, we determined the prevalence of HHV-8 in seven patients of Jewish origin in whom KS developed during immunosuppressive therapy for different primary diseases (ISKS). METHODS The study included HHV-8 DNA detection by polymerase chain reaction (PCR) coupled with Southern blot and sequence analysis as well as by in situ hybridization. RESULTS HHV-8 sequences were detected by PCR with confirmation by Southern blot and sequence analysis in 100% of the ISKS samples. Direct sequencing revealed several previously unknown base changes within the 208 bp region from open reading frame 26 (ORF26[208]) of HHV-8 in ISKS. CONCLUSION Ours is the largest known study describing the presence of HHV-8 in iatrogenic KS from immunosuppressed nontransplant patients and provides data of previously unknown sequence variations within the ORF26 of HHV-8 DNA.

Leukemia cutis: Darier's sign in a neonate with acute lymphoblastic leukemia†

Infantile leukemia accounts for only 3% of childhood leukemia. Leukemia cutis occurs in 25% to 30% of infants with congenital leukemia and is more frequently associated with acute myeloid leukemia than with acute lymphoblastic leukemia. We describe an infant in whom hyperpigmented macules that developed when the patient was 2 weeks old demonstrated Dariers sign when he was 4 weeks old. Acute lymphoblastic leukemia, early pre-B-cell type, was diagnosed when the patient was 10 weeks old. Examination at that age revealed 1 to 2 cm, firm, mildly tender nodules clustered on the scalp, face, and extremities, less severe involvement of the trunk, and marked induration of the face and eyelids. Dariers sign was elicited from the less infiltrated truncal lesions. Histologic examination revealed a dense monomorphous infiltrate consisting of pleomorphic, undifferentiated cells. No mast cells were revealed by Giemsa staining. This case is to our knowledge the first reported example of leukemia cutis demonstrating Dariers sign.

Congenital Smooth Muscle Hamartoma Presenting as a Linear Atrophic Plaque: Case Report and Review of the Literature

Abstract: Congenital smooth muscle hamartoma usually manifests as a well‐circumscribed, hyperpigmented plaque, frequently hypertrichotic, on the trunk or extremities. We report such a lesion in a 7‐month‐old girl that presented as a linear, mottled, purplish red plaque appearing In areas to be atrophic, involving her right buttock, posterior thigh and leg, and fifth toe. Although the clinical appearance suggested linear morphea, a biopsy specimen had numerous haphazardly oriented bundles of smooth muscle in the reticular dermis. Masson trichrome staining, smooth muscle specific actin, and electron microscopic studies confirmed the smooth muscle nature of the cells. A diagnosis of smooth muscle hamartoma was made. To our knowledge, this linear clinical presentation has not been described previously.

Mid-dermal elastolysis in an adolescent subsequent to lesions resembling granuloma annulare

First described by Shelley and Wood in 1977, mid-dermal elastolysis (MDE) is a rare acquired disorder in which there is a bandlike absence of elastic tissue limited to the mid-dermis. In their patient, MDE developed in an area previously involved with recurrent episodes of urticaria. We describe a 15-year-old white girl with well-circumscribed, minimally palpable yellow-white plaques and wrinkling diagnosed histologically as MDE in areas clinically diagnosed 5 years previously as granuloma annulare. As in the first described patient, five years elapsed between clearance of the original skin lesions and the clinical appearance of MDE. To our knowledge, we report the first adolescent case of MDE localized to previous sites of lesions clinically consistent with granuloma annulare and propose that MDE represents an abnormal end-stage reaction to multiple processes.

Erythema induratum of bazin as a tuberculid : Confirmation of Mycobacterium tuberculosis DNA polymerase chain reaction analysis

coidal granuloma, granulomatous vasculitis, Kaposis sarcoma, and pseudolymphoma. 1-3 To our knowledge, the literature contains only one observation of a lymphoma occurring under the same circumstances. 4 In our patient, the papulonodular eruption of C LL developed in healing lesions of varicella. The presence of the same clonal rearrangement of the immunoglobu~in heavy chain gene in the skin, blood, and bone maJxow showed that this patient had specific cutaneous lesions of B-cell CLL. PCR clearly identified the presence o f VZV in the cutaneous lesions, whereas it is absent f rom the bone marrow and from the leukocytes of the peripheral blood. It is likely that this represents a nonspecific stimulation of the B lymphocytes.

Papular mucinosis associated with AIDS: response to isotretinoin.

bei mycosis fungoides. Klin Wochenschr 1965;43:10612. 6. Schott HJ, Holzmann H. Polarisationoptischer nachweis yon amyloid-ablerung bei mycosis fungoides. Arch Klin Exp Dermatol 1965;222:632-41. 7. Hashimoto K, Kobayashi H. Histogenesis of amyloid in the skin. Am J Dermatopathol 1980;2:165-71. 8. Maeda H, Ohta S, Saito Y, et al. Epidermal origin of the amyloid in localized cutaneous amyloidosis. Br J Dermatol 1982;106:345-51. 9. Hashimoto K. Diseases of amyloid, colloid, and hyalin. J Cutan Pathol 1985;12:322-33. 10. Ishii M, Kobayashi H, Chanold M, et al. Possible formarion of cutaneous amyloid from degenerative collagen fibers. Acta Derm Venereol (Stockh) 1990;70:37884. 11. Wong C. Cutaneous amyloidoses. Int J Dermatol 1987;26:273-7. 12. Danielsen L, Kobayashi T. An ultrastructural study of cutaneous amyloidosis. Acta Derm Venereol (Stockh) 1973;53:13-21. 13. Virchow R. Zur Cellulose-Frage. Virchows Archiv Pathalog Anat Physiol Klin Med 1854;8:416-26. 14. Hashimoto K, Kumakiri M. Colloid-amyloid bodies in PUVA-treated human psoriatic patients. J Invest Dermatol 1979;72:70-80. 15. Greene I, Cox AJ. Amyloid deposition after psoriasis therapy with psoralen and long-wave ultraviolet light. Arch Dermatol 1979;115:1200-2. 16. Masu S, Hosokawa M, Seigi M. Amyloid in localized cutaneous amyloidosis: immunofluorescence studies with anti-keratin antiserum especially concerning the difference between systemic and localized cutaneous amyloidosis. Acta Derm Venereol (Stockh) 1981;61:381-4. 17. Yoneda K, Watanabe H, Yanagihara M, et al. Immunohistochemical staining properties of amyloids with anti-keratin antibodies using formalin-fixed, paraffin-embedded sections. J Cutan Pathol 1989;16:133-6. 18. Kobayashi H, Hashimoto K. Amyloidogenesis in organlimited cutaneous amyloidosis: an antigenic identity between epidermal keratin and skin amyloid. J Invest Dermatol 1983;80:66-72. 19. Noren PP, Westermark P, Cornwell GG, et al. Immunofluorescence and histochemical studies of localized cutaneous amyloidosis. Br J Dermatol 1983;108:277-85. 20. Teppo A, Kariniemi A, Maury CP. Immunohistochemical demonstration of hyalinosis-associated 90 kD glycoprotein in amyloid deposits of lichen amyloidosus. Am J Clin Pathol 1986;86:175-9.

Primary cutaneous blastomycosis: report of a case acquired by direct inoculation of a bullous pemphigoid lesion.

methoprim-sulfamethoxazole, methyldopa, hydrochlorothiazide, terfenadine, and prednisone. Examination revealed a 1.0 X 0.5 em, erythematous, slightly crusted nodule at the site of a previous vesicle (Fig. 1). A biopsy specimen revealed epidermal hyperplasia and a mixed granulomatous infiltrate with microabscesses and epithelioid histiocytes in the dermis (Fig. 2, A). A periodic acid-Schiff stain showed large broad-based budding yeast forms consistent with blastomycosis (Fig. 2, B). A repeat biopsy specimen for culture failed to grow the organisms. However, a fluorescent antibody test of the original biopsy specimen revealed 2+ staining for Blastomyces dermatitidis, confirming the diagnosis of blastomycosis. Enzymelinked immunosorbent assay for blastomycosis revealed a serum titer of I:8. A chest radiograph was normal. There was no history of a dog bite or previous trauma to the area other than the opening of a blister with a needle. The patients lesion resolved with 4 weeks of ketoconazole therapy (400 mg daily). No signs suggestive of disseminated blastomycosis developed. A repeat chest radiograph was normal, From the Universityof Texas Medical Branch. Reprint requests:Stephen K. Tyring, MD, PhD, Department of Dermatology, University of Texas Medical Brunch, Route G-83, Galveston, TX 77555-0783. JAM ACAD DERMATOL 1994;31 :277-8. Copyright @ 1994 by the American Academy of Dermatology, Inc. 0190-9622

HHV8 and skin cancers in immunosuppressed patients

3.00 + 0 16/54/54352 CASE REPORT