[Successful therapy of retronychia]. / Erfolgreiche Therapie einer Retronychie.

ANAMNESIS: We report on a 66-year-old patient who had had painful swelling of his left big toe for about 9 months with subjectively stopped growth. EXAMINATION: Previously, bacteriological, and mycological smears and an MRI examination had not provided any groundbreaking findings, and previous antibiotic, antiseptic and anti-inflammatory therapies had not contributed to alleviating the symptoms. DIAGNOSIS AND THERAPY: Based on the clinical findings with a reddened, piston-like distended distal phalanx with a raised proximal nail wall, we made the diagnosis of retronychia and performed a nail plate extraction. CLINICAL COURSE: In the follow-up checks, which lasted more than two years, the patient was symptom-free with recovered nail growth. CONCLUSION: As in the case presented, retronychia is often misdiagnosed. The knowledge of groundbreaking clinical and anamnestic parameters and the correct therapy options allows a quick, inexpensive, and long-term successful treatment.

[Artificial ulcer with perforating bone defect on the scalp due to repeated - scratching attacks in dementia syndrome]. / Artifizielles Ulkus mit perforierendem Knochendefekt an der Schädelkalotte durch wiederholte ­ Kratzattacken bei demenziellem Syndrom.

HISTORY: We report on a 72-year-old demented patient with a palm-sized skin defect on the scalp, who has been manipulating her head several times a day with her fingers but also with sharp objects for many months. CLINICAL FINDINGS: A 4 × 6 cm circular, cleanly granulated ulcer was visible on the high parietal surface. On general examination, the patient was little affected: no fever, no signs of inflammation around the ulcer, no evidence of meningitis. EXAMINATIONS: There was no histological evidence of the presence of a malignant or benign tumor. A CT of the skull showed a bony defect measuring 2.6 × 3.3 cm without involvement of the dura. The whole body CT without pathological findings. THERAPY AND COURSE: First, a protective helmet was made for the patient. Subsequently, a titanium plate was surgically inserted as a bone substitute and the defect was closed by means of transpositionoplasty. In the two-year follow-up, no tumor disease was detected. CONCLUSION: Due to the lack of evidence of tumorigenesis in a follow-up period of two years, we evaluate the lesion as an artificial ulcer with perforating bone defect caused by repeated scratching attacks in the context of dementia syndrome.

[Interdisciplinary Management of Tumours of the Eyelid and Periorbital Region]. / Interdisziplinäres Management von Tumoren der Lid- und Periorbitalregion.

INTRODUCTION: Skin malignancies are typically localised in areas of the head that are exposed to the sun. Basal cell carcinomas (BCC) are the most frequent malignancies on the facial skin. Their incidence is raising - due to demographic changes. As regards strategies for resection and reconstruction, the eyelids and the periorbital region are extremely complex and have to be treated in an interdisciplinary context. The aim of the present investigation was to analyse the results of interdisciplinary treatment of periorbital and eyelid non-melanotic malignoma. MATERIALS AND METHODS: All treated non-melanotic malignancies of the eyelid/periorbital region were analysed in a pilot study from September 2017 until July 2019. Patients were treated by an ophthalmologist and a maxillofacial plastic surgeon. The clinical and pathological parameters were collected in a databank. In all interdisciplinary cases, the tumour localisation, histology, R-status and the reconstructive strategy were analysed. RESULTS: Out of 349 patients, 14 were analysed as they were treated with an interdisciplinary approach. The youngest was 12, the oldest 98 years old. There were 6 women (average age 80.3 years) and 8 men (average age 65.3 years). BCC (n = 10) were the most frequent malignancy; 3 patients suffered from squamous cell carcinoma. Actinic keratosis was diagnosed in one case. In all patients, complete resection (R0) was certified by histopathological examination. The reconstruction was performed immediately in 6 cases, and the reconstruction strategy was accomplished after definitive histology (at least two step procedures) in 8 cases. Only one patient had three resections before starting the reconstruction procedure. DISCUSSION: Surgical treatment of malignancies of the eyelid or periorbital non-melanotic malignancies can be an interdisciplinary challenge. BCC is the most frequent entity. These critically localised BCC present with extremely deep infiltration, followed by subtotal or total eyelid resection, often including bony structures. The reconstruction is extremely complex in such cases and requires the whole plastic reconstructive repertoire of both medical disciplines.

[Pitfalls of Differential Diagnosis in Dermatologic Surgery]. / Differenzialdiagnostische Fallstricke aus der Dermatochirurgie.

Evaluation of skin diseases can be challenging for non-dermatologists. Even obvious well-characterized skin pathologies might be misleading and thus treatment can fail. Particularly the differentiation of surgical treated entities is important, for example the management of a wound healing disturbance profoundly differs from that of a pyoderma gangrenosum. This article outlines several easily mistaken pairs of dermatologic entities on one hand and surgical on the other. For example, a livedo vasculopathy can be confused with a leg ulcer, a nail melanoma with a simple hematoma and finally a hidradenitis suppurativa with an axillary abscess. Typical clinical signs and anamnestic data may often lead to the right diagnosis also assisted by the simple fact to "keep it in mind".

Morphology of pilonidal sinus disease: some evidence of its being a unilocalized type of hidradenitis suppurativa.

BACKGROUND: The term 'pilonidal sinus' (PS) reflects an acute exacerbating, purulent, fistulating chronic inflammation located in the coccyx region. Systematic histological investigations are scarce, and the etiology has remained controversial. METHODS: Histological and immunohistochemical characterization of totally excised material of 27 patients (68 specimens) with PS (no antecedent and no current clinical signs of hidradenitis suppurativa, HS) and its correlation with data on HS which we published earlier. RESULTS: Follicular hyperplasia/hyperkeratosis and interfollicular epidermal hyperplasia are main features of PS. Early pathology seems to take place at terminal hair follicles, whereas sinus tract formations are a secondary event. Focused regions show an inflammatory mixed infiltrate consisting of CD3+, CD4+, CD8+, CD68+ and CD79+ cells. CONCLUSIONS: PS and HS have various common characteristics at the histological and immunohistochemical level. Considering PS as a unilocalized type of HS, risk factors known in the latter should henceforth be evaluated in PS as well.

Hidradenitis suppurativa (acne inversa): early inflammatory events at terminal follicles and at interfollicular epidermis.

Hidradenitis suppurativa (acne inversa) is a chronic suppurative and scarring inflammatory disease with predilection in the apocrine gland-bearing areas. Histological investigations in the 1990s showed keratotic occlusion of the terminal follicle structure to be the initial cause. Our investigations describe and reproduce the morphology and try to figure out very early lesions of HS. A total of 262 operative specimens from 60 patients were investigated by routine histology and 11 operative specimens by immunohistochemistry: HS is dominated by a heterogeneous histological image. 82% of the surgical specimens showed mild or pronounced follicular hyperkeratosis, whereas an isotopic hyperplasia of follicular epithelium was evident in 77%. Pronounced perifolliculitis was seen in 68% and rupture of the follicle structure in 28%. Features which had not so far been described in detail were: epidermal psoriasiform hyperplasia (43%) and subepidermal interfollicular inflammatory infiltrate (78%). In all 11 specimens, immunohistochemical investigations showed a perifollicular and subepidermal inflammation of CD-3-, CD-4-, CD-68-, CD-79- and CD-8-cells, the latter with a striking selective epitheliotropism. To conclude, we could show follicular hyperkeratosis and lymphocytic perifollicular inflammation as early patterns in pathogenesis, whereas rupture of the follicle structure takes place later. Finally, it seems that there are two hot spots of inflammatory events (perifollicular and subepidermal) composed of a comparable inflammatory cell mixture. The CD-8 cell epitheliotropism (follicular and epidermal) described here and its influence in follicular hyperkeratosis, in hyperplasia of follicular epithelium and in epidermal psoriasiform hyperplasia will be of further interest, for instance, concerning early pharmacological intervention.

Detection of silver sulfide deposits in the skin of patients with argyria after long-term use of silver-containing drugs.

Five patients with generalized slate-gray discoloration of the skin have been diagnosed histologically as argyria in the last 35 years in the Department of Dermatology and Venereology of Rostock and Halle. Light microscopically, there was visible black pigmentation in histiocytes, fibroblasts, and multinucleated giant cells of the dermis. In the transmission electron microscope (TEM), the authors observed electron-dense deposits inside lysosomes and residual bodies of phagocytes as well as outside the cells in the connective matrix. These deposits were identified by elemental analysis in TEM and electron energy loss spectroscopy (EELS) as well as scanning electron microscope (SEM) and energy dispersive x-ray analysis (EDX) containing silver and sulfur. Therefore, they seem to consist of silver sulfide. Argyria is of low medical relevance and is very rarely induced because of silver-containing drugs. Nevertheless, there are still a lot of silver products on the market, easily available over-the-counter. Therefore, argyria should not be forgotten or missed in the diagnostics of human dermis.

Radiotherapy in multilocalized lymphedema-associated angiosarcoma.

We report an 80-year-old woman, suffering from a recurrence of a multilocalized lymphedema-associated angiosarcoma of the right arm. The tumor consisted of solid tumor cell formations and "classical" spongiform tumor complexes. In the tumor periphery, pathological endothelial cell proliferates on pre-existing dilated lymphatic capillaries were detectable, which, together with immunohistology (CD 31+/Desmoplakin-1-2.17+/CD 34-), supported the diagnosis of lymphangiosarcoma. Complete remission was achieved under radioimmunotherapy (54 Gy/Interferon beta). A further recurrence 3 months later outside the primary therapy fields was successfully treated with radiotherapy alone. During a follow-up observation period of 3 years, there was neither local recurrence nor metastasis. This case demonstrates for the first time the long-lasting efficacy of photon radiation in a case of histologically-defined lymphangiosarcoma. Further studies should elucidate the suitability of radio monotherapy as first-line therapy in lymphedema-associated angiosarcoma with lymphatic endothelium-like immunohistology.

[Argyria--an almost-forgotten dyschromia]. / Die Argyrose--eine heute fast vergessene Dyschromie.

A 79-year-old woman presented with a long history of grey-blue discoloration of the light-exposed areas. Her face, forearms, and the backs of both hands were affected. The proximal parts of her fingernails were impressively discolored, but her toenails were not affected. The patient reported that she had received oral therapy with the silver-containing drug Gastrarctin in 1959. The diagnosis of argyria was confirmed by documenting high tissue levels of silver in light-exposed skin. Argyria has become uncommon as medications containing silver are no longer employed and occupational protection for those with exposure to silver salts has become more refined.

[Reynolds syndrome--a rare combination of 2 autoimmune diseases]. / Das Reynolds-Syndrom--seltene Kombination zweier Autoimmunerkrankungen.

HISTORY AND CLINICAL FINDINGS: Since several years, two women, 69 and 75 years of age, developed a paroxysmal blanching of the fingers on both hands. The attacks were accompanied by local pain and followed by a short period of cyanotic discoloration. INVESTIGATIONS: The diagnosis of progressive systemic sclerosis was reached by skin biopsies, immunoserological profile and significant blood vessel alteration at the proximal nail fold (capillary microscopy) in both patients. Initial esophageal sclerosis was only found in one patient. Both women were conspicuous by sonographically proven hepatomegaly, elevated hepatic serological parameters, in particular aP and GGTP. Positive AMA titers of M2 specificity and specific features in liver biopsies yielded the additional diagnosis of primary biliary cirrhosis). TREATMENT AND COURSE: One of the women was treated by pentoxifylline and nifedipine in order to improve rheology. Both received physiotherapeutic assistance. Primary biliary cirrhosis was treated with ursodeoxycholic acid which led to normalization of the cholestatic parameters. CONCLUSIONS: Reynolds' syndrome constitutes the coexistence of progressive systemic sclerosis and primary biliary cirrhosis. It remains unknown whether or not there is a constant time-related sequence of the clinical manifestations of the two immunogenetically determined disorders. However, it is remarkable that the affected elderly women described so far had Raynaud's symptoms long before the final diagnosis of Reynolds' syndrome was settled. Thus, the Raynaud's phenomenon is an early clinical symptom for the evolution of a Reynolds' syndrome. By experience, the coincidence of progressive systemic sclerosis and primary biliary cirrhosis seems to be a favorable factor concerning the progression of primary biliary cirrhosis. So, an elderly woman with recently manifested progressive systemic sclerosis should alert the physician to a concomitant primary biliary cirrhosis.