Keisuke Hagiwara

Mast Cell “Densities” in Vascular Proliferations: A Preliminary Study of Pyogenic Granuloma, Portwine Stain, Cavernous Hemangioma, Cherry Angioma, Kaposi's Sarcoma, and Malignant Hemangioendothelioma

The “densities” of mast cells (MCs) in six kinds of vascular proliferation, pyogenic granuloma, portwine stain, cavernous hemangioma, cherry angioma, Kaposis sarcoma, and malignant hemangioendothelioma (MHE), measured per mm2 were studied using respective specimens prepared with tryptase stain and a personal computer. The average densities of MCs in pyogenic granuloma and MHE were 103.5 ± 25.2/mm2 (n=10) and 106.3 ± 40.2/mm2 (n=10) [mean ± standard deviation (SD)]; that in normal skin was 6.85 ± 4.9/mm2 (n=20) (mean ± SD). is a significant difference [t‐test (p<0.0001) and Wilcoxon‐test (p<0.01)]. The results in portwine stain (n=4), cavernous hemangioma (n=9), cherry angioma (n=4), and Kaposis sarcoma (n=4) were 68.6 ± 28.9/mm2, 105.7 ± 56.9/mm2, 85.3 ± 45.6/mm2, 82.2 ± 28.4/mm2 (mean ± SD), respectively, all of which were greater than that in normal skin by a simple comparison. The results of immunofluorescence microscopy were positive with basic fibroblast growth factor staining in the tissues of pyogenic granuloma, Kaposis sarcoma and MHE. These facts may morphologically indicate a role of MCs in the angiogenesis of these vascular tumors.

Neonatal pemphigus vulgaris

A male newborn with skin erosions was born to a 32‐year‐old woman who was under treatment for pemphigus vulgaris that had been diagnosed 16 months earlier. Antibodies to desmoglein (Dsg)1 and Dsg3 were analyzed by enzyme‐linked immunosorbent assay. Index values of antibodies to Dsg1 and Dsg3 were 49 (normal index values, <14) and 121 (normal index values, <7), respectively. Those findings concluded a diagnosis of neonatal pemphigus vulgaris. No new vesicles or bullae appeared in the newborn after the birth. Non‐corticosteroid ointments produced prompt epithelialization on the erosive lesions. All the eruptions disappeared in 3 weeks. The level of serum anti‐Dsg3 autoantibodies when measured at the 76th day was negative (<5).

Phacomatosis Pigmentovascularis Type IIb Associated with Sturge‐Weber Syndrome and Pyogenic Granuloma

A case of phacomatosis pigmentovascularis (PPV) in a 6‐year‐old girl with Sturge‐Weber syndrome, pyogenic granuloma, and other complications is described. It is relatively rare that a complete form of Sturge‐Weber syndrome was associated with PPV. A review of the literature on PPV, focusing on total number of reported cases and etiological speculations, is presented. To our knowledge, a total of 118 cases of PPV, including the present one, have been reported to date. Regardless of many speculations, the true etiology remains unknown. The average “density” of mast cells (MCs) per mm2 appearing in the central region of the pyogenic granuloma was calculated to be 86.3/mm2 and that in the adjacent nevus flammeus was 37.9/mm2. The “density” of mast cells in pyogenic granuloma separately calculated from ten other cases was 105.5 ± 28.6/mm2 (mean ± SD), compared with that in normal skin, 6.85 ± 4.9/mm2 (n=20). There was a significant difference between the two, indicating that MCs are closely associated with angiogenesis in pyogenic granuloma.

Case of skin injuries due to stings by crown-of-thorns starfish (Acanthaster planci)

A case of skin injuries due to stings by crown‐of‐thorns starfish, Acanthaster planci, in a 53‐year‐old Okinawan woman is reported. She went to a beach to gather shellfish on 8 April 2001 and fell to the ground with her left palm on a crown‐of‐thorns starfish that happened to be close to her. She hurried to the emergency section of our hospital. An emergency doctor sterilized the wound and administered an antibiotic, an analgesic agent and an injection of a tetanus antitoxin. He tried to remove the remaining spines from the palm with great difficulty. Because swelling and subcutaneous indurations of the left palm had persisted thereafter, oral and topical administration of corticosteroid started on 13 April. Physical examination at the dermatology section revealed approximately 10 stab wounds of the left palm with pus, subcutaneous bleeding and many abrasions around them. X‐rays of the left hand showed foreign bodies, 2–10 mm in size, located on the lesions. The patient was treated with a topical injection of 2 mg triamcinolone acetonide (Kenacort‐A), diluted fivefold with 1% Xylocaine, once a week. Some of the foreign body granulomatous lesions improved but pain and subcutaneous indurations persisted in most of the lesions. Because the X‐ray photographs showed many remaining spines, surgical excision to remove them was performed under local anesthesia 3 months after the injury. All the symptoms improved after the operation. Scanning electron microscopic examination of the spines revealed that their tips had fragile lattice‐like structures.

Comparative Studies of the Detection Rates of Leishmania Parasites from Formalin, Ethanol‐fixed, Frozen Human Skin Specimens by Polymerase Chain Reaction and Southern Blotting

In this study, detection rates of Leishmania parasites from human skin were compared among three different types of specimens, formalin‐fixed, ethanol‐fixed, and frozen, by polymerase chain reaction (PCR) and Southern blotting. For this purpose, we used biopsy specimens collected from 19 leishmaniasis patients and performed PCR and Southern hybridization with the probe specific for Leishmania (Viannia) braziliensis complex. Among these 19, 16 specimens were from cutaneous leishmaniasis (CL), one, diffuse cutaneous leishmaniasis (DCL) and 2, mucocutaneous leishmaniasis (MCL) and were formalin‐fixed and paraffin‐embedded. The causative agents for one case of CL and one case of DCL were already identified as L. (Leishmania) complex. Six specimens of CL were preserved in 100% ethanol. Two specimens of MCL were frozen tissues. PCR using the formalin‐fixed and paraffin‐embedded specimens revealed positive bands at 70bp in 9 (47.4%) out of 19 specimens of CL, MCL and DCL. Southern blotting detected the signals in 12 (63.2%) out of the 19. PCR using the 100% ethanol‐fixed specimens revealed positive bands in 4 (66.7%) out of 6, and Southern blotting also detected the signals in 4 (66.7%) out of the 6. PCR and Southern blotting using 2 frozen specimens of MCL were always positive (100%). Although we failed to detect significant differences by Chi‐square test between the results from the formalin‐fixed, paraffin‐embedded specimens and those from 100% ethanol‐fixed ones, we concluded that ethanol‐fixed specimens, convenient for transportation and storage, would be more useful for diagnosis of leishmaniasis by PCR in a developing country.

Squamous Cell Carcinoma Arising from Lupus Vulgaris on an Old Burn Scar: Diagnosis by Polymerase Chain Reaction

A 66‐year‐old Japanese woman with a squamous cell carcinoma (SCC) arising from lupus vulgaris (LV) on an old burn scar on the left lower extremity is described. Ziel‐Neelsen stain of a direct smear from the surface exudate showed acid‐fast bacilli. Repeated culture for tubercle bacilli was negative, probably due to a technical error. The diagnosis of LV was successfully made by polymerase chain reaction (PCR). LV and burn scar are common preceding diseases for SCC. The former is rare in the U.S., Europe, and Japan. We were unable to determine whether only one of the two conditions or a combination of both was the true predisposing factor responsible for the development of this SCC. However, this case may be the first report of SCC arising from coexistent LV and a burn scar in which the diagnosis was confirmed by PCR.

A case of tinea nigra palmaris in Okinawa, Japan

We report a case of tinea nigra on the left palm of a 13‐year‐old girl. She had noticed a pigmented, asymptomatic macule on the left palm approximately 4–5 years prior to her first visit to our hospital. The color of the lesion tended to change before and after a bath; it became lighter after a bath and darkened some time later. Physical examination revealed that the macule was 4 cm × 5 cm in size, dark brown in color and irregularly shaped. Direct potassium hydroxide (KOH) microscopic examination from skin scrapings revealed branched brown hyphae with light brown septa. A fungal culture on Sabourauds agar media produced wet, medium brown, yeast‐like colonies, the surface of which later became black and shiny. A slide culture disclosed light brown, elliptic or peanut‐shaped conidia comprised of one to two ampullaceous cells. Scanning electron microscopic examination of the conidia showed both annellation conidia with lunate bud scars and sympodial conidiogenesis. Using extracted DNA from separately cultured fungi, we performed polymerase chain reaction with the primers specific to Hortaea werneckii. The results showed positive bands. We performed direct sequencing with the DNA segments from the positive bands. The causative fungus in our case was determined to be type C of H. werneckii on the grounds of the base sequences obtained. The final diagnosis of the present case was made as tinea nigra by H. werneckii. We also report a brief survey of all the cases of tinea nigra reported in Japan to date.

Detection of human papilloma virus type 56 in extragenital Bowen's disease

A case of Bowens disease arising on the medial part of the first metatarsal bone of an 81-year-old Japanese woman is described. Histopathologically, proliferation of atypical cells was found throughout the epidermis. Electronmicroscopy revealed virus particles 40-50 nm in diameter in the nuclei of tumour cells at the granular cells just on or below the horny layer. Positive bands were obtained by polymerase chain reaction using a consensus primer of human papilloma virus L1 portion. Sequencing analysis of the amplified DNA revealed the same base sequences and homology as human papilloma virus 56. To the best of our knowledge, this case is the first report in which human papilloma virus 56 was found in a case of extragenital Bowens disease. We consider it important to understand that human papilloma virus 56, often found in cervical lesions, can be detected in extragenital Bowens diseases.

Necrotizing fasciitis caused by Streptococcus pneumoniae

A 69‐year‐old woman presented with shivering and pain in the lower extremities on 5 April 2006; she was referred to the dermatology division of our hospital on the following day with difficulty in walking. She had been suffering from non‐viral, non‐alcoholic liver cirrhosis, and was being treated by the Division of Internal Medicine. Physical examination showed edema in the lower extremities and light purpuras on the groin and legs. Low blood pressure had been observed since admission. Necrotizing fasciitis (NF) was suspected on the basis of the skin symptoms, systemic conditions, and magnetic resonance imaging. During surgical debridement under general anesthesia, cardiopulmonary arrest occurred, and the patient died 12 h after admission. NF, in its early stages, exhibits few skin changes. In order to differentiate it from other skin infections, it is necessary to take into account blood pressure, abnormal systemic conditions, and severe pain out of proportion to its minor skin changes. In the present case, Streptococcus pneumoniae was detected by blood culture. Soft tissue infectious diseases caused by S. pneumoniae, especially NF, are very rare. We have reviewed reported cases of NF caused by S. pneumoniae.

Case of linear immunoglobulin A bullous dermatosis associated with acquired hemophilia

Linear immunoglobulin (Ig)A bullous dermatosis is a rare autoimmune subepidermal bullous dermatosis caused by circulating IgA autoantibodies directed against the antigens at the basement membrane zone. Most linear IgA bullous dermatosis cases are idiopathic, but some are associated with the use of certain drugs, infections, lymphoproliferative disorders, internal malignancies, autoimmune disorders, collagen diseases or, very rarely, other skin diseases, including autoimmune bullous diseases. Acquired hemophilia is also rare; it is a coagulation disease caused by anti‐factor VIII IgG antibodies. Acquired hemophilia has been reported to be associated with malignant tumors, pregnancy or postpartum, drug reactions, collagen diseases such as rheumatoid arthritis, autoimmune disorders, and skin diseases such as psoriasis and pemphigus. We report a case of hemophilia acquired during the course of linear IgA bullous dermatosis and review reported cases of autoimmune bullous dermatoses associated with acquired hemophilia.