The clinical features and histopathologic patterns of folliculotropic mycosis fungoides in a series of 38 cases.

BACKGROUND: The recognition of folliculotropic mycosis fungoides (FMF) may pose diagnostic challenges, owing to the variety of histopathological findings. OBJECTIVE: In this study, our aim is to describe the broad spectrum of the histopathological patterns in a total of 86 biopsies from 38 patients with FMF, together with the clinical features. RESULTS: The most frequent histopathologic pattern was the folliculocentric/folliculotropic pattern, with or without follicular mucinosis. Keratin-filled cysts and comedones were the second most common pattern in the biopsies. Other less common findings included widening of the hair follicle orifis with keratotic plugging, reminiscent of keratosis pilaris, granuloma formation, eosinophilic or suppurative folliculitis and basaloid folliculolymphoid hyperplasia. Coexisting syringotropism was present in some biopsies. The CD4 : CD8 ratio was at least 4 : 1 or more in most biopsies. Grouped follicular papules and patch/plaque lesions with follicular prominence were the most frequent clinical findings. Folliculocentric lesions such as milia, cysts and acneiform lesions, alopecia, loss of hair or eyebrows were also seen. In 6 out of 38 (15.8%) patients, transformation to large-cell lymphoma was observed during the follow-up. CONCLUSION: The awareness and the identification of the various histopathological presentations of FMF by pathologists, as well as by clinicians, are imperative to prevent diagnostic errors.

The role of various immersion liquids at digital dermoscopy in structural analysis.

BACKGROUND: Dermoscopy is a useful method that allows dermal and epidermal structures to be easily analysed non-invasively. AIM: In this study, immersion oil, which is widely used in dermoscopy, and ultrasound gel, which is less preferred, are evaluated comparatively in terms of displaying structural parameters and number of air bubbles in the image. METHODS: A total of 71 nevomelanocytic or non-melanocytic pigmented lesions were taken up for this study. Structural characteristics of the obtained images were assessed by an experienced observer who scored the images in terms of color, pigment network, globule, vascular structure, number of air bubbles and other pigmentation parameters. RESULTS: In the images obtained through immersion oil or ultrasound gel from all of the lesions, no statistical difference was found between the average values of air bubbles and in the evaluation of structural components (t=1.09, P=0.2). In the identification of pigment network in melanocytic lesions, immersion oil was observed to be more appropriate than ultrasound gel (t=0.01, P=0.02). CONCLUSIONS: Ultrasound gel may be preferred in the assessment of mucosa and nail bed lesions. Ultrasound gel is a good alternative compared to immersion oil in pigmented skin lesions as it is cheap and easily removable.

Kaposi sarcoma related to an ectopic hypothalamic adrenocorticotropic hormone-secreting adenoma: case report.

OBJECTIVE: We aim to report a case of Kaposi sarcoma (KS) with Cushing's syndrome caused by endogenic glucocorticoid-induced immunosuppression. CLINICAL PRESENTATION: A 43-year-old woman presented with delirium, hirsutism, fatigue, and hypertension. At the time of presentation, physical findings showed a Cushingoid appearance, with moon-like facies, hirsutism, and hyperpigmentation. Laboratory findings showed the following: adrenocorticotropic hormone, 86.7 pg/mL (normal range, 0-46 pg/mL); baseline cortisol level, 50 microg/dL (normal range, 6.2-19 microg/dL); potassium, 2.2 mEq/L (normal range, 3.5-5 mEq/L); and midnight cortisol level, 33 microg/dL. Serum cortisol levels failed to suppress after low and high doses of dexamethasone; these findings confirmed the diagnosis of ectopic adrenocorticotropic hormone production. Magnetic resonance imaging revealed a 12 x 15-mm, round, hypothalamic mass lesion in the center of the median eminence. INTERVENTION: Endoscopic biopsy from the floor of the third ventricle was performed, and pathological examination of the lesion showed a diffuse adrenocorticotropic hormone-secreting adenoma. The patient developed diffuse skin lesions that were proven to be a KS by skin biopsy while she was prepared for transcranial surgery. After surgical removal of the adenoma, she became hypocortisolemic and required cortisol replacement. Within 1 month after surgery, all KS lesions disappeared spontaneously. CONCLUSION: Excessive cortisol may induce immunosuppression. KS is one of the most common malignant tumors of patients with immunosuppression. To the best of our knowledge, this is the first case of Cushing's syndrome with KS caused by endogenous glucocorticoid-induced immunosuppression.

Non-pustular palmoplantar psoriasis: is histologic differentiation from eczematous dermatitis possible?

The differential diagnosis of psoriasis affecting palmoplantar skin might be troublesome because of the anatomic properties of this region. Both palmoplantar psoriasis and eczematous dermatitis of this skin area share similar histologic features. The punch biopsies from 17 patients with psoriasis and 25 with eczematous dermatitis were evaluated, blind to the clinical diagnosis. Vertically situated multiple foci of parakeratosis, alternating with orthokeratosis,were the only statistically significant feature in the differential diagnosis of palmoplantar psoriasis [76.5% (13/17), p = 0.005]. In contrast,multiple foci of parakeratosis [70.6% (12/17)], loss of granular layer at least in focal areas [41.2% (7/17)], presence of neutrophils at the summits of parakeratosis [4% (1/17)], presence of neutrophils and/or plasma in the parakeratotic foci [94.1% (16/17) and 11.8% (2/17)],psoriasiform epidermal hyperplasia [88.2% (15/17)], spongiosis restricted to the lower parts of the epidermis [47.1% (8/17)],dyskeratotic cells [82.4% (14/17)], thinning of suprapapillary plate[58.8% (10/17)], edema of the papillary dermis [29.4% (5/17)],presence of tortous and dilated capillaries in the papillary dermis[52.9% (9/17) and 76.5% (13/17)] and extravasated erythrocytes[29.4% (5/17)] were found to be more common in palmoplantar psoriasis compared with eczematous dermatitis; but none of them was statistically significant. Interestingly, spongiotic vesicles were seen in most of the patients with psoriasis [76.5% (13/17)]. In conclusion,according to our findings, many features of palmoplantar psoriasis overlapped with those of eczematous dermatitis. However, detection of multiple parakeratotic foci, placed vertically, alternating with orthohyperkeratosis, could be considered in favor of palmoplantar psoriasis.

Alemtuzumab in Sézary syndrome: efficient but not innocent.

Mycosis fungoides is the most common form of cutaneous T-cell lymphomas. The related Sézary syndrome is a more aggressive form in which the skin is diffusely affected and the peripheral blood is involved. Although easily managed during its early phases, late-stage mycosis fungoides/Sézary syndrome is usually difficult to treat and becomes refractory to chemotherapy. Recently, promising case-based results have been obtained with alemtuzumab, a humanized immunoglobulin G1 monoclonal antibody that binds to CD52 cell surface antigens, in the treatment of advanced stage mycosis fungoides/Sézary syndrome. We report a case of Sézary syndrome treated successfully with alemtuzumab but who died of treatment-related infection.

Dissociative identity disorder presenting as dermatitis artefacta.

Dermatitis artefacta is a rare psychiatric condition characterized by rubbing of skin blisters and denial of self-infliction. Dissociation may be comorbid with self-injurious behavior. A background of emotional disturbances during formative years and in later life often results in feelings of isolation and insecurity, which can lead to dissociation as a primary defense mechanism used to overcome traumatic events. In this case report, we describe a female patient with dermatitis artefacta associated with dissociative identity disorder. The patient was a 14-year-old girl. Multiple large, deep ulcerations with unnatural shapes were seen on her left forearm. The ulcerations were thought to be self-inflicted. Psychiatric examination revealed that she had a different identity, and inflicted the lesions when this was assumed. This case leads us to suggest that patients with dermatitis artefacta might have comorbid dissociative experiences, which cannot be identified easily.

The correlation of angiogenesis with metastasis in primary cutaneous melanoma: a comparative analysis of microvessel density, expression of vascular endothelial growth factor and basic fibroblastic growth factor.

AIM: To establish whether there is a correlation between angiogenesis and metastasis in primary cutaneous melanoma (PCMM). METHODS: We studied the microvessel density and the expression of vascular endothelial growth factor (VEGF) and basic fibroblastic growth factor (bFGF) in 22 cases of PCMM with metastasis at presentation (metastatic group) and 28 cases of PCMM without metastasis for 24 months or more (non-metastatic group). Microvessels were stained with CD31/PECAM-1 antibody and counted. We assessed the proportion of VEGF expression in tumour cells, lymphocytes infiltrating the tumour (TIL) and lymphocytes at the periphery of the tumour, as well as the proportion of bFGF expression in tumour cell cytoplasms, nuclei and intra- and peritumoral vessels. RESULTS: An increased microvessel density was detected in the metastatic group (15-33 [24.09 +/- 5.55] versus 2-24 [12.96 +/- 6.02]). Moreover, enhanced expression of VEGF in tumour cells and peritumoral lymphocytes (Chi-square p = 0.038 and p = 0.018) and bFGF in peritumoral vessels (chi(2) p = 0.013) correlated with the simultaneous presence of melanoma metastasis in PCMM. Furthermore, microvessel density was correlated with the expression of bFGF in peritumoral vessels (rs = 0.53, p = 0.049) and VEGF in tumour cells (rs = 0.37, p = 0.019). CONCLUSION: Microvessel density as well as the expression of both VEGF and bFGF might be informative concerning the progression of melanoma.

Evaluation of time-dependent response to psoralen plus UVA (PUVA) treatment with topical 8-methoxypsoralen (8-MOP) gel in palmoplantar dermatoses.

BACKGROUND: Topical psoralen plus UVA (PUVA) is an effective treatment for localized forms of eczema, psoriasis, and palmoplantar pustulosis, which avoids some of the undesirable side-effects of systemic psoralens. Aims In this study, the efficacy of topical PUVA treatment with 8-methoxypsoralen (8-MOP) gel was compared with placebo plus UVA in chronic recurrent palmoplantar dermatoses. METHODS: Twenty-two patients with palmoplantar disease (11 with psoriasis vulgaris, six with eczema, and five with pustulosis) were enrolled in the study. The study design was a left-right comparison: one hand or foot was treated with 8-MOP 0.01% gel plus UVA, whilst the contralateral hand or foot received placebo and UVA for 6 weeks. Twenty minutes after application of the gel, both sides were exposed to UVA. The treatment regimen was three times a week, and the UVA dose was increased weekly by 20%. RESULTS: A comparison of the pre- and post-treatment scores with regard to the severity of the clinical picture and the infiltration of plaques showed a significant decrease (from 7.5 +/- 2.0 to 2.5 +/- 2.1 and from 2.0 +/- 0.7 to 0.3 +/- 0.5, respectively) in the sites treated with 8-MOP gel compared with placebo after 6 weeks. CONCLUSION: The results of the study indicate that at least 18 courses of local PUVA within 6 weeks, with a cumulative dose of 87 J/cm(2), are required to induce a significant decrease in the disease severity and an improvement in the infiltration of plaques due to 8-MOP gel at a concentration of 0.01% when treating chronic recurrent palmoplantar dermatoses.

Milia in regressing plaques of mycosis fungoides: provoked by topical nitrogen mustard or not?

OBJECTIVE: To report three cases of mycosis fungoides with milia formation in the regressing lesions. PATIENTS AND SETTING: Dermatology clinic of a university hospital (referral center). Three patients with mycosis fungoides with body surface involvement of 10% in one case (stage IIb) and exceeding 30% in two cases (stages IIb and III). All patients were treated with photochemotherapy and topical nitrogen mustard ointment in a concentration of 0.01%. After approximately 3 months multiple milia erupted on regressing plaques. RESULTS: The presence of milia was evident and was confirmed by histopathology. Regression of mycosis fungoides was noted in these plaques both clinically and in comparison with the pretreatment histologic appearance. Two of the patients showed a histological picture of follicular mucinosis. CONCLUSIONS: We do not know the significance of milia in mycosis fungoides (MF). However, we suggest that follicular rupture or a degenerative process might result in milia formation.