N. Francis

Penile intraepithelial neoplasia: clinical spectrum and treatment of 35 cases.

Summary Background  Penile intraepithelial neoplasia (PIN) is the term used to describe erythroplasia of Queyrat (EQ), Bowens disease (BD) and bowenoid papulosis (BP). These conditions are distinct clinical entities and have different epidemiological and aetiological associations and prognostic implications.

Rosai–Dorfman disease complicated by autoimmune haemolytic anaemia: case report and review of a multisystem disease with cutaneous infiltrates

We report a patient with sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) who presented with widespread nodal and extranodal involvement affecting the skin, orbits and nasal sinuses, complicated by the development of autoimmune haemolytic anaemia. The aetiology and pathogenesis of this multisystem disorder are unknown but are thought to represent a reactive histiocytic process to an infective agent rather than a neoplastic or other primary condition. Prognosis is generally good but clinical or laboratory evidence of immune dysfunction tends to predict a poorer outcome. We describe the clinical course of the patient and review the literature on this disease.

Cutaneous sarcoidal granulomas developing after facial cosmetic filler in a patient with newly diagnosed systemic sarcoidosis

Erratum. Notice is given that it has been drawn to the attention of the journal that the dermal filler used in this case was erroneously identified as Artecoll®. The authors submitted this article in good faith on the strength of documentary evidence that Artecoll® had been used. Subsequent expert opinion indicates that the particles seen in the skin biopsy are not Artecoll®.

Magnetic resonance imaging in eosinophilic fasciitis

A 30-year-old, Asian female nurse presented with a 9-month history of limb stiffness and myalgia. Examination revealed symmetrical induration of skin and subcutaneous tissues on her limbs, most pronounced over the forearms; here there was tenderness on palpation and bilateral limitation of elbow extension and forearm supination. A blood test performed by her general practitioner had shown peripheral eosinophilia, but the repeat full blood count, including differential white cell count, was normal. Further routine laboratory investigations were unremarkable other than elevated serum IgG and IgA levels of 22.2 g ⁄ L (normal range, 5.3–16.5 g ⁄ L) and 3.85 g ⁄ L (normal range, 0.8–2.8 g ⁄ L), respectively, and a raised erythrocyte sedimentation rate of 20 mm in the first hour (normal range, 1–12 mm). Serum electrophoresis reflected a polyclonal hypergammaglobulinaemia and antinuclear and extractable nuclear antibodies were negative. Magnetic resonance imaging (MRI) of her thighs, trunk and upper arms was performed. Short-tau inversion recovery (STIR) images revealed diffuse oedema and inflammatory changes along the muscle fascial planes; however, the muscle itself appeared to be uninvolved. The findings were best observed in her thighs (Fig. 1). An incisional skin biopsy taken from her upper arm demonstrated a mild lymphoplasmacytic infiltrate and sclerosis in the deep dermis similar to that seen in eosinophilic fasciitis but not pathognemonic. The biopsy was unfortunately suboptimal. A haematological assessment including bone marrow examination was also undertaken and there were no abnormalities. After consideration of the differential diagnosis (Table 1), a diagnosis of eosinophilic fasciitis was made and the patient was started on prednisolone 40 mg daily. She responded rapidly, and the prednisolone dose was reduced. Five months after the start of treatment, she completely recovered and the prednisolone was discontinued. At this stage the thigh MRI scan was repeated and demonstrated a significant reduction in the fascial signal intensity (Fig. 1). These findings correlated with her clinical improvement. Eosinophilic fasciitis is a rare fibrosing disorder of unknown aetiology. It classically develops in young males and there is a history of preceeding trauma or strenuous exercise in approximately one-third of cases. Initial symptoms are usually swelling and discomfort of distal extremities sparing the hands and feet. This evolves into symmetrical skin induration and stiffness; a peau d’orange appearance may also follow. Associated blood results include elevated aldolase and erythrocyte sedimentation rate, hypergammaglobulinaemia and peripheral eosinophilia. Ideally, the diagnosis is confirmed histologically: important features are fascial thickening, deep dermal fibrosis and infiltration of lymphocytes, plasma cells and eosinophils. Prednisolone is the treatment of choice for eosinophilic fasciitis although the disease may remit spontaneously. Our patient was particularly responsive to treatment; however, alternative therapies such as hydroxychloroquine, ciclosporin, methotrexate, d-penicillamine and cimetidine, alone or in combination with corticosteroids, have shown some benefit in case reports. Eosinophilic fasciitis can pose a diagnostic challenge because sometimes, as in our case, it may be difficult to obtain a suitably deep, skin to skeletal muscle, tissue biopsy Figure 1 MRI examination of thighs using STIR imaging demonstrates (a) pretreatment high fascial signal intensity which (b) reduces following prednisolone treatment. Viewpoints in dermatology • Correspondence

Is Disseminated Juvenile Xanthogranulomatosis Benign Cephalic Histiocytosis

Abstract:  We describe an infant with a congenital form of non‐Langerhans cell histiocytosis with clinical and pathologic features of both disseminated juvenile xanthogranulomatosis and benign cephalic histiocytosis. The findings in this case support the concept of these non‐Langerhans cell histiocytoses forming part of a spectrum of disease rather than being separate pathologic entities.

Borrelia burgdorferi is not associated with genital lichen sclerosus in men

SIR, Lichen sclerosus (LSc) is an acquired, chronic inflammatory and atrophic skin disorder that is about 10 times more common in women than in men. Genital LSc is responsible for significant sexual dysfunction and is associated with the development of squamous cell carcinoma in both sexes; the risk in men is estimated to be between 2% and 12Æ5%. LSc shares clinical and histological features with acrodermatitis chronica atrophicans and erythema chronicum migrans, cutaneous manifestations of Lyme borreliosis caused by Borrelia burgdorferi. Since the identification of B. burgdorferi as the causative agent in Lyme borreliosis there has been debate about a possible association between B. burgdorferi and LSc. Borrelia burgdorferi has been divided into several genospecies the collective term for which is B. burgdorferi sensu lato. Various techniques have been employed to detect B. burgdorferi and implicate it in the pathogenesis of LSc. The literature contains reports of immunohistochemical, biological, serological, enzyme-linked immunosorbent assay (ELISA), culture and molecular methodologies. Findings to date have been inconsistent and contradictory. Most recently Eisendle et al. claim to have identified Borrelia species from tissue samples of extragenital and genital LSc in men and women using a novel modified immunohistochemical technique called free-floating microscopy. They detected Borrelia species in 63% of cases of LSc, 90% of cases of classic borreliosis and none in normal controls. This approach has yet to be extended to male genital LSc (MGLSc). The Scottish Lyme borreliosis testing service laboratory tests approximately 4000 samples a year. In accordance with the Centers for Disease Control and Prevention guidelines laboratory diagnosis of Lyme disease is a two-step process. Because of cross-reactions, especially with other spirochaetes, ELISA tests lack specificity. This is why all our samples were further tested by Western blotting. This technique has been validated by Evans et al. and is currently used at the Raigmore Hospital, Inverness, Scotland. Sera from 30 adult male patients (age range 18–57 years) with biopsy-proven MGLSc and 32 male, age-matched controls were selected from men attending the male genital dermatoses clinic at the Chelsea and Westminster Hospital, London, U.K. between April 2001 and January 2007. All ELISAs were negative. Usually in the absence of strong clinical suspicion, no further testing would be performed; however we tested the 62 samples by IgG Western blotting. One control was found to be weakly positive, nine samples (14Æ5%) were equivocal (five diseased, four controls) and 53 samples were negative. No matter how carefully the Western blot test is carried out and interpreted, its usefulness, like that of all tests that measure B. burgdorferi antibodies, ultimately depends on the reliability of the human immune response as an indicator of exposure to B. burgdorferi, and the possibility that strain variations in B. burgdorferi might produce unusual banding patterns must be considered. A positive Western blot is not necessarily an indication of active Lyme disease. A patient’s immune response to B. burgdorferi can remain intact long after curative treatment for a Lyme infection; therefore, the results of a Western blot assay should always be interpreted in the context of the total clinical picture. Unfortunately no further serum was available from our positive patient for retesting. Although the concept that some if not all cases of LSc might be caused by an infectious agent is longstanding, no organism has been consistently identified. The overall tenor of LSc is not an infectious one. There is only one reported case of LSc occurring between sexual partners and in this instance there was a 10-year interval. These observations mean that any putative infectious process must be acquired in a nonsexual communicable manner. To our knowledge this is the largest sample of patients with MGLSc that has been tested for B. burgdorferi using validated techniques and we conclude that B. burgdorferi cannot be a causative organism. Whether or not the same pertains to patients with MGLSc outside the U.K. or indeed women with genital LSc needs to be confirmed.

Bone marrow involvement in cutaneous mastocytosis

Background  Cutaneous mastocytosis is considered a relatively benign and indolent form of mast cell disease, which either ultimately regresses, remains stable or is only slowly progressive. Previously, it has been purported that no more than 60% of adult patients with cutaneous mastocytosis will have occult bone marrow involvement.

Kissing naevus of the penis.

Divided or ‘kissing’ naevus was first described on the eyelids in 1908. Other types of divided naevus reported include naevus spilus on the eyelids, mast cell naevus and epidermal naevus in a divided form on the fingers. Six cases of kissing naevus of the penis appear in the literature. In this paper, we discuss a seventh case.

Periocular autologous Coleman fat graft survival and histopathology.

Purpose: Autologous Coleman fat transfer is used in the periocular area and intraorbitally for soft-tissue volume augmentation in postenucleation socket syndrome. This article aims to identify areas of low fat absorption and the histopathology of excised fat. Methods: Noncomparative case series of patients who underwent excision/debulking of injected autologous fat in the inferior sulcus/lower eyelid. Results: Five of 20 patients with postenucleation socket syndrome who had received intraorbital and sulcus Coleman fat transfer, required fat reduction (sculpting) from the inferior sulcus and lower eyelid due to persistent “overfill” 8 months to 17 months after fat transfer. Between 0.2 ml and 1.4 ml of fat was removed. The excised fat was pale, rubbery, and compact in comparison with the normal lower eyelid fat pad. Histopathology showed healthy adipose cells but with chronic inflammation and fibrous septa. Conclusions: Fat survival is greater than anticipated in the lower eyelid and inferior sulcus. Therefore, judicious use in these areas is suggested. The grafted fat is distinguishable from the fat in the lower eyelid both clinically and histopathologically.

Diffuse trichostasis spinulosa in chronic renal failure

Trichostasis spinulosa is a relatively common but underdiagnosed disorder of the pilosebaceous follicles in which there is follicular hyperkeratosis of a dilated vellus hair follicle with retention of successive telogen hairs arranged in parallel among keratinaceous material. The condition is apparent as dark follicular plugs in the affected areas, most commonly affecting the face (nose and cheeks) or the trunk (interscapular area in particular). We describe a 33‐year‐old Indian man who was on haemodialysis for chronic renal failure and who developed unusually widespread trichostasis spinulosa over almost the entire body including the lower limbs over a period of 18 months.