We report the clinico-pathological features of 7 cases of dermatofibrosarcoma protuberans. The tumor size were from 1.0 to 27cm in diameter. Local recurrence was seen in two cases under the follow up period of 4 months to 22 years. Histologicaly, the tumors of all cases were composed of spindle-shaped cells, and arranged in a storiform pattern. Immunoperoxydase staining with anti-CD34 (human progenitor cell antigen) and anti-factor XIIIa antibodies showed that CD34 was positive and XIIIa was negative in all cases. Clinically, early diagnosis and wide surgical excision are considered to be important to evade recurrence.
Journal Article Late development of antidesmoglein 1 antibodies in pemphigus vulgaris: correlation with disease progression Get access S. Miyagawa, S. Miyagawa Department of Dermatology, Nara Medical University, Kashihara City, Nara 634, Japan Search for other works by this author on: Oxford Academic Google Scholar M. Amagai, M. Amagai Department of Dermatology, Keio University School of Medicine, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar T. Iida, T. Iida Department of Dermatology, Nara Medical University, Kashihara City, Nara 634, Japan Search for other works by this author on: Oxford Academic Google Scholar Y. Yamamoto, Y. Yamamoto Department of Dermatology, Nara Medical University, Kashihara City, Nara 634, Japan Search for other works by this author on: Oxford Academic Google Scholar T. Nishikawa, T. Nishikawa Department of Dermatology, Keio University School of Medicine, Tokyo, Japan Search for other works by this author on: Oxford Academic Google Scholar T. Shirai T. Shirai Department of Dermatology, Nara Medical University, Kashihara City, Nara 634, Japan Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 141, Issue 6, 1 December 1999, Pages 1084–1087, https://doi.org/10.1046/j.1365-2133.1999.03209.x Published: 01 December 1999
Sir, One agent that induces pemphigus is d‐penicillamine, which contains the sulphydryl radical.1 We report a case of pemphigus induced by bucillamine, a related thiol drug. A 62‐year‐old man with severe arthralgia had been treated with oral bucillamine for rheumatoid arthritis during the 6‐month period before presentation at our clinic. He exhibited two types of pemphigus lesion on his neck, legs and chest (Fig. 1). One type consisted of small, pigmented, vesiculated, and erythematous macules, similar to pemphigus foliaceus (PF), and the other type involved deep erosions as seen in pemphigus vulgaris (PV). Mucosal lesions were not observed. Laboratory tests showed positive antinuclear antibody (1 : 320, nucleolar type), low serum total protein (5.9 g dL−1; normal range, 6.5–8.0), elevated serum total cholesterol (396 mg dL−1; normal range, 130–230), and proteinuria (8.7 g daily). A renal biopsy showed typical features of membranous glomerulonephritis, which was most probably a side‐effect of bucillamine. A biopsy specimen taken from a lesion on the chest revealed acantholysis at the granular, spinous and suprabasal layers in the same section (Fig. 2). Direct immunofluorescence revealed intercellular deposition of IgG and C3 throughout the epidermis. No deposition of immunoglobulin or complement was observed at the basement membrane zone. Indirect immunofluorescence (IIF), using normal human skin as substrate, showed IgG reactivity in the nuclei of keratinocytes, reflecting a positive serum antinuclear antibody. No apparent IgG anti‐intercellular antibodies were detected. The patient's serum reacted with neither epidermal desmoglein 1 (Dsg1) nor desmoglein 3 (Dsg3) in immunoblot analysis using normal human epidermal extracts. The indices of a novel enzyme‐linked immunosorbent assay (ELISA) using baculovirus‐expressed recombinant desmogleins as antigen2,3 were 55·2 (positive) and 15·2 (grey zone; 10–20) for circulating anti‐Dsg1 and anti‐Dsg3 antibodies, respectively. The skin lesions rapidly but incompletely improved 1 month after discontinuation of bucillamine. The skin lesions disappeared completely after a few weeks of oral prednisolone at 40 mg daily for the treatment of nephrotic syndrome.
