Tetsuri Matsumura

Kikuchi's disease and the skin: case report and review of the literature

We report scattered indurated erythematous lesions that presented in an 18‐year‐old Japanese man with Kikuchis disease (KD; histiocytic necrotizing lymphadenitis). A skin biopsy showed a proliferation of histiocytes and abundant nuclear debris without the presence of neutrophils, which is characteristic of KD. The specific dermatological and pathological details of KD have been yet to be fully described. In order to assess the typical skin features of KD better, we have reviewed all the previously well‐documented reports of such lesions. As the clinical and histopathological cutaneous findings in KD are so heterogeneous, it is important that scattered indurated erythematous lesions should be included as one of the possible cutaneous manifestations of this disease.

Squamous cell carcinoma developing in a 12‐year‐old boy with nonHallopeau–Siemens recessive dystrophic epidermolysis bullosa

Summary We report a 12‐year‐old boy with nonHallopeau–Siemens recessive dystrophic epidermolysis bullosa (nHS‐RDEB) who developed two skin lesions of squamous cell carcinoma (SCC) on the left foot. The incidence of SCC in nHS‐RDEB is much lower than in the HS‐RDEB subtype. Furthermore, this boy is the youngest among 92 previously described patients with DEB to develop SCC. This study emphasizes the importance of vigilance in monitoring the possible development of SCC in DEB patients regardless of age or clinical severity.

Ichthyosis follicularis with alopecia and photophobia in a mother and daughter

A mother and daughter having ichthyosis follicularis with alopecia and photophobia (IFAP) are reported, with histopathological and electron microscopic findings. We have followed the clinical course of the mother for 26 years since she was 5 years old, and the daughter since birth. They have had almost all the classical and some of the minor symptoms of IFAP, including severe photophobia, extensive non‐inflammatory follicular hyperkeratosis, generalized non‐scarring alopecia, hyperkeratosis of the extensor aspect of the four extremities, nail deformity and recurrent cheilitis. In addition, their facial appearance greatly resembles that of previously reported patients. A consistent feature in the mother was florid keratotic inflammatory eruptions on the genital region during each of her pregnancies, which rapidly improved after the delivery. Skin biopsy of the genital lesion showed marked acanthosis with dyskeratosis and spongiotic changes. The electron microscopic examination of diseased skin showed damaged desmosomes with spongiosis. No obvious changes were found in normal appearing skin.

Detection of Selenium in Generalized and Localized Argyria: Report of Four Cases with X‐ray Microanalysis

Electron microscopic and X‐ray microanalytic studies were performed on four cases of argyria; one generalized and three localized. Deposition of electron dense granules was predominantly found on elastic fibers and around basal laminas of secretory portions of eccrine glands, although the amount of deposition was much less in the case of generalized argyria. In all four cases, X‐ray microanalysis revealed that the depositions consisted mainly of silver, selenium, and sulfur. The importance of selenium in the detoxification of heavy metals was discussed.

Two cases of pyoderma gangrenosum complicated with nasal septal perforation

We report two patients with pyoderma gangrenosum complicated with nasal septal perforation. An 18‐year‐old woman and a 65‐year‐old man had typical lesions of pyoderma gangrenosum on the legs that responded well to oral prednisolone. Both patients complained of mild nasal discharge, and nasal fibroscopy revealed nasal septal perforation. Biopsy of the nasal lesions showed an active inflammatory infiltrate, mainly of neutrophils. Systemic investigations failed to show any pulmonary or renal lesions of Wegener’s granulomatosis. Cytoplasmic immunofluorescent pattern antineutrophil cytoplasmic antibody was negative. In both cases, intense neutrophilic infiltration was observed not only in skin lesions but also in nasal lesions, which may indicate that the nasal lesions had a pathogenesis in common with the skin lesions.

Serological and immunohistochemical detection of human herpesvirus 8 in Kaposi's sarcoma after immunosuppressive therapy for bullous pemphigoid

Kaposis sarcoma (KS) developed in an 87‐year‐old human immunodeficiency virus‐negative woman from Hokkaido island 4 months after oral administration of prednisolone for the treatment of bullous pemphigoid (BP), and rapidly disseminated to almost the entire body within 2 months. The open reading frame (ORF) 59 and ORF73 proteins encoded by human herpesvirus 8 (HHV‐8) were detected immunohistochemically in the nuclei of the tumour cells of KS. The protein coded by ORF73, latent protein, was detected in most of the nuclei of the tumour cells, but only a few tumour nuclei were positive for the ORF59 protein, a lytic protein expressed during active infection. The antibodies against both lytic and latent proteins of HHV‐8 were detected retrospectively in the serum 4 months before the appearance of KS and before prednisolone therapy had been started. Immunosuppression associated with the treatment for BP possibly activated latent HHV‐8 infection and induced the development of KS.

Adenoid Cystic Carcinoma of the Skin: -An Immunohistochemical and Ultrastructural Study-

Adenoid cystic carcinoma of the skin was studied. Histologically, tumor cells were arranged in a tubular and a cribriform pattern, mainly in the reticular dermis. Immunohistochemically, epithelial membrane antigen was reactive with the tumor cells, but S‐100 protein, vimentin, and carcino‐embryonic antigen were not. On electron microscopy, we confirmed the findings of previous reports; tumor cells were arranged to form luminal structures; most of them were pseudolumina containing fine mucin granules, basal laminae, and collagen fibers, but some were true lumina with numerous microvilli and junctional complexes. New findings of this study were bizarre‐shaped, electron‐dense, net‐like structures within the true lumina which were considered to be a type of mucin.

Three‐dimensional reconstruction in dermatopathology – A personal computer‐based system

The purpose of the present study was to build a system for three‐dimensional (3D) reconstruction of dermalopathological specimens using an easily available personal computer and graphic programs. A stereogram was generated by projecting thirty serial sections transferred into a Macintosh computer. The quality of the 3D images obtained by this method were high enough to show the net‐like structure of rete ridges in normal epidermis, the antler‐like branching of dermal papillae in seborrheic keratosis, and the bulge‐like proliferation of tumor nests in basal cell carcinoma. This method was also helpful to assess oblique sectioning of the specimens, which sometimes produces a strange appearance in dermatopathological sections. The method presented here would not only help to better understand the 3D structures of dermatopathological specimens, but could also be used to examine 3D patterns of tumor invasion, which may contribute to the differential diagnosis of malignant tumors.

Simultaneous occurrence of three squamous cell carcinomas in a recessive dystrophic epidermolysis bullosa patient.

Sir, Squamous cell carcinoma (SCC) is a life-threatening and frequent complication in recessive dystrophic epidermolysis bullosa (RDEB) (1). SCC in RDEB usually develops between the fourth and fifth decades and predominantly on the extremities (2). We have identified three independent SCCs occurring on the extremities of an RDEB patient who had failed to undergo regular dermatological consultations.

Localization of annexin I (lipocortin I, p35) mRNA in normal and diseased human skin by in situ hybridization.

Annexin I is a calcium- and phospholipid-binding protein that is involved in the regulation of cellular differentiation. The aim of the present study was to determine the localization of annexin I mRNA expression in normal and diseased human skin. In situ hybridization with a specific digoxigenin-labelled RNA probe was used throughout. We detected no annexin I mRNA, signals in basal and suprabasal cells of normal epidermis, but positive signals were evident in the sudoriferous ducts. Annexin I mRNA expression was detected in the keratinizing squamous cells in keratotic type seborrhoeic keratosis and in keratinocytes at the periphery of the horn pearl in well-differentiated squamous cell carcinoma. Positive signals were also seen at the border between involved and noninvolved skin in psoriasis vulgaris and in dyskeratotic epidermal keratinocytes in keratosis follicularis Darier. By contrast, no annexin I mRNA signals were detected in tumour cells in basal cell carcinoma. The present results suggest that annexin I expression is related to, and may play a role in, keratinization disorders.