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In daily practice, nail pigmentation can be a diagnostic challenge, especially if the dermoscopic findings are nonspecific. We present examples of cases, in which optical coherence tomography-a rapid, noninvasive imaging method-showed typical changes that were indicative for the diagnosis.In der täglichen Praxis können Nagelpigmentierungen eine diagnostische Herausforderung darstellen, insbesondere wenn sie dermatoskopisch unspezifische Veränderungen zeigen. Wir stellen exemplarische Fälle vor, bei denen die optische Kohärenztomographie als schnelle, nichtinvasive bildgebende Methode typische Veränderungen zeigte, die hinweisend für die Diagnose waren.

Skin signs in acute necrotizing pancreatitis (e. g., Cullen's and Grey-Turner's sign) have been described in clinical medicine. The aim of the present study was to evaluate if, and how often, skin signs were noted in autopsy-confirmed cases of necrotizing pancreatitis. In a first step, the literature was extensively reviewed to establish which skin signs have so far been described in acute pancreatitis cases. As a second step, the reports of 16,000 autopsies performed at the Institutes of Legal Medicine in Frankfurt am Main and Giessen, Germany, were retrospectively evaluated. Twenty cases with necrotizing pancreatitis were found. In almost all of these, necrotizing pancreatitis was - either solely or in combination with other factors - the cause of death. In three cases, discolorations of the skin were found that were considered to be skin signs due to their location. At least in the evaluated medicolegal autopsy pool, skin signs in necrotizing pancreatitis thus appear to be a rare occurrence. Nevertheless, in cases of unexpected death, particularly of individuals with a history of alcohol abuse, necrotizing pancreatitis should be considered in the differential diagnosis of hematomas possibly attributed to trauma.

An abnormal change in skin colour is designated as discolouration of the skin. A 73-year-old male patient with circumscribed discolouration of the upper lid margin near the lacrimal pathway is presented. Slit lamp biomicroscopy showed no other signs of pathomorphological change. An excisional biopsy was performed. The whole abnormal area was removed. The oculoplastic procedure was the surest way to establish a definite diagnosis of Prurigo nodularis.

Pigmentary nail changes may have a variety of causes, e. g., chronic inflammation, and occur more often in patients of African descent compared to Caucasians. Subungual malignant melanoma is the most important differential diagnosis and must be ruled out in any case. Dermatoscopy might be helpful. If no biopsy is taken, clinical follow-ups are necessary at short intervals. In case of chronic paronychia, optimization of occupational skin protection measures may contribute to the healing process.

Homogenous pigmentation can be induced by α-melanocyte-stimulating hormone (MSH) homologues. Cosmetically inacceptable pigmentation is mostly located on the face.Although afamelatonide is a prescription drug for the orphan disease erthropoetic protoporphyria, structurally related α-MSH derivatives are available via the internet. Preventive and therapeutical options are necessary for postinflammatory hyperpigmentation, melasma, and lentigines.Case reports address activation of dysplastic naevi by melanotan I. Wood's lamp is of some use in analyzing the level of hyperpigmentation. However, no guidelines have been established; thus, a summary of current studies is presented.Melanotan I leads to the activation of dysplastic nevi. The gold standard for hyperpigmentation is triple therapy with hydrochinon, tretinoin, and steroids, which can cause irritation and lead to ochronosis in some individuals. Tyrosinase inhibitors, substances that increase the cell turnover, and plant derivatives are less efficient but more tolerable.Melanotan I and bleaching creams, which may possibly contain mercury, are dangerous. Hyperpigmentation is best treated using a combination therapy that inhibits melanocyte activity and melanin synthesis, removes melanin, destroys melanin granules, and includes UV protection. Especially in Fitzpatrick skin types IV-VI, cryotherapy and laser are not the first line treatment options due to renewed posttreatment hyperpigmentation.

Progressive pigmented purpuric dermatosis (PPPD, Schamberg disease) is a rare benign, but chronic dermatosis frequently misdiagnosed as vasculitis or bleeding disorder. Although affected patients experience significant impairment in quality of life no effective treatment has been established. The aim of our two center case series was to evaluate efficacy and tolerability of the antioxidants rutoside and ascorbic acid as combination treatment for PPPD.A retrospective review was performed on 35 patients with PPPD treated with 2 × 50 mg rutoside and 1,000 mg ascorbic acid daily between 2004 until 2011. The mean treatment duration was 8.2 months.71.4% of the participants experienced complete clearance and 20.0% an improvement of more than 50%, accompanied by increased quality of life. Nine participants (25.1%) relapsed after discontinuation. In seven, rutoside and ascorbic acid was re-initiated, and all responded again. Only three participants reported mild adverse effects. Participants with shorter disease duration showed better therapeutic success, shorter time to response and lower risk of recurrence.Oral rutoside and ascorbic acid may be an efficient and well tolerated treatment for PPPD. Early treatment is recommended to achieve best clinical outcome.

A dark colored pigmentation of nails can be due to a subungual malignant melanoma. There are, however, many other benign causes and this differentiation is an important challenge in dermatological practice.When should nail pigmentation be clarified by histological investigations?This article gives a survey of the literature on this topic and a review of own experiences with clinical case examples. The various causes of melanonychia are presented and criteria for the early recognition of melanoma of the nail matrix are formulated.The width and depth of color of longitudinal melanonychia do not allow any conclusions on the dignity. If the nail striation is proximally broader than distally, this is evidence of a proliferative event. Confirmation of a subungual hemorrhage does not exclude a malignancy per se. Also detection of pigmentation due to fungi does not exclude the additional presence of a subungual melanoma. A systematic clinical analysis using clear criteria leads to a working diagnosis and suitable treatment.An early and correct designation of nail pigmentation can avoid unnecessary nail interventions but can also be life saving.

Pigmented and nonpigmented nail abnormalities often represent a challenge for clinicians because many, and sometimes potentially life-threatening differential diagnoses must be taken into consideration. Although many details of nail diseases can already be assessed with the naked eye, dermoscopy opens up a second microscopic level of inspection, which can be very useful for the diagnostic process. In the last 20 years dermoscopy has made rapid progress in the further development of criteria for the early recognition of melanoma. In addition, the use of dermoscopy has been extended to the examination of cutaneous adnexa, such as hairs (trichoscopy) and nails (onychoscopy). Many, sometimes highly specific criteria for the dermoscopic assessment of nail diseases have been described in a series of recently published articles. This review article provides important diagnostic aids for a well-founded dermoscopic assessment of nail diseases.

The oral mucosa contains melanocytes, even though one might not suspect this when examining white subjects. Drug-induced pigmentation is usually irregularly distributed over the oral mucosa; typical causes are contraceptives and tetracyclines. Localized traumatic pigmentation can be due to injuries contaminated by foreign material (dust). Not infrequently an amalgam tattoo can be seen, caused by introduction of amalgam during dental treatment with rotating instruments. Focal melanosis is harmless. Neoplastic pigmentation is rare. Melanotic nevi are small with indistinct borders. Malignant melanoma occurs predominantly on the maxilla or hard palate. Frequently it has already metastasized by the time of diagnosis. Verification by biopsy is essential if a lesion has suddenly appeared, is extensive, elevated, with irregular pigmentation and has no obvious cause.

In a 69-year-old man with ochronosis, circumscribed brown-black scleral spots with injection of conjunctival vessels and some small pigmentations of the peripheral superficial cornea occurred. Ochronosis is an autosomal recessive chronic disease, whereby early detection of cardiac valvular defects and arthritis is important particularly in patients who are older than 40 years.

A 16-year-old boy with multiple nevi presented with an enlarging pigmented lesion on his right chest. Previously excised nevi were all benign. Dermoscopy of the symmetrical lesion revealed a trizonal globular-homogeneous pattern. A peripheral rim of brown globules was followed by a zone with homogeneous pigmentation. The centre of the lesion showed black dots and globules. Histopathology confirmed a dysplastic compound nevus and found no diagnostic aspects of pigmented spindle cell nevus (Reed).

Pigmentation in human skin differs individually and is regulated by more than 100 genes. The discovery of an increasing number of these genes has shed light on the molecular basis and pathogenesis of genetic pigmentary disorders. They are very rare and can be caused by changes in melanocyte number or melanin synthesis as well as development, transport and transfer of melanosomes. Pigmentary disorders can be divided into hyper- and hypopigmentation, of which the distribution can be diffuse or localized. Localized hypopigmentation can be found in piebaldism, Waardenburg syndrome and Tietz syndrome, whereas diffuse forms are typical for oculocutaneous albinism, Hermansky-Pudlak syndrome, Chediak-Higashi syndrome and Griscelli syndrome. Hyperpigmentation can be divided into diffuse, reticular or localized forms. They must be distinguished from endocrinopathies which may show hyperpigmentation, and from poikilodermatous syndromes displaying internal involvement.

Treatment of hyperpigmentation disorders can be a lengthy process. There are several topical treatment options and hydroquinone is the most frequently used. Both combination and monotherapy are employed. These treatment options apply primarily to epidermal hyperpigmentation disorders. Dermal hyperpigmentation disorders cannot yet be effectively managed with currently available therapeutic approaches. Cosmetic disfigurement caused by hypomelanosis, especially vitiligo, can constitute a serious impairment for the patient. So far there is no definitive remedy. Better cosmetic results are generally reported for combination therapies.

A 55-year-old man presented with a pigmented skin tumor on the left thorax but refused excision. At the 2-month follow-up a change was evident and the patient agreed to excision. This example emphasizes the importance of regular monitoring since this was the only way to prevent an atypical growth from becoming malignant.

A 70-year-old man with a 14 month history of chronic severe diarrhea treated with budenosid and mesalazin was admitted because of peripheral oedema and weight loss of about 26 kg.A general pigmentation of the skin, especially on scull and hands, as well as dystrophic nail changes and alopecia of scalp and facial hair were seen. The tests showed a slight macrocytotic, normochromic anemia. Total protein and the clotting factors were decreased. Endoscopy revealed multiple sessile polyps in the stomach and duodenum in appearance to colon and rectum. Endoscopic removal of a polyp showed histologically cystic dilatation of foveolae and oedematous mucosa. The histological features, the wide distribution of the polyps together with the skin changes, lead to the diagnosis of Cronkhite-Canada syndrome (CCS).The patient was initially treated with prednisolon 60 mg/d i. v. for 2 weeks, resulting in a marked improvement of symptoms and weight gain. He is at present in good health under prednisolon 20 mg/d per os and is followed up in our outpatient department.CCS is in up to 14 % of the cases associated with a carcinoma of the gastrointestinal tract. At present there are only reports about a successful treatment by steroids, prophylactic gastrectomy and proctocolectomy. Typical myopathic lesions of CCS have not been described to date, but the demonstrated improvement of creatinin kinase with successful treatment suggests a common pathophysiological mechanism.

Skin color is highly individual and the variations are controlled by numerous genes. The different skin colors result from the size and number of melanosomes and do not mirror the amount of melanocytes. Disorders of pigmentation can result from migration abnormalities of melanocytes from the neural crest to the skin during embryogenesis. In addition, impairment of melanosome transfer to the surrounding keratinocytes, an alteration in melanin synthesis and a defective degradation or removal of melanin may lead to abnormal skin pigmentation. Immunologic or toxic mediated destructions of melanocytes can end in pigmentation disorders. Disorders of pigmentation are classified in hypo- or hyperpigmentation which can occur as a genetic or acquired disease. They can manifest locally or diffuse. Congenital hypopigmentation can be restricted to the skin as in piebaldism or they represent a systemic disease as in Menkes disease or phenylketonuria. Localized hypo- or hyperpigmentation in children may serve as markers for systemic diseases. Ash-leaf hypopigmentation are characteristic for tuberous sclerosis and more than 5 cafe-au-lait spots suggest neurofibromatosis 1 (von Recklinghausen disease). The most common autoimmune-induced depigmentation is vitiligo. Generalized hyperpigmentation only rarely reflects a primary genetic disorder but is most often from acquired diseases as in Addison disease, secondary hemochromatosis or primary biliary cirrhosis. Treatment of pigmentation disorders are based on a diagnosis which sometimes allow a specific intervention. Cosmetically acceptable results are difficult to obtain.

The Kindler syndrome is a new form of inherited epidermolysis bullosa and the first genodermatosis caused by a defect of the focal adhesions. Kindlin-1, the deficient protein, plays an essential role in integrin activation and in the adhesion of keratinocytes to the extracellular matrix. The adhesion defect leads to skin blistering which begins at birth and ameliorates with age, and to mucosal fragility which leads to scarring and stricture formation. Skin atrophy and poikiloderma develop progressively. Photosensitivity is rather mild, but squamous cell carcinomas develop on sun-exposed areas mainly after the age of 40 years. The most important differential diagnoses are epidermolysis bullosa with mottled pigmentation and dystrophic epidermolysis bullosa. Management aims to treat the symptoms and prevent complications.

We report on a case of a bufexamac-induced allergic contact dermatitis with hematogenous dissemination presenting with the clinical and histological picture of a pigmented purpuric eruption. To our knowledge this is the first report on a bufexamac-induced pigmented purpuric dermatosis. It represents a further example of the clinical variety of cutaneous side-effects caused by bufexamac.

Summary Terra Firma forme dermatosis (TFFD) is an infrequently described disorder which features dirt-like lesions that clear after rubbing with alcohol. We describe a series of four patients with the disorder, discuss differential diagnostic considerations and review the literature.

The medical history of a 46-year-old female patient with dark-blue sclerae showed repeated fractures of the extremity skeleton and clavicular following minor trauma up to the age of 15. The sclerae indicated osteogenesis imperfecta (OI), which leads to disrupted collagen synthesis due to various mutations of the type-1 procollagen gene, which in turn leads to brittle bones with reduced bone density and greater susceptibility to fracture. In OI, distinction is made between varying clinical types which show differing degrees of severity. Bisphosphonates, which can reduce significantly increased bone turnover, are a very promising therapy approach.

Summary Background: Aesthetic laser treatments of the skin result in collateral thermal damage to the adjacent tissue if it is not cooled.A recently developed gel cooling pad is designed to be applied to the treated area and then penetrated by the laser beam during treatment. Patients and methods: 97 treatments of 61 patients were documented.Vascular malformations (telangiectasia, hemangioma, spider nevi, nevi flammei, scars) were treated 52 times with the LBO laser Idas (532 nm).With the alexandrite laser Arion (755 nm), 13 treatments for hypertrichosis were evaluated. Pigmentation disorders (tattoos,lentigines,segmented melanocytic hyperpla-sia, nevus of Ota) were treated 32 times with the ruby laser Sinon (694 nm). Additionally, we measured the energy of the laser systems through a glass plate with and without the hydrogel pad. Furthermore, we examined patient satisfaction as well as noting application experiences of the treating doctors. Results: Our examinations showed a negligible loss of energy in clinical use, with good patient satisfaction and good cosmetic results. Conclusions:Use of the hydrogel pad is recommended during non-ablative cosmetic laser treatments of the skin.It is especially convenient for the treatment of vascular malformations of the face.

Spindle cell and epithelioid cell differentiation occur in both benign and malignant hyperpigmented melanocytic lesions. Reed nevus is characterized by compact, sharply circumscribed junctional cellular nests composed of slender hyperpigmented melanocytes shaped like spindle cells. Deep penetrating nevus is characterized by a diffuse dermal proliferation composed of small nests and fascicles of pale ovoid and epithelioid melanocytes. Cellular blue nevi often have a characteristic hourglass or dumbbell shape, with sharply circumscribed elongated nests and fascicles of pale, densely layered ovoid melanocytes and adjacent melanophages. Epithelioid blue nevus is characterized by large epithelioid melanocytes with abundant cytoplasm and melanin often concentrated to some degree in the cell membrane. Animal-type melanoma is a particularly hyperpigmented variant of melanoma in which large melanophages predominate and there are varying proportions of melamin-rich spindle-shaped and large atypical epithelioid melanocytes. Morphologically, pigmented epithelioid melanocytoma combines characteristics of both animal-type melanoma and pigmented epithelioid nevus. Malignant melanoma may occur in conjunction with a preexistent blue nevus. Malignant blue nevus is now regarded as a malignant melanoma mimicking a blue nevus in structure and pattern. It is therefore of paramount importance to view multiple mitoses within a cellular blue nevus-like proliferation as an alarm signal as they are usually indicators of a malignant melanoma.