Olga Ciocca

Sjögren's syndrome and localized nodular cutaneous amyloidosis: coincidence or a distinct clinical entity?

OBJECTIVE To report 8 patients with Sjögrens syndrome (SS) and localized nodular cutaneous amyloidosis and to examine serologic and immunohistologic findings that may link the 2 diseases. METHODS The databases for 3 amyloidosis centers were searched for patients with localized nodular cutaneous amyloidosis and SS. Eight patients with this combination were identified, and clinical, serologic, and histologic parameters were retrospectively evaluated. RESULTS Among the 8 patients with a clinical diagnosis of SS, 6 fulfilled the American-European Consensus Group criteria for SS. All of the patients were women in whom SS had been diagnosed at a median age of 47 years (range 30-61 years) and amyloid had been diagnosed at a median age of 60 years (range 42-79 years). The presence of the immunoglobulin light chain type of amyloid (AL amyloid) was confirmed in 4 patients. In 3 of these 4 patients as well as 2 other patients, a light chain-restricted plasma cell population was observed near the amyloid deposits. Progression to systemic amyloidosis was not observed in any patient during a median followup of 3.5 years. CONCLUSION SS should be considered in patients with cutaneous amyloidosis. The combination of cutaneous amyloidosis and SS appears to be a distinct disease entity reflecting a particular and benign part of the polymorphic spectrum of lymphoproliferative diseases related to SS.

Eosinophilic folliculitis occurring in a patient affected by Hodgkin lymphoma

A 25‐year‐old female had a 4‐month history of bilateral cervical lymphoadenopathies, weight loss, mild fever, and severe pruritus. A few months earlier, allergological analysis had been performed which revealed raised IgE (4.6 kU/L) against fungi antigens. A right cervical node biopsy led to histological diagnosis of nodular sclerosis‐type Hodgkin lymphoma. Clinical symptoms, bilateral cervical lymphoadenopathies, and a large mediastinal involvement (revealed by chest X‐ray and thoracic TC) were characteristic of stage IIB (bulky disease). The patient was treated with adriblastine, bleomycin, vinblastine, dacarbazine polychemotherapy (ABVD). Immediately after the administration of this cycle, she developed slightly erythematous, follicular, itchy papules and pustules, localized on her scalp ( Fig. 1 ), trunk ( Fig. 2 ), and thighs. A biopsy from the scalp was obtained under local anesthesia, after patients consent. Histopathologic findings were characterized by a superficial and deep, dense perifollicular and interstitial infiltrate composed mainly of eosinophils, accompanied by few neutrophils and lymphocytes. Eosinophilic folliculitis involved the whole follicle (inferior segment, isthmus and infundibulum), and also the sebaceous gland ( Fig. 3 ). PAS revealed the presence of some spores of Pityrosporum on the epidermis and also in the follicle. Gram stains proved negative. Eosinophilic folliculitis in an immunocompromised patient was diagnosed. Laboratory findings ascertained that the eosinophil count was in the normal range (eosinophils: 1%; white blood cell count: 10 700/mm3). The lesions healed spontaneously in about 10 days. After a second cycle of polychemotherapy (without dacarbazine, because the patient suffered repeated vascular injection injuries due to this chemotherapeutic agent), new crops of lesions in the same areas developed and in a few days faded away spontaneously. The blood clotting analysis performed just before this new cycle of ABV therapy revealed an eosinophil count of 10%, with a white blood cell count of 4500 mm3. Radiotherapy and four additional cycles of ABV polychemotherapy were made; in the meanwhile, the patient did not develop cutaneous lesions. Actually she is free from Hodgkin lymphoma and from eosinophilic folliculitis.

HIV seronegative eosinophilic pustular folliculitis successfully treated with doxicycline

Eosinophilic pustular folliculitis (EPF) is an unusual disease, first described in adult East Asians in 1970 by Ofuji. It is characterized by follicular papules and pustules tending to coalesce and form plaques involving the trunk, face and extremities. In recent years, it has been often associated with human immunodeficiency virus (HIV) infection or with immunosuppressed and/or oncohaematological patients. EPF has been described in immunocompetent adult caucasian patients only occasionally. The diagnosis requires clinical and microbiological features such as sterile folliculitis and histopathological findings characterized by folliculitis and perifolliculitis with eosinophilic infiltrate. We describe an HIV seronegative caucasian male with EPF, allergic to non‐steroidal anti‐inflammatory drugs and indomethacin, treated with oral doxicycline. The treatment led to the complete remission of the lesions within 2 months.

Mycosis fungoides in childhood: Description and study of two siblings

Primary cutaneous T-cell lymphomas are exceedingly rare in children and adolescents. However, mycosis fungoides (MF) is the most frequent primary cutaneous lymphoma diagnosed in childhood. Two cases of MF in siblings (a 14-year-old boy and his 10-year-old sister) are reported. On the basis of clinical features (histopathological and immunophenotypical findings) a diagnosis of MF patch lesions was made in both siblings. Since recent data in the literature have underlined a high frequency of the HLA-DQB1*03 allele in patients with familial MF (including child patients), the HLA profile of the patients was analysed, indicating the presence of a haplotype (HLA-DQB1*03,*03 in the girl, HLA-DQB1*02,*03 in the boy) corresponding with that described in recent literature. Two rare and exceptional cases of MF in siblings are reported, highlighting the presence of a peculiar haplotype.

Clinical, pathological and immunohistochemical effects of arsenical-ferruginous spa waters on mild-to-moderate psoriatic lesions: a randomized placebo-controlled study.

Thermalism and spa treatments are traditionally considered effective in a number of dermatologic inflammatory conditions, yet there is scarce evidence about spring water effectiveness on psoriasis in a daily setting. We enrolled 34 patients with mild-to-moderate psoriasis in a double-blind, randomized, placebo-contralaterally-controlled trial, to evaluate Levico and Vetriolo arsenical-ferruginous water effectiveness on psoriatic lesions by daily 20-minute wet packing for 12 consecutive days. Clinical, histopathologic and immunohistochemical parameters were considered. A statistically significant difference between spa water-treated lesions and placebo-treated lesions in the same patients was demonstrated for histopathologic and immunohistochemical parameters. Since iron ions have an antiproliferative effect on epithelia, and magnesium ions have an anti-inflammatory effect, Levico and Vetriolo water effectiveness on psoriasis could be addressed to their content of these ions.

Sporotrichoid metastases to the skin from cutaneous squamous cell carcinoma in an immunocompetent patient.

Squamous cell carcinoma (SCC) of the skin may metastasize in more than 5% of cases. Metastases usually involve the primary regional lymph nodes in 85% of cases. The skin is rarely involved by metastases of SCC of the skin. The presence of multiple cutaneous metastases in a figurate array is a very rare occurrence, mostly reported in SCC occurring in immunocompromized patients. The case of a 70-year-old immunocompetent patient, with an ulcerated primary SCC on his right palm and 3 nodules in a linear array on his right arm is described. He also presented with homolateral axillary lymphadenopathy. Histopathologic examination from the 3 nodules revealed SCC of variable grading, with focal fusion of the metastases with the overlying epidermis. The patient had been previously treated with chemotherapy and radiotherapy in another Department, with no benefit. Despite right arm amputation and homolateral axillary lymphadenectomy, the patient died 4 months after a procedure, for pulmonary embolism.

Su un caso di panniculite granulomatosa lobulare con pattern sarcoideo e comportamento clinico inaspettato

Panniculitides are an heterogeneous group of inflammatory cutaneous diseases involving the subcutaneous fat. Traditionally, the etiologic diagnosis of these pathologies is difficult because different panniculitides usually present with the same, nonspecific, clinical appearance, and always requires a histopathologic study. The classification of panniculitis is histopathological; this is based both on the localization where the inflammatory infiltrate is more significant (mostly lobular/ mostly septal) and on the blood vessel involvement (with/without vasculitis) [1]. We report the case of a 62 years old man presenting panniculitic lesions for more than two years, yet not well indetificated and treated, because of the relevant discrepancies between the histopathology pattern and laboratory and clinical results.

Meccanobiologia della riparazione tissutale: dalla fisiopatologia alle implicazioni terapeutiche

Molte delle funzioni cellulari sono controllate o mediate da forze meccaniche. Le cellule sono in grado di rispondere a variazioni delle proprieta meccaniche della matrice trasducendo tali informazioni in risposte molecolari mirate: i segnali meccanici sono in grado di determinare lo sviluppo di una cellula staminale, o di modulare il funzionamento di un intero tessuto, e giocano un ruolo centrale in numerose patologie. Inoltre, sulla base delle proprieta meccaniche dell’ambiente circostante le cellule sono in grado di modificare la loro motilita e la loro attivita metabolica, agendo non come passivi recettori degli stimoli meccanici, ma anche manipolando attivamente la matrice nella quale si trovano sia producendone le principali componenti, che modificandone attivamente la tensione meccanica. Di conseguenza, la meccanotrasduzione svolge un ruolo fondamentale nella riparazione e nel rimodella mento tissutale, suggerendo importanti implicazioni terapeutiche, e permettendo di valutare sotto una nuova luce vecchie terapie empiriche spesso sottovalutate.

Ulcere cutanee in corso di crioglobulinemia di tipo I: descrizione di due casi e revisione della letteratura

Le ulcere cutanee sono manifestazioni di condizioni locali e sistemiche alterate, e possono essere la prima manifestazioni di malattie gravi e potenzialmente fatali. La crioglobulinemia di tipo I e una paraproteinemia monoclonale generalmente asintomatica la cui manifestazione d’esordio puo essere la comparsa di lesioni cutanee, generalmente di tipo ischemico o vasculitico, talora con ulcere. Si descrivono due casi di crioglobulinemia di tipo I in cui la manifestazione d’esordio sono state delle ulcere alle gambe con aspetti clinici molto simili. Nel secondo caso, in particolare, la patologia era associata ad una sindrome di Sjogren, condizionandone la prognosi. La diagnosi eziologica delle ulcere cutanee e quindi di fondamentale importanza non solo perche costituisce il primo passo per un trattamento risolutivo delle lesioni, ma anche perche puo permettere di identificare e trattare precocemente malattie sistemiche potenzialmente molto gravi o evolutive delle quali possono essere la prima manifestazione clinica.

Vitiligine e melanoma

La vitiligine e un’affezione caratterizzata dalla presenza di lesioni amelanotiche di forma ovalare o rotondeggiante aventi abitualmente margini netti, e interessa in egual misura il sesso maschile e quello femminile con un picco di incidenza tra la prima e la terza decade. La sua prevalenza e pari a circa l’1% nella popolazione generale. Tuttavia se si considera la popolazione costituita dai pazienti affetti da melanoma, tale prevalenza aumenta a circa il 2.8%. L’insorgenza di vitiligine puo precedere o seguire la diagnosi di melanoma e assume delle caratteristiche cliniche differenti a seconda dell’uno o dell’altro caso. La valutazione della sopravvivenza a cinque anni dalla diagnosi nei soggetti affetti da melanoma e da vitiligine ha consentito di osservare un aumento della sopravvivenza di un valore statisticamente significativo nella popolazione di soggetti affetti sia da vitiligine che da melanoma in stadio III e IV, rispetto ai pazienti affetti solo da melanoma. Questi dati preliminari suggeriscono l’importanza di condurre uno studio clinico retrospettivo per valutare il dato di associazione vitiligine e melanoma e un possibile ruolo prognostico di tale associazione.