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Buruli ulcer is the third most common mycobacterial infection next to tuberculosis and leprosy caused by Mycobacterium ulcerans. Although it affects the skin, subcutaneous tissues, muscles and sometimes bones, there is no reliable evidence to determine the extent of debridement. We present here a case of Buruli ulcer treated successfully with a preoperative mapping biopsy procedure, which had been self-medicated with occlusive dressing. Because Buruli ulcer is accompanied by subtle pain, patients and clinicians tend to initially treat the ulcer with occlusive dressing therapy, which leads to the misdiagnosis of Buruli ulcer as a common bacterial infection only judging from bacterial culture of the surface of the ulcer. We propose the efficacy of mapping biopsy for treating Buruli ulcer., (© 2017 Japanese Dermatological Association.)

Digital mucous cysts (DMC) are common benign myxoid cysts typically located on the fingers and toes. Recently, dermoscopic patterns of DMC were reported. However, only a small number of cases were described in the published work; therefore, information on this topic is scarce in the published work. We investigated dermoscopic patterns of histopathologically diagnosed DMC. In total, 23 cases were enrolled in this study. Polarized dermoscopy revealed vascular patterns in 13 cases (56.5%), with arborizing vascular patterns, dotted vessels, linear vessels and polymorphous vessels in eight (34.8%), three (13.0%), one (4.3%) and one (4.3%) case, respectively. Red-purple lacunas, ulceration, nail dystrophy and white shiny structures were detected in five (21.7%), two (8.7%), seven (30.4%) and six cases (26.1%), respectively. We report the largest case series regarding dermoscopic features of DMC to date. Dermoscopy can be used as a helpful adjuvant and non-invasive tool in the diagnosis of DMC., (© 2017 Japanese Dermatological Association.)

Necrolytic acral erythema (NAE) is a recently recognized dermatosis almost exclusively associated with hepatitis C virus (HCV) infection, and closely related to zinc deficiency. We present the case of a 60-year-old man with a history of psoriasis and chronic HCV infection, who developed new lesions of NAE extending from previous elephantine psoriatic plaques on bilateral lower legs. According to previous reports, resolution of NAE has been successfully achieved by treatment of the underlying HCV infection, or the use of oral zinc therapy. Our patient exhibited good response to zinc therapy. By reporting this case, we would like to raise the awareness of physicians to this unique acrally distributed dermatosis, which is distinct from psoriasis by its pathological feature of aggregated necrotic keratinocytes and its good response to zinc therapy rather than topical corticosteroids., (© The Author(s) 2016.)

Circumscribed palmar or plantar hypokeratosis (CPH) is defined clinically as well-circumscribed areas of erythematous eroded skin mostly over thenar or hypothenar eminences of the palms and less commonly soles. Histologically, lesions demonstrate a characteristic abrupt drop-off in the cornified layer leading to broad areas of hypokeratosis. In the original description in 2002, Perez et al favored these lesions to be a distinctive epidermal malformation. Since then, some reports implicate trauma; however, the exact etiology remains uncertain. The authors present 11 cases in which the histologic changes of CPH are present as an incidental finding that they favor to represent a reaction pattern to trauma. The changes of CPH overly traumatized neoplasms [ie, a poroma, squamous cell carcinoma (2), dermatofibroma], verruca vulgaris (3), inflammatory processes (lichen amyloid and granulomatous inflammation secondary to a ruptured infundibular cyst), and scar and fibrosing granulation tissue from previous procedures. Classic clinical findings of CPH are not present. The changes most commonly but not exclusively occur on acral skin (8/11), both volar (4) and dorsal skin (4). Six patients are female and 5 are male. Ages range from 21 to 87 years (median 64 years). The authors propose that, in some instances, the histologic changes of CPH are present as a secondary phenomenon and represent a reaction pattern to trauma. They name this finding of secondary histologic change of CPH as "pseudo-CPH" to distinguish it from primary lesions of CPH ("primary CPH") with classic clinical and histologic features.

Background: Dermatophytosis is a very common skin infection with a broad clinical spectrum. Biopsies are often used to confirm the diagnosis, especially when the clinical presentation is unusual. Not uncommonly, organisms are hard to find even with periodic acid-Schiff stains. Polymerase chain reaction (PCR) for dermatophytes can be used in such cases., Objectives: To test a new PCR assay allowing species identification of dermatophytes on paraffin-embedded biopsies, and to reassess histopathological criteria for diagnosis of dermatophytosis., Methods: In total, 121 biopsies of 92 patients with clinical suspicion of tinea were included. In 42 samples the clinical diagnosis had been confirmed histopathologically, and in 79 no fungal elements had been identified. PCRs targeting the internal transcribed spacer (ITS)2 region of dermatophytes were performed on the biopsies with subsequent sequencing. Sections were reassessed for the presence/absence of hyphae/spores, pattern and composition of infiltrate, and epidermal/follicular changes. Patient charts were reviewed for clinical data., Results: The new ITS2 PCR assay detected 94% of the dermatophyte infections (compared with 79% identified by microscopy). Trichophyton rubrum was the dominant species (89%), and other species identified were Trichophyton verrucosum (2%), Microsporum canis (4%), Epidermophyton floccosum (2%) and Trichophyton interdigitale (4%). In particular, infections with T. interdigitale and manifestations with prominent spongiosis were not diagnosed histologically. Intracorneal neutrophils, which have been emphasized as a histopathological clue to dermatophytosis, were present in only 46% of PCR-positive samples., Conclusions: Molecular species identification of dermatophytes via ITS2 PCR can easily be implemented in a routine dermatopathology setting. It is fast and highly specific and improves the sensitivity of histopathological diagnosis of dermatophytosis., (© 2015 British Association of Dermatologists.)

Pachyonychia congenita (PC) is a rare genodermatosis caused by a mutation in keratin genes, which can lead to hypertrophic nail dystrophy and focal palmoplantar keratoderma (predominantly plantar), amongst other manifestations. Painful blisters and callosities, sometimes exacerbated by hyperhidrosis, are major issues that can have a significant impact on patient quality of life. Many alternative treatments for this condition have been applied with variable and partial clinical response, but a definitive cure for this disease has yet to be discovered. After obtaining informed consent, two patients with genetically confirmed PC type 1 were treated with plantar injections of botulinum toxin type A. Both patients showed a marked improvement in pain and blistering with an average response time of one week, a six-month mean duration of effectiveness, and a lack of any side effects or tachyphylaxis., (© 2015 Wiley Periodicals, Inc.)

Circumscribed palmar or plantar hypokeratosis was first described by Pérez et al in 2002 as a unique entity of the skin in which they reported 10 patients who presented with well-circumscribed areas of erythematous depressed or eroded skin mostly over the thenar or hypothenar eminences of the palms and less commonly on the soles. Histologically, the lesions demonstrated an abrupt drop-off in the cornified layer resulting in a broad area of hypokeratosis. Pérez et al hypothesized that these lesions were a distinctive epidermal malformation. There have been several reports since, some of which implicate trauma as an etiologic agent; however, the exact etiology remains unclear. The authors present the first case of circumscribed palmar or plantar hypokeratosis on a nonacral site (chest of a 63-year-old man) with novel histological features, including granular parakeratosis and evidence of trauma (subepidermal fibrin and ulcerations).

Background and Objective: Neurofibromatosis (NF) is a neurocutaneous disorder with seven subtypes;segmental NF-1 is characterized by neurofibromas and or café-au-lait macules in one or more dermatomes, primarily the thorax and abdomen. We present the rare case of a 36-year-old African-American female with isolated, multiple soft nodules in a linear configuration on the plantar and dorsal surfaces of the left foot., Conclusion: This is the first report of isolated segmental NF-1 in a unique linear pattern confined to the L5 dermatome.

Background: Necrolytic acral erythema (NAE) is considered a cutaneous sign of hepatitis C virus infection. Its exact pathogenesis is still not fully understood, with some reports about decreased serum zinc levels but none about its level in the skin., Objectives: To assess skin (lesional and perilesional) and serum zinc levels in patients with NAE and compare them with levels in control subjects., Methods: Fifteen patients with NAE and 10 healthy controls were included in this study. Assessment of zinc level, in serum by graphite furnace atomic absorption spectrophotometry and in lesional and perilesional skin biopsies by flame atomic absorption spectrometry, was done in all subjects. Re-evaluation of serum and lesional skin zinc level was done after oral zinc treatment., Results: Mean±SD zinc levels were significantly lower in patients (serum 0·44 ± 0·13 mg L(-1) ; lesional skin 42·6 ± 18·9 mg L(-1) ; perilesional skin 32·5 ± 17·2 mg L(-1) ) than controls (serum 1·17 ± 0·29 mg L(-1) ; skin 100·1 ± 2·77 mg L(-1) ), with a positive correlation between lesional and perilesional skin zinc (r = 0·91, P < 0·01). Oral zinc supplementation significantly increased serum and skin zinc levels (by 159% and 4%, respectively; P < 0·05)., Conclusions: NAE is associated with decreased serum and skin zinc levels. Oral zinc supplementation corrects decreased levels of plasma and skin zinc much earlier than the desired clinical benefits appear., (© 2010 The Authors. Journal Compilation © 2010 British Association of Dermatologists.)

Background: A callus is a local thickening of skin, characterized by accelerated keratinization and a reduced rate of desquamation. However, the mechanism of callus formation is not fully understood., Objectives: To evaluate the expression patterns, in callused skin, of genes that are implicated in keratinization and adhesion/desquamation., Methods: Samples of skin from the dorsum of the foot (DF), centre of the plantar arch (CP) and anterior aspect of the heel (AH) were obtained from fresh cadavers, and protein and gene expression were determined by immunohistochemistry and reverse transcription-polymerase chain reaction, respectively., Results: The stratum corneum in the DF showed a splitting phenotype by conventional haematoxylin and eosin staining, while the stratum corneum was normal in the AH. Cells of the stratum corneum in the AH were nonsquamous. Expression of cornification-related molecules including involucrin, filaggrin, caspase 14 and calcium-sensing receptor was higher in the AH. Similarly, expression of adhesive proteins such as corneodesmosin, desmoglein 1 and desmocollin 1 was increased in the AH. However, protease-activated receptor 2 expression was reduced in the stratum granulosum in the AH. The number of proliferating cells in the stratum basale was significantly increased in the AH, compared with the DF and CP., Conclusions: Our data suggest that calluses form as a result of hyperproliferation and incomplete differentiation of epidermal keratinocytes, and increased expression of adhesion molecules., (© 2010 The Authors. Journal Compilation © 2010 British Association of Dermatologists.)

Palmoplantar keratodermas (PPK) are a diverse group of disorders. We report a boy with PPK, grayish-blue hyperkeratotic lesions on the lips and peri-oral area, opacities on the lower portions of the corneas, mutilation of his right auricle and many other skin lesions.

Background: Necrolytic acral erythema (NAE) is a distinctive skin lesion that was first described in 1996 with only few cases being reported, mostly from Egypt. It is unique in its acral distribution and exclusive association with hepatitis C virus (HCV) infection., Methods: Twenty-three patients (mean age 41.7 +/- 11.5 years; M:F 10:13) with clinical features consistent with NAE were enrolled in a 3-year period. Five of those were known HCV-infected individuals and 18 were referred by the dermatologist for evaluation and HCV screening. Liver function tests, serum zinc, hepatitis B markers, HCV antibodies and HCV-RNA were tested. All patients were subjected to skin biopsy examination; five lesional biopsies were selected for electron microscopic examination and capillary endothelium was scanned for hepatitis C viral particles. An additional five patients were subjected to detection of HCV-RNA in their skin biopsies by polymerase chain reaction. All patients received oral zinc sulfate supplementation while interferon-alpha therapy combined with ribavirin was available for four patients., Results: Most NAE patients were adults (91.3%) and the skin lesions were predominantly chronic (78.3%), with affection of the dorsa of toes and/or feet in all cases. Skin biopsies showed hyperkeratosis, psoriasiform epidermis and upper epidermal necrosis. Electron microscope examination demonstrated clumped tonofilaments in the keratinocytes, yet HCV-RNA could not be detected in the skin lesions of examined cases. Interferon-alpha combined with ribavirin caused regression of skin lesions in three patients and complete clearance in one patient. Some improvement was induced by oral zinc administration., Conclusion: Necrolytic acral erythema is considered to be a cutaneous marker for HCV infection. The majority of patients are diagnosed by dermatologists. Therefore, improved awareness of this cutaneous lesion should prompt early diagnosis and treatment of HCV, which should in turn cure the lesion and prevent progression of liver disease.

Recurring scarlatiniform scaled erythema of Féréol-Besnier is a rare disease characterized by recurrent episodes of a prodromal phase with general malaise, head and muscle aches, gastrointestinal complaints and fever followed by an erythematous rash leading to extensive desquamation of the involved skin. It exists in a generalized and localized variant, the latter mainly involving the hands and feet. Its cause is unknown, although it has been speculated that a hyperergic reaction to infectious agents or medications may be etiopathologically involved. A typical case of the localized variant of this obscure disease is described and the common literature is reviewed., (2006 S. Karger AG, Basel)

Background: Volar melanotic macules are asymptomatic light-brown or tannish-grey macules usually found on the palms and/or soles of blacks, although they have also been reported on the volar surfaces of whites. Similar lesions have not been reported before in Japanese people. Since the cause is as yet unknown, it remains to be discussed whether they are a distinct entity., Methods: In this report, a 52-year-old Japanese man with volar melanotic macules is reported with the clinical and histopathological findings., Results: A 52-year-old Japanese man presented with many light-brown macules on his bilateral soles. He had a 20-year history of tinea pedis. Histopathological examination revealed melanophages and inflammatory infiltrates in the superficial dermis. There was a slight increase in melanin granules around the acrosyringium. Fontana-Masson stain revealed a slight increase in melanin granules in the basal layer including the acrosyringium and superficial dermis. These changes corresponded with postinflammatory pigmentation., Conclusions: This is the first report of volar melanotic macules in Japanese people. We suggest that volar melanotic macules is not an independent entity but a clinicopathological one that includes postinflammatory pigmentation, and that the condition is the volar counterpart of mucosal melanotic macules.

Background: Chronic venous insufficiency (CVI) can originate onychopathy per se. We have anecdotally observed nail changes in patients with CVI, but there are few studies which determine the frequency of both onychopathy and onychomycosis in these patients, Objective: The aim of the study was to determine the frequency of nail pathology and onychomycosis in patients with CVI PATIENTS AND METHODS: We included 36 adult patients, both men and women, aged from 18 to 59 years, with clinically documented venous leg ulcers. All patients were examined by a dermatologist and the venous leg ulcers were classified according to severity in three grades. The nail changes were described and a mycological examination was performed. We obtained a small fragment of the nail for histological examination. In 27 patients, we also performed functional studies to determine the type of venous insufficiency., Results: The ratio of women to men was 5 : 1. The mean age of patients was 46.39 +/- 8.51 years, men being slightly younger than women. Ten patients had ulcers of grade I severity, 12 had grade II, and 14 had grade III. The overall time of evolution of the cutaneous lesions was 11.02 +/- 10.11. Fourteen patients had superficial venous insufficiency, whereas 13 had deep venous insufficiency. Twenty-two (61.11%) of our patients had nail alterations. These nail changes were related more to the type of vascular affection than with the severity of cutaneous involvement. In more than half of the cases (59.09%), onychomycosis was the cause of the nail changes. The overall frequency of onychomycosis was 36.11%. The etiologic agent of onychomycosis was isolated in 38.46% of the cases, and Trichophyton rubrum was the most frequent agent. The histologic examination of the nail plate showed a low sensitivity (62%) but a high specificity (100%) in the detection of nail plate parasitization. No clinical differences could be established between the nail changes observed in patients with true onychomycosis and those with nonfungal onychopathy., Conclusions: Nail changes are common in patients with venous leg ulcer, and onychomycosis accounts for slightly more than half of the cases. We therefore recommend a routine mycological examination in patients wit nail changes and cutaneous manifestations of CVI, to diagnose or rule out onychomycosis, and therefore avoid overtreating patients without onychomycosis with antimycotics.

Reticulate acropigmentation of Kitamura is now reported from all over the world. Additional features are being readily recognized. Our cases had pigmentation and pits on the dorsa of the distal phalanges of the fingers and toes - the classical features - as well as widely distributed pits on the palms, palmar aspect and sides of the fingers. The involvement in our cases was more pronounced than in the previously reported ones., (Copyright (R) 2000 S. Karger AG, Basel)

Introduction: Homocysteine is a sulfur amino acid occurring in methionine intermediary metabolism. It was recently shown to be a vascular risk factor even without high serum levels., Case Report: A 29-year-old woman had painful plantar nodules and necrosis of the fifth toe on the right foot with cyanosis of the forefoot. The histology examination of a nodule biopsy gave the diagnosis of subcutaneous thrombophlebitis. Vascular explorations disclosed advanced-stage arteriopathy of the leg. The methionine loading test was abnormal and search for the heterozygous form of cystathionine ss was positive., Discussion: Different diagnoses could be possible for cases associating an inflammatory venous disease and stage IV arteritis. Latent hyperhomocysteinemia was diagnosed in this case on the basis of the methionine loading test and identification of the heterozygous form of cystathionine ss. Patients with latent hyperhomocysteinemia have an increased risk of both thrombosis and arteritis. Our case emphasizes the importance of searching for hyperhomocysteinemia in patients with early arterial and/or venous thromboembolism, either as repeated episodes or occurring in an unusual territory.

We report a case of Costello syndrome, which is an uncommon multisystemic condition with cutaneous manifestations on the palms and soles. In the literature there are 29 cases described, all the studies are published in the genetic literature with a few exceptions. We add a further case associated with impaired glucose tolerance. The diagnostic clinical signs are impressive, and highly characteristic. Cutaneous manifestations are: loose skin of the hands and feet "washer woman's hand", hyperkeratosis palmoplantaris, curly or sparse hair, acanthosis nigricans, papillomata nasi. Coarse, progeroid facial features with a bulbous nose, feeding difficulties in infancy, cardiac involvement with cardiomyopathy or conduction defect, and in our case impaired glucose tolerance also presented. Postnatal growth retardation, mental retardation, and a distinctive friendly personality is characteristic. Hyperextensible fingers with broad distal phalanges and joint contractures were observed, and peroneal hypertonicity required treatment by Achilles tendon lengthening. The decreased glucose tolerance is interesting in the view of the acanthosis nigricans. No storage disease and no chromosomal abnormality were observed. Only in one case is a balanced translocation described in the literature.

The N-terminal extracellular domain of the cadherins, calcium-dependent cell adhesion molecules, has been shown by X-ray crystallography to be involved in two types of interaction: lateral strand dimers and adhesive dimers. Here we describe the first human mutation in a cadherin present in desmosome cell junctions that removes a portion of this highly conserved first extracellular domain. The mutation, in the DSG1 gene coding for a desmoglein (Dsg1), results in the deletion of the first and much of the second beta-strand of the first cadherin repeat and part of the first Ca2+-binding site, and would be expected to compromise strand dimer formation. It causes a dominantly inherited skin disease, striate palmoplantar keratoderma (SPPK), mapping to chromosome 18q12.1, in which affected individuals have marked hyperkeratotic bands on the palms and soles. In a three generation Dutch family with SPPK, we have found a G-->A transition in the 3" splice acceptor site of intron 2 of the DSG1 gene which segregated with the disease phenotype. This causes aberrant splicing of exon 2 to exon 4, which are in-frame, with the consequent removal of exon 3 encoding part of the prosequence, the mature protein cleavage site and part of the first extracellular domain. This mutation emphasizes the importance of this part of the molecule for cadherin function, and of the Dsg1 protein and hence desmosomes in epidermal function.

A 7-year-old castrated male domestic shorthair cat was admitted to the veterinary teaching hospital for evaluation of symmetric necrosis of the skin of its hind feet and high liver enzyme activities. Lymphoma was diagnosed on cytologic examination of a fine needle aspirate of the liver. The owner declined treatment for the lymphoma. On postmortem histologic examination, lymphoma was found in the liver, stomach, and multiple lymph nodes. Immunohistochemical staining revealed the neoplasm to have a mixed B- and T-cell follicular arrangement, and a diagnosis of multicentric follicular lymphoma was made. The distal portion of the feet were necrotic, but a neoplastic infiltrate was not seen on histologic examination. After thrombosis and vasculitis were excluded as causes, the ischemic necrosis of the feet of the cat in this report was considered a paraneoplastic syndrome, as can be seen in people with lymphoma or other internal malignancies.

Skin necrosis is an uncommon complication of warfarin (Coumadin; Dupont Pharma, Wilmington, DE) therapy. The presentation may mimic other disorders. This article reports a case of a 72-year-old woman who presented to the emergency department complaining of swelling and ecchymosis to her left breast and right foot. The patient had been hospitalized for coronary artery bypass grafting, and had been discharged from the hospital earlier that day. This article reviews the pathophysiology and clinical features of warfarin-induced skin necrosis.

As the body's point of contact with the weight-bearing surface, the foot resists abrasive forces and is constantly bombarded with dermatologic insults. It is the site of a variety of local and systemic afflictions, some which even indicate a state of relative emergency. Paying careful attention to the consideration and exclusion of serious or systemic conditions, this article presents an algorithmic approach that is useful in a clinical setting.

History and Clinical Findings: A 52-year-old man had been in terminal renal failure for 6 years. On haemodialysis under heparin without complications, acral skin necroses occurred. Even with low-molecular heparin anticoagulation further lesions developed. Within 12 weeks of haemodialysis being performed without heparin the necroses healed, but they recurred when heparin was again added for dialysis. On admission the patient was in poor general condition, with a weight of 55 kg (height 175 cm). LABORATORY INVESTIGATIONS: The heparin-induced platelet aggregation (HIPA) test was positive in the absence of thrombocytopenia. Na-heparin reacted positively in three out of four tests, but Danaparoid did not react., Treatment and Course: The skin necroses once again healed after the heparinoid Danaparoid, which had not reacted in the HIPA test, had been substituted for heparin., Conclusion: This case illustrates that skin necroses, thrombocytopenia and thromboembolism can be independent signs of immunologically induced platelet aggregation.

Two dogs with metabolic epidermal necrosis had hyperkeratosis of the footpads accompanied by erythematous, erosive and crusting lesions affecting the muzzle, external genitalia, perineum and periocular regions. Histopathological examination of skin biopsies revealed a superficial hydropic dermatitis with marked parakeratosis. Both dogs had high plasma activities of alkaline phosphatase and alanine aminotransferase and high concentrations of glucose, and also a marked hypoaminoacidaemia. Despite these similarities, the cutaneous eruptions were associated with different underlying diseases. One dog had a pancreatic carcinoma which had metastasised widely; the primary tumour and the metastases showed glucagon immunoreactivity on immunocytochemical staining, and the dog's plasma glucagon concentration was markedly greater than that of control dogs. The other dog had diffuse hepatic disease; its plasma glucagon concentration was similar to that of control samples and cirrhosis was identified post mortem. Metabolic epidermal necrosis in dogs is a distinct cutaneous reaction pattern which may be associated with different underlying systemic diseases; however, the pathogenesis of the skin lesions remains unclear.

The clinical and histological features of 49 lesions induced by human papillomavirus type 1 (HPV-1) were studied. The majority of cases showed the typical clinical features and location of such lesions. They were usually located on the ventral or lateral surfaces of the hands or feet, and were dome-shaped lesions with a central depression. However, HPV-1-induced lesions located on other body areas displayed different clinical features, such as those of common warts, a digitate wart, and a cutaneous horn. One lesion on a healing burn scar had a herpes-like appearance. As these 49 lesions were induced by the same type of HPV (HPV-1), differences in their clinical features are likely to have been due to host-related factors.

Lesional and perilesional skin samples from a 57-year-old man who had hyperkeratosis lenticularis perstans (HLP) (Flegel's disease) were studied by light and electron microscopic examination. Keratohyalin granules were diminished at the center of a fully-developed lesion. In contrast, keratohyalin appeared normal and membrane-coating granules were found in reduced numbers at the edges of the HLP lesion and were easily detected in normal numbers in clinically normal, perilesional skin. The inflammatory infiltrate in the HLP lesion was composed of small lymphocytes, which often displayed nuclei with deep infoldings resembling Sézary cells, and larger histiocytic cells, many of which were in close contact with the lymphocytes. Peripheral blood mononuclear cells did not show an abnormal ultrastructural appearance. Treatment with topical 5-fluorouracil cream led to the disappearance of the HLP lesions, whereas topical tretinoin was ineffective.