Identification of the cause of severe skin infection by Fournier transform infrared spectroscopy: A case of Fournier's gangrene caused by fish bone

Fournier's gangrene (FG) is an infrequent but highly lethal infection. Here we report a 74‐year‐old man who presented with genital swelling and severe malaise. Based on the physical and imaging examination results, the diagnosis of FG was confirmed. Intraoperative findings showed dirty necrosis of soft tissue, and a splinter‐shaped foreign body was found in the perirectal region. The foreign body was thought to be the cause of the condition, and it was analyzed using Fourier transform infrared spectroscopy. We found that the foreign body was a mixture of calcium phosphate and protein, suggesting that the splinter was a bone. Moreover, during the medical interview, the patient mentioned about intake of fish around the time of onset of symptoms. Therefore, to confirm the results of the analysis, DNA was extracted from the foreign body, and genomic PCR with subsequent sequence analysis was performed. The DNA sequence was identical to that of Oncorhynchus kisutch, a salmon that is a very popular food in Japan. On the basis of these findings, we concluded that FG in this case was caused by the penetration into the rectum of an accidentally ingested fish bone. Although some cases of intra‐abdominal abscess due to accidental ingestion of fish bone have been reported, FG caused by fish bone is extremely rare.     

Five cases of necrotizing fasciitis: Lack of skin inflammatory signs as a clinical clue for the fulminant type

Herein, we describe five patients with necrotizing fasciitis (NF) who had variable outcomes and clinical manifestations. At the onset, all patients exhibited purpura with or without blister and ulceration accompanied by severe pain and tenderness in the affected skin. Out of five patients, three lacked inflammatory signs such as redness and heat, and two of the three patients showed fulminant progression and died despite intensive treatments including surgical debridement, antimicrobial therapy, close monitoring and physiological support. Tissue specimens from the patients without skin inflammatory signs showed mild neutrophil infiltration in addition to necrosis from the epidermis to subcutaneous fat, and variable amounts of thrombi. Furthermore, numerous bacteria were detected by Gram stain. By contrast, the remaining two patients with skin inflammatory signs revealed slower progression, and tissue specimens from both patients showed heavy neutrophil infiltration, but bacteria were hardly detected. Therefore, these cases suggest the possibility that the paucity of skin inflammatory signs, such as redness and heat, in NF may be a clinical clue to predict the fulminant type.     

Eccrine Syringofibroadenoma Associated with Bowen's Disease: A Case Report and Review of the Literature

Eccrine syringofibroadenoma (ESFA) is a rare, benign adnexal neoplasm which usually manifests as a solitary nodule on the extremities of elderly patients. Few case reports have described an association between ESFA and carcinomas including squamous cell carcinoma, porocarcinoma, and basal cell carcinoma. A 66-year-old male presented with a pruritic, erythematous brownish solitary plaque with crusted and verrucous surface on the extensor side of the right thigh. The lesion developed 6 to 7 years ago, and had been growing slowly. Biopsy revealed anastomosing epithelial strands which were composed of 2 areas: the upper area consisting of dysplastic cells with prominent nucleoli and abundant mitoses, and the lower area consisting of oval and round cells, and occasionally small luminal ducts. Dysplastic cells comprised almost the entire epidermis but did not invade into the dermis. Benign syringofibroadenomatous lesion surrounded the dysplastic cells in the lowermost portion of the epidermis. Although it is still unclear whether ESFA undergoes malignant transformation or it is a reactive change to carcinoma, complete excision should be performed to prevent malignant transformation with unknown risk.     

A Case of Sweet's Panniculitis Associated with Spinal Metastasis from Prostate Cancer

Sweet''s panniculitis is a rare variant of Sweet''s syndrome in which neutrophilic infiltrate can be found either in the subcutaneous fat or in both the dermis and the subcutaneous tissue. Due to the rarity of this entity, the association between Sweet''s panniculitis and malignancies is inconclusive, but cases of Sweet''s panniculitis have largely been associated with hematological malignancies. Herein, we present a case of Sweet''s panniculitis accompanied by bone metastasis from prostate cancer. Clinicians should be aware that Sweet''s panniculitis may be associated with malignancies of solid organs.     

A Case of Gardner's Syndrome Associated with Desmoid Tumor

Desmoid tumors are uncommon benign neoplasm of the fibroblasts. They occur rarely in the general population, but they are comparatively common in patients with familial polyposis coli with or without other elements of Gardner''s syndrome. Herein, we report a 16-year-old woman with Gardner''s syndrome complicated by desmoid tumors on the right subscapular area.     

Cowden's Disease: Report of the First Case in a Chinese

A 19-year-old Chinese female presenting mucocutaneous manifestations including facial trichilemmomas, oral papillomatosis, and acral keratosis was diagnosed, according to the criteria proposed by Salem and Steck, as a definite case of Cowden's disease, although no members of her family were affected. The cutaneous reticulohistiocytic granuloma and congenital nystagmus of eyes that occurred in this case were new combinations in the syndrome. To the best of our knowledge, this is the first documented case of Cowden's disease in a Chinese.     

A Case of Progressive Systemic Sclerosis Associated with Sarcoidosis and Esophageal Adenocarcinoma

A 50-year-old Japanese female with progressive systemic sclerosis (PSS, CREST syndrome) is reported. During treatment for PSS, she was diagnosed by clinical and laboratory findings as having sarcoidosis, which was confirmed by histological examination of the skin and lymph nodes in July of 1991. She complained of back pain in August of 1991. Reflux esophagitis and Barrett's esophagus, found by endoscopy, progressed into a well-differentiated tubular adenocarcinoma. This is a very rare case of PSS associated with sarcoidosis and esophageal adenocarcinoma developing from Barrett's esophagus.     

Recurrent Diffuse Neurofibroma of Nose Associated with Neurofibromatosis Type 1: A Rare Case Report with Review of Literature

Diffuse neurofibroma is an unusual variant of neurofibroma with the head and neck being the common sites of involvement. It is benign in nature and spreads superficially and has many ectatic blood vessels. Histologically it is similar to conventional neurofibromas except for a few peculiar distinguishing features. We report a case of a 14-year-old boy who presented with a diffuse recurrent painless swelling over the dorsum of the nose with the clinical stigmata of neurofibromatosis. Microscopy revealed a diagnosis of diffuse neurofibroma with a few foci showing differentiation towards Meissner''s type of tactile corpuscles. It is important to recognize this entity as it has a tendency to recur, yet hardly ever become malignant and is almost always associated with neurofibromatosis type 1.     

Sister Joseph's Nodule: A Case Report and Review of the Japanese Literature

We report a case of metastatic carcinoma on the umbilicus, which is known as Sister Joseph's nodule. A 65-year-old man with the chief complaint of a painful nodule on the umbilicus was refered to our hospital. Histopathological examination revealed moderately differentiated tubular adenocarcinoma in the dermis. Endoscopic examination found gastric cancer of Borrmann type IV. The umbilical lesion represented a metastasis from the gastric cancer. We reviewed 80 cases of metastatic carcinomas to the umbilicus that have been reported in the Japanese literature.     

Prurigo Nodularis Associated with Advanced Gastric Cancer: Report of a Case

In a male Japanese patient, prurigo nodularis (PN) appeared in association with gastric cancer. The cutaneous pruriginous lesions dramatically improved soon after total gastrectomy without any treatment for the skin lesions. Peripheral eosinophilia seen before the operation also rapidly disappeared. These data suggest that some cytokines involved in gastric cancer might have played an important role in the development of PN in our patient.     

Intralymphatic Histiocytosis Associated with Osteoarthritis: A Case Report

Intralymphatic histiocytosis (ILH) is a rare cutaneous condition with uncertain pathogenesis. It is characterized by dilated lymphatic vessels that contain histiocytes within their lumina. Although the etiology of ILH remains unknown, it has been associated with various inflammatory and neoplastic diseases, such as rheumatoid arthritis (RA), reaction to metal joint implants and Merkel cell carcinoma, breast cancer and colon cancer. An 83-year-old female presented with an erythematous patch on the left forearm that had appeared six months previous. She had suffered from osteoarthritis (OA) and the cutaneous lesion was located in the vicinity of the affected joint. Skin biopsy from the lesion showed dilated dermal vessels and some ectatic vessels that contained many mononuclear histiocytes. Based on the clinical and histopathological findings, we diagnosed her with ILH with OA. Two sessions of intralesional triamcinolone acetonide injection (5 mg/ml) were administered to treat the skin lesion, which gradually improved over a period of a few months. We here report a rare case of ILH associated with degenerative OA.     

A Case Report on the Dermoscopic Features of Spark's Nevus

Spark''s nevus is a compound word composed of Spitz nevus and Clark''s nevus. It is one of the combined melanocytic nevi which is more common in female and usually presents as a sharp circumscribed hyperpigmented macule on the lower extremities. On histopathologic findings, both cytologic features of Spitz nevus characterized as large spindle or epithelioid melanocytes containing large nuclei with abundant cytoplasm, and architecture of Clark''s nevus characterized as elongation of rete ridges, bridging of the nests, concentric and lamellar fibrosis can be seen. A 24-year-old female presented with an asymptomatic, solitary, dark-brown-colored papule surrounded by brownish patch that looked similar to dysplastic nevus or malignant melanoma on the buttock. On dermoscopic examination, it showed brown-to-black globules, diffuse homogenous pigmentation with blue-white structures, and a surrounding brownish reticular pattern that faded away. On histopathologic findings, overall asymmetrical structure, epithelioid large melanocytes containing large nuclei with abundant cytoplasm, and Kamino body were seen in the central portion. Also, lentiginous hyperplasia, bridging of the nests composed of melanocytes containing foamy cytoplasm, concentric and lamellar fibrosis along with the elongation of rete ridge, and perivascular lymphocytic infiltration were seen in the peripheral portion. The diagnosis of Spark''s nevus was made. Following its definition, this combined nevus is diagnosed histopathologically, but the clinicodermoscopic features have not been well described. Herein, we report a case of Spark''s nevus in which dermoscopy was helpful for differentiating it from malignant melanoma.     

Sjögren-Larsson Syndrome Associated with the Dandy-Walker Malformation: Report of a Case

Abstract: The Sjdgren-Larsson syndrome is defined as the association of ichthyosis, spastic diplegia or tetrapiegia, and mental retardation. Although most cases described have come from the Swedish county of Vasterbotten, isolated reports from the United States and elsewhere do exist Numerous neurologic abnormalities have been reported with this syndrome, including speech defects, seizures, and pyramidal tract disorders. Abnormal ophthaimologic findings are not rare. Prevlous studies with computerized tomographic (CT) scans have revealed no abnormalities of cranial anatomy. We report a 6-year-old boy with Sidgren-Larsson syndrome who had a Dandy-Walker malformation as demonstrated by diagnostic imaging by CT and magnetic resonance imaging scans. This case illustrates that developmental defects of the central nervous system can also occur in association with Sjdgren-Larsson syndrome.     

A Case of Urticarial Vasculitis Associated with Macroglobulinemia (Schnitzler's Syndrome)

We report a case of urticarial vasculitis associated with macroglobulinemia in a 50-year-old woman. She suffered from intermittent fever with wheals persisting for more than 24 hours. Skin histology revealed perivascular neutrophilic infiltrates with nuclear dusts. Laboratory study demonstrated leucocytosis and a high serum IgM level (2350 mg/dl) with an M component. No overt lymphoid proliferative disease was found. As far as we know, this is the first report of such a case in Japan. Predonizolone and nonsteroidal anti-inflammatory drugs have proved transiently effective for controlling the urticaria, but not for the IgM gammopathy.     

Linear and Whorled Nevoid Hypermelanosis: Report of a Case Associated with Cerebral Palsy

Abstract: A child had cerebral palsy and linear and whorled nevoid hypermelanosis on the right side of his body. He had spasticity and wasting in both lower limbs, with electroencephalographic changes and brain abnormalities on computerized tomographic scan. Chromosomal study of peripheral blood leukocytes found a normal male karyotype. The pathogenesis of this condition is still unclear.     

A Case of a Surviving Male Infant with Incontinentia Pigmenti

Incontinentia pigmenti (Bloch-Sulzberger''s disease) is an X-linked dominantly inherited disorder which is usually lethal in hemizygous males, but rarely found in male infants. It can be explained by the presence of an extra X chromosome (Klinefelter''s syndrome), hypomorphic mutations, and somatic mosaicism. We herein report a rare case of incontinentia pigmenti with typical course of skin manifestation in normal karyotype (46, XY) male infant.     

不典型水痘并发噬血细胞综合征1例

患者男,16岁。全身红斑、丘疹和水疱5d,发热2d。实验室检查示:白细胞和血小板减少、肝酶升高、高甘油三酯、高铁蛋白、水痘带状疱疹病毒IgM抗体阳性。骨髓穿刺细胞学检查示:网状细胞增生,并多见噬血现象。诊断:不典型水痘并发噬血细胞综合征。     

Woolly-Hair Nevus: Report of a Case Associated with a Verrucous Epidermal Nevus in the Same Area

We cared for a patient with woolly-hair nevus (WHN) with hair of reduced diameter seen as oval shaped on transverse section. Scanning electron microscopy revealed a longitudinal canal along the length of the hair shaft. Although the association of WHN and epidermal nevus has been described previously, this report of their localization in the same site is, to our knowledge, the first in the literature.