Chih-Ming Hung

Familial Mucinous Nevus

1. Netherton Syndrome; NETH. OMIM #256500. Available at http://www.nbci.nlm.nih.gov/entrez/dispomim.cgi?id= 256500. 2. Powell J, Dawber R, Ferguson D et al. Netherton’s syndrome: increased likelihood of diagnosis by examining eyebrow hairs. Br J Dermatol 1999;141:544–546. 3. Smith V, Anderson G, Malone M et al. Light microscopic examination of scalp hair samples as an aid in the diagnosis of paediatric disorders: retrospective review of more than 300 cases from a single centre. J Clin Pathol 2005;58:1294– 1298. 4. KatoA,FukaiK,OisoNet al.Associationof SPINK5gene polymorphisms with atopic dermatitis in the Japanese population. Br J Dermatol 2003;148:665–669.

Persistent erythema multiforme treated with thalidomide.

Erythema multiforme is a common self-limited disorder that predominantly affects younger individuals. It is characterized by typical iris or target lesions on the skin and mucous membranes. Three clinical subgroups of erythema multiforme have been identified: classical erythema multiforme, recurrent erythema multiforme, and persistent erythema multiforme. By definition, persistent erythema multiforme is characterized by the occurrence of continuous typical and atypical lesions without interruption. We report a 15-year-old boy who developed persistent erythema multiforme for 6 months and responded to treatment with thalidomide.

Cutaneous myxoid fibroblastoma

extended onto the chest wall. There were scattered, erythematous follicular papules on his trunk and groin (Fig 1). Histopathologic sections from a follicular papule showed an acute spongiotic dermatitis with a lymphoeosinophilic infiltrate. The infiltrate was concentrated in the region of a hair follicle, consistent with a follicular hypersensitivity reaction (Fig 2). Immunostaining revealed a CD4 predominant infiltrate in the dermis. Prednisone was started (60 mg/d), which was tapered over 2 weeks. The eruption improved allowing tolerance of the final dose of CTLA4 antibody 3 weeks later. Several trials have been conducted with CTLA4 antibody with or without peptide vaccine. Rash has been reported among patients who received CTLA4 antibody with and without vaccine, suggesting that the skin findings are a result of CTLA4 antibody. The morphologic appearance of the eruption presented here, including the small follicular-based papules and the larger plaques with a predilection for the axillae, does not resemble any of the previously reported cases, highlighting the heterogeneous clinical and pathologic appearance of drug eruptions to CTLA4 antibody. Jean L. Lim, MD, Madhu Dahiya, MD, and Susan Burgin, MD

A solitary facial tumor with erosion on an 81-year-old oriental woman

a solitary, dark red mass measuring 1.5 × 1.3 cm in diameter with erosion on the surface [Figure 1]. No regional lymphadenopathy or systemic symptom was noted. The histopathological examination revealed a well-circumscribed tumor in the dermis with epithelial collarette and adjacent solar elastosis. No invasion into subcutaneous fat was found. The tumor was mainly composed of admixtures of pleomorphic spindle and epithelioid cells with bizarre nuclei and some multinucleated giant cells. Atypical mitotic figures were also present. In addition, immunohistochemical study demonstrated strong cytoplasmic staining for CD68. Stains for cytokeratins, S-100, and desmin were all negative [Figures 2-5].

Azathioprine-Induced Severe Bone Marrow Toxicity-A Report of 3 Cases

Dermatologists have been using azathioprine as a ”steroid sparing agent” for the treatment of various dermatoses including photodermatoses, immunobullous diseases, psoriasis and eczematous diseases. However, in Taiwan, the currently approved indications of azathioprine include only adjunct therapy of renal transplant, systemic lupus erythematosus, severe rheumatoid arthritis, and leukemia. Owing to the off-label nature of azathiopine use in most dermatological practice, clinical vigilance must be taken. We herein report 3 cases of severe bone marrow toxicity after the use of azathioprine and discuss the pathogenesis and suggestive management of this rare complication. The first patient had prolonged pancytopenia lasting for 3 months despite immediate azathioprine withdrawal and 2 consecutive doses of granulocyte colony-stimulating factor. The full blood count of the other two patients had returned to normal one week after treatment. The result of genotyping of three patients to detect most prevalent mutant allele (TPMT(superscript *)3C) was negative.

An Asymptomatic Zosterifrom Skin-Colored Plaque on the Right Upper Back in a 25-year-old Women

From the Department of Dermatoloy, Show Chwan Memorial Hospital and National Taiwan University Hospital* Accepted for publication: October 18, 2005 Reprint requests: Chih-Ming Hung, M.D., Department of Dermatology, Show Chwan Memorial Hospital, 542, Sec. 1 Chung-Shang Rd. Changhua, Taiwan, R.O.C. TEL: 886-4-7256166 ext. 1212 FAX: 886-4-7236226 E-mail: wudermo@yahoo.com.tw Fig. 2 Sparse, fragmented elastic fibers in the middermis (Elastic tissue stain, X100) An Asymptomatic Zosterifrom Skin-Colored Plaque on the Right Upper Back in a 25-year-old Women

Dermatomyositis presenting with erythroderma - A case report

Dermatomyositis is a common autoimmune disorder characterized by both cutaneous and muscle involvement. The characteristic cutaneous features included photosensitivity and the presence of heliotrope erythema and Gottrons papules. A number of uncommon cutaneous manifestations had been reported. We reported a female patient who developed erythroderma after dermatomyositis was diagnosed ten years later.