Non-scarring alopecia of lupus erythematosus: A comprehensive review.

BACKGROUND: Although non-scarring alopecia (NSA) is a frequent clinical finding in patients with systemic lupus erythematosus (SLE), it has been poorly described in the literature. It is considered a nonspecific sign in the current classification of skin lesions of LE. The aim of this study was to give an updated overview of the spectrum of NSA in LE patients, with emphasis on the clinical significance thereof. METHOD: We conducted a review of the English literature using the PubMed-Medline database using the keywords "Alopecia" + "Lupus erythematosus". Publications describing LE patients with NSA were included. RESULTS: Data for 237 patients from 27 publications were analyzed. Ninety-one patients had diffuse NSA, 43 had patchy NSA, 83 had lupus hair, 3 had alopecia of dermal cutaneous LE, and 17 had alopecia of linear and annular lupus panniculitis of the scalp. Patients with diffuse/patchy NSA and lupus hair shared the following features: strong association with systemic activity of LE, subtle clinical/trichoscopic signs of inflammation, histological aspect consistent with lesions specific to cutaneous LE, high likelihood of response to SLE therapy, and absence of progression to scarring alopecia. Association with SLE was rare in patients with dermal cutaneous LE or linear and annular lupus panniculitis of the scalp, and skin-directed therapies were most often effective. One patient of each subtype progressed to scarring alopecia. DISCUSSION: Diffuse/patchy NSA and lupus hair may represent a topographic variation of a single entity specific for LE. Prospective studies are warranted to further document the clinical significance of this manifestation.

Lupus erythematosus: Significance of dermatologic findings.

Herein, the different skin manifestations in patients with lupus erythematosus are reviewed, and their diagnostic, pathogenic and prognostic relevance are discussed, as well as their impact on therapeutic choices. The so-called specific lesions of LE result from an autoimmune pathomechanism and they allow diagnosis of LE by simple clinicopathological correlation since the findings are characteristic. They include the classic acute, subacute and chronic variants, characterised microscopically by interface dermatitis; the dermal variants of lupus, such as tumid lupus, displaying dermal perivascular lymphocytic infiltrate with mucin deposition under the microscope, and lupus profundus, in which lymphocytic lobular panniculitis progressing to hyaline fibrosis is found. Antimalarials are the treatment of choice for patients with specific LE lesions. The presence of some dermatological signs is the result of thrombotic vasculopathy. Their recognition allows the identification of lupus patients at increased cardiovascular risk and with a worse overall prognosis. Those signs include reticulated erythema on the tip of the toes, splinter hemorrhages, atrophie blanche, pseudo-Degos lesions, racemosa-type livedo, anetoderma, ulceration and necrosis. Those clinical manifestations, often subtle, must be recognised, and if present, patients should be treated with antiplatelet drugs. Finally, neutrophilic cutaneous lupus erythematosus includes a few entities that suggest that autoinflammatory mechanisms might play a key role in certain lupus manifestations. Among those entities, it is very important to diagnose neutrophilic urticarial dermatosis, which can mimic a classic lupus flare, because it is characterised by rash with joint pain, but immunosuppressants are not helpful. Dapsone is the treatment of choice.

[Anatomoclinical and immunohistochemical study of 8 cases of dermatomyofibroma]. / Étude anatomoclinique et immunohistochimique de 8 cas de dermatomyofibromes.

INTRODUCTION: Dermatomyofibroma (DMF) is a rare, benign tumour that is little-known among clinicians. However, it has typical clinical, histological and immunohistochemical features that distinguish it from other fibrous tumours. METHOD: We report herein on the clinical, histological and immunohistochemical aspects of eight cases of DMF identified between 2008 and 2019 at the dermatopathology laboratory of Strasbourg. RESULTS: Five men and three women of average age at diagnosis of 21 years and 9 months (range: 9 to 54 years) were included. Lesions ranged in size from 1 to 11cm. Most cases involved the upper body (6 cases), with one case on the abdomen and one on the side. The lesions presented as a solitary asymptomatic red or reddish brown nodule or plaque that gradually developed. The plaques were hard and caused functional discomfort on movement of the neck. Well-circumscribed spindle cell proliferation was noted in the reticular dermis parallel to the epidermis, without mitotic figures or cytological atypia. The subcutis was infiltrated in 5 cases. Expression of calponin was positive in all cases but one, while that of caldesmon, PS100 and desmin was negative. Expression of smooth muscle actin was positive in 2 cases, and both cases were also positive for stromylesin-3. CD34 was positive in 2 cases. DISCUSSION: DMF is an extensive tumour capable of attaining large diameters and must be completely excised. The main differential diagnoses of DMF are dermatofibrosarcoma protuberans, dermatofibroma, fibrous hamartoma, myofibromatosis and cheloid. It can be identified based on various factors, whether clinical (young age, extensive lesion), histological (horizontal proliferation in the reticular dermis) or immunohistochemical (positive expression of calponin).

[Ungual lesions in lupus erythematosus: A retrospective study of 14 patients]. / Lupus érythémateux et atteinte unguéale : étude rétrospective sur 14 patients.

INTRODUCTION: There are few studies focusing on ungual lesions in patients with lupus erythematosus (LE). The aim of this study is to describe our experience with ungual lesions in LE patients. MATERIALS AND METHODS: A multicentric retrospective descriptive study was performed at the dermatology departments of the university hospitals in Strasbourg and at the Tenon hospital in Paris and involved reviewing the medical records and photographs of patients with ungual lesions. RESULTS: Fourteen patients were included: 12 (86 %) were women with a median age of 38 years (28-78 years). All patients had cutaneous LE presenting as follows: 3 isolated forms (21 %), and associated with systemic LE (LES) for remaining 11 patients (79 %). The most frequent ungual or peri-ungual lesions were longitudinal ridging (12 patients, 86 %), onycholysis and cuticular alterations (8 patients each, 57 %), pterygium (7 patients, 50 %), melanonychia, onychoschizia and subungual hyperkeratosis (5 patients with each, 36 %). Among patients with pterygium and onychoschizia, respectively 6 (86 %) and 5 (100 %) presented the discoid LE subtype, while respectively 6 (86 %) and 4 (80 %) had multisystemic involvement. DISCUSSION: Ungual lesions do not appear specific and do not in themselves allow diagnosis of LE. They can in fact occur in other diseases such as connective tissue disorders. However, their diagnosis is important because certain of them, such as pterygium, can lead to severe ungual dystrophia, with functional consequences. In our study, pterygium and onychoschizia appeared to be associated with cutaneous discoid lupus erythematosus and multisystemic involvement. The coexistence of peri-ungual lesions related to cutaneous lupus erythematosus and/or multisystemic involvement does not out differentiation of lupus ungual lesions and post-inflammatory lesions.

[Juvenile dermatomyositis: A series of 22 cases]. / Dermatomyosite de l'enfant. Série descriptive de 22 cas.

PURPOSE: To report on the characteristics of juvenile dermatomyositis (JDM). PATIENTS AND METHODS: This was a retrospective, descriptive, cross-sectional, non-interventional, multicenter study conducted in Alsace between 2000 and 2015. The patients, aged 0 to 16years, had JDM according to both the Bohan and Peter and the EULAR/ACR criteria. RESULTS: A total of 17 girls and 5 boys were included with a median age at disease onset of 7,8years (Q1-Q3: 4.4-12.9). Median duration of JDM and median patient follow-up were 2.8years and 6.2years, respectively. The most common skin symptoms were papules or Gottron's sign (86 %), nail lesions (82 %), erythema of the face (77 %) and eyelids (59 %), photosensitivity (59 %), and calcinosis (27 %). One patient presented papules with a depressed and porcelain-white center ("Degos-like" lesions). One patient had algodystrophy. Two patients were clinically amyopathic. One girl had intestinal vasculitis. Respiratory function tests were abnormal in 27 % of cases. Median treatment duration was 42 months (Q1-Q3: 19-63). Three patients had a monocyclic form, 12 had a polycyclic form, and 7 had chronic disease. CONCLUSION: The frequency of cutaneous and musculoskeletal signs is comparable to that of other large cohorts of JDM. "Degos-like" lesions and algodystrophy have not yet been described in JDM. This study highlights the type and extent of the dermatological manifestations that frequently constitute the presenting complaint in this disease.

[A case of cutaneous tuberculosis evolving for 50 years]. / Un cas de tuberculose cutanée évoluant depuis plus de 50 ans.

INTRODUCTION: Tuberculosis is an infection caused by Mycobacterium (M.) tuberculosis. It is rare in France. Clinical presentations vary, making demonstration of the cause of M. Tuberculosis difficult and rendering diagnosis and management difficult. PATIENTS AND METHODS: A 58-year-old man, born in Morocco, consulted for ulceration of the right forefoot that had been present since the age of 3 years. He had previously consulted at several dermatology departments. He had undergone numerous skin biopsies and bacteriological and mycobacteriological cultures but these did not contribute to the diagnosis. Slow extension and oozing were observed over time and resulted in functional disability. Given the evocative clinical aspect and despite further negative screening for mycobacteria, anti-TB quadrotherapy was prescribed and resulted in complete cure of the lesion. DISCUSSION: This case underscores the difficulty of diagnosing cutaneous tuberculosis. Such a diagnosis must be clinically suspected in the presence of long lasting destructive or verrucous skin lesions that fail to heal, even where cultures are negative, and anti-TB therapy should be putatively prescribed.

[Clinical heterogeneity of poikilodermatous mycosis fungoides: A retrospective study of 12 cases]. / Hétérogénéité clinique du mycosis fongoïde poïkilodermique : étude rétrospective de 12 cas.

INTRODUCTION: Poikilodermatous mycosis fungoides is a rare and indolent clinical variant of mycosis fungoides (MF). It can be difficult to distinguish from poikilodermatous parapsoriasis, a group of chronical dermatoses that may sometimes progress to MF. We aimed to specify the clinical, histopathological and developmental features of these entities by means of a retrospective study of 12 cases followed in our center. PATIENTS AND METHODS: We identified cases of poikiloderma for which a diagnosis of MF or parapsoriasis was made by the physician. Photographs and histological slides were reviewed, and a final diagnosis of MF was made if the International Society for Cutaneous Lymphoma criteria for the diagnosis of early MF were fulfilled. RESULTS: Twelve patients were included, 10 of whom met of the MF criteria. 5 patients had large poikilodermatous patches or thin, well-defined plaques ; 3 patients had the same lesions associated with classical MF lesions ; finally, 4 patients had widespread ill-defined erythematous lesions in a net-like pattern, described as parakeratosis variegata, including 3 MF. 2 patients with well-defined lesions (one associated with classical MF lesions) progressed to the tumoral stage whereas none of the patients with parakeratosis variegata presented such progression. A total of 5 patients had a high skin phototype (IV and V). Two patients had squamous cell carcinoma on poikilodermatous lesions. DISCUSSION: Our study suggests that poikilodermatous MF covers a heterogeneous clinical spectrum comprising on one hand a presentation of delimited lesions sharing classical MF risk of progression, and on the other, an entity similar to parakeratosis variegata, an entity overlooked in the French nomenclature, which was particularly benign in our small series, raising the question of its affiliation to the MF group. This question merits further investigation in a larger-scale study.

[Generalized granuloma annulare: A clinicopathological study]. / Granulome annulaire généralisé : étude anatomoclinique.

BACKGROUND: Granuloma annulare (GA) is a benign granulomatous skin disorder that is generalized (GGA) in 15 % of cases. Although many case reports describe a relationship between GGA and systemic diseases, few large series have been published, and their association is debated. We present herein a series of GGA in order to describe their clinical and histological features. PATIENTS AND METHODS: We included all biopsy-proven cases of GA presenting at the dermatopathology laboratory of Strasbourg where generalized (i.e. over 10 lesions). Clinical features were obtained from patients' medical files. RESULTS: We included 35 GGA, with a sex ratio of 0.5. The mean age was 54 years. Lesions were annular or non-annular in equal measure and were symptomatic in 25 % of cases. Most patients (77 %) had an associated disease, already known in 60 % of cases, including dyslipidemia (27 %), diabetes mellitus (20 %), immunosuppressive drugs (17 %), atopy (17 %), auto-immune disease (17 %), hematological disease (14 %), and cancer (9 %). Histological analysis revealed the predominant pattern to be interstitial (54 %) rather than palisading (20 %), having no correlation with clinical type. Eosinophils were frequent (46 %) in GA but were not correlated with systemic disease or drug taking. Among the 40 % of patients treated, 50 % had a successful outcome on topical corticosteroids, doxycycline, antimalarial drugs or phototherapy. DISCUSSION: GGA differs from localized GA, which is mostly associated with an already known systemic disease, whether metabolic, infectious or neoplastic, uncorrelated with clinical or histological features, and screening is necessary.