Erythematous brownish plaques with an annular configuration.

Granuloma annulare (GA) and cutaneous sarcoidosis show clinicopathological overlap and they are also aetiopathogenically related. Given the similarities of sarcoidal GA and sarcoidosis, and the reports of association of sarcoidal GA with systemic sarcoidosis, this diagnosis should prompt further investigation to exclude systemic involvement. Being aware of the subtle histopathological clues is of the utmost importance for an accurate diagnosis of this rare variant, but correlation with the clinical setting and use of ancillary investigations are also warranted to confidently exclude sarcoidosis.

[Sarcoid Granulomas Over Scars: Beyond Sarcoidosis]. / Granulomas Sarcóides Sobre Cicatriz: Além da Sarcoidose.

Sarcoid granulomas can be found in a wide range of diseases and differentiating sarcoidosis from a sarcoid-like reaction may be a challenge. We present a woman with erythematoviolaceous papulonodular lesions located on the ears where piercings were placed. A skin biopsy showing an infiltrate of sarcoid and focal tuberculoid granulomas did not exclude sarcoidosis. There was a slight increase in the level of angiotensin-converting enzyme. Systemic involvement due to sarcoidosis was excluded. Epicutaneous tests performed revealed a strong positive reaction to palladium and nickel, supporting the diagnosis of granulomatous contact dermatitis. There are only a few reports of granulomatous contact dermatitis to palladium with piercings as the source of sensitization. The formation of sarcoid granulomas can represent either a sarcoid-like reaction or a form of cutaneous sarcoidosis, and patch tests are essential in order to establish the diagnosis.

Os granulomas sarcóides podem ser encontrados num vasto leque de doenças, pelo que diferenciar a sarcoidose de uma reação tipo sarcóide constituiu um desafio. Apresentamos o caso de uma jovem com lesões papulonodulares eritematovioláceas localizadas nos pavilhões auriculares sobre os locais de aplicação de piercings. A biópsia cutânea mostrou um infiltrado constituído por granulomas sarcóides ou focalmente tuberculóides, não excluindo a hipótese de sarcoidose. O estudo complementar, apesar de uma discreta elevação da enzima conversora da angiotensina, excluiu envolvimento ocular ou pulmonar por sarcoidose. Perante a localização das lesões foram realizados testes epicutâneos que revelaram uma reação fortemente positiva ao paládio e ao níquel, apoiando o diagnóstico de dermatite de contacto granulomatosa. Estão descritos escassos casos de dermatite de contacto granulomatosa ao paládio tendo o metal dos piercings como fonte de sensibilização. A formação de granulomas sarcóides pode representar quer uma reação tipo sarcóide, quer uma forma de sarcoidose cutânea, e os testes epicutâneos adquirem um papel fundamental no diagnóstico correto.

Does the pattern of lymphatic drainage influence the risk of nodal recurrence in trunk melanoma patients with negative sentinel lymph node biopsy?

Abstract Background: There are conflicting data regarding the prognostic value of the lymphatic basin drainage pattern in melanoma patients and the evidence is scant in the setting of negative sentinel lymph node biopsy. Objective: To investigate whether the pattern of lymphatic basin drainage influences the risk of nodal disease in patients with melanoma of the trunk and negative sentinel lymph node biopsy. Methods: A case series of patients with trunk melanoma and negative sentinel lymph node biopsy was retrospectively evaluated. Clinicopathological features, the pattern of lymphatic drainage and nodal, metastatic, and overall recurrence-free survival were reviewed. Results: Of the 135 patients included, multiple lymphatic basin drainage was identified in 61 (45.2%). Ten of the 74 (13.5%) patients with single drainage developed nodal recurrence, compared with 2 of the 61 (3.6%) patients with multiple drainages (p = 0.04). Nodal recurrence-free survival was significantly longer in the group with multiple drainages than in the group with single drainage (175.6 vs. 138.7 months; p = 0.04). In multivariate analysis, single drainage was associated with a higher risk of nodal recurrence (HR = 4.54; p = 0.05). No significant differences in metastatic and overall recurrence-free survival were found between groups. Study limitations: Retrospective analysis, single-center study, small sample, detailed histopathologic information not always present. Conclusions: In patients with trunk melanoma and negative sentinel lymph node biopsy, multiple lymphatic basin drainage may be an independent risk factor for nodal disease recurrence. This factor may help to identify patients with negative sentinel lymph node biopsy with a higher risk of nodal recurrence.

Does the pattern of lymphatic drainage influence the risk of nodal recurrence in trunk melanoma patients with negative sentinel lymph node biopsy?

BACKGROUND: There are conflicting data regarding the prognostic value of the lymphatic basin drainage pattern in melanoma patients and the evidence is scant in the setting of negative sentinel lymph node biopsy. OBJECTIVE: To investigate whether the pattern of lymphatic basin drainage influences the risk of nodal disease in patients with melanoma of the trunk and negative sentinel lymph node biopsy. METHODS: A case series of patients with trunk melanoma and negative sentinel lymph node biopsy was retrospectively evaluated. Clinicopathological features, the pattern of lymphatic drainage and nodal, metastatic, and overall recurrence-free survival were reviewed. RESULTS: Of the 135 patients included, multiple lymphatic basin drainage was identified in 61 (45.2%). Ten of the 74 (13.5%) patients with single drainage developed nodal recurrence, compared with 2 of the 61 (3.6%) patients with multiple drainages (p = 0.04). Nodal recurrence-free survival was significantly longer in the group with multiple drainages than in the group with single drainage (175.6 vs. 138.7 months; p = 0.04). In multivariate analysis, single drainage was associated with a higher risk of nodal recurrence (HR = 4.54; p = 0.05). No significant differences in metastatic and overall recurrence-free survival were found between groups. STUDY LIMITATIONS: Retrospective analysis, single-center study, small sample, detailed histopathologic information not always present. CONCLUSIONS: In patients with trunk melanoma and negative sentinel lymph node biopsy, multiple lymphatic basin drainage may be an independent risk factor for nodal disease recurrence. This factor may help to identify patients with negative sentinel lymph node biopsy with a higher risk of nodal recurrence.

Specific cutaneous infiltrates in patients with haematological neoplasms: a retrospective study with 49 patients.

BACKGROUND: Haematological neoplasms account for around 9% of all cancers, and they are recognised as an important cause of skin infiltration. However, studies analysing cutaneous metastasis of haematological neoplasms are scarce. We describe the clinical spectrum and outcomes of specific cutaneous manifestations of leukaemias, lymphomas, multiple myeloma (MM), and blastic plasmacytoid dendritic cell neoplasm (BPDN) and make a review of the literature. METHODS: Data from 49 patients diagnosed with secondary cutaneous infiltration of systemic haematological neoplasms over the last 10 years in a tertiary dermatology centre were retrospectively collected, and clinical-evolutive features were analysed. RESULTS: Most cases were lymphoma (44.9%, n = 22), followed by leukaemia cutis (38.8%, n = 19), secondary plasmacytoma (10.2%, n = 5) and BPDN (6.1%, n = 3). Nodules were the predominant type of lesion, and most patients presented with multiple (≥3) lesions. In 51% (n = 25) of cases, cutaneous infiltration was detected before the diagnosis of the underlying malignancy. The patients in diverse nosological groups did not differ in terms of survival (P = 0.052). CONCLUSIONS: We recognise the clinical heterogeneity of specific cutaneous infiltrates. The high proportion of cases in which skin involvement was key to the diagnosis of systemic malignancy emphasises the role of the dermatologist in recognising and correctly managing these patients.

Coats plus syndrome (cerebroretinal microangiopathy with calcifications and cysts-1): A case report.

We present a 6-year-old girl with skin hyperpigmentation, leukoplakia, and onychodystrophy, the classic mucocutaneous triad usually associated with dyskeratosis congenita. The patient also had premature graying of the hair, bone marrow failure, hepatitis, exudative retinopathy, osteopenia with multiple long bone fractures, and intracranial calcifications and brain cysts. Coats plus syndrome is a rare disease with a clinical and genetic overlap with dyskeratosis congenita. This disease is reviewed, with a focus on the pathogenesis of the genetic anomalies and its background as a telomere biology disorder.

Conradi-Hünermann-Happle syndrome: report of a novel heterozygous mutation on the emopamil-binding protein gene, c.333delC.

Conradi-Hünermann-Happle Syndrome, also called X-linked rhizomelic chondrodysplasia punctata, is a rare genodermatosis that presents with cutaneous, skeletal, and ophthalmological abnormalities. Herein, we report a full-term newborn that presented at birth with scattered blaschkolinear bands of adherent scales and scalp erosions in a spiral distribution. Genetic analysis of emopamil-binding protein gene revealed a previously undescribed heterozygous mutation of c.333delC.

Hypersensitivity to antibiotics in drug reaction with eosinophilia and systemic symptoms (DRESS) from other culprits.

BACKGROUND: Antibiotics have been implicated in the reactivation of exanthema and systemic involvement in drug reaction with eosinophilia and systemic symptoms (DRESS); however, it is not clear whether these patients become sensitized to the antibiotic. OBJECTIVE: To evaluate if, after DRESS, patients become sensitized to antibiotics. METHODS: We retrospectively reviewed the patch test (PT) data and clinical files of DRESS patients who were administered antibiotics during DRESS from other culprits. RESULTS: Nine patients out of 17 (53%) were positive to antibiotics in PT: six to the penicillin group and three to cephalosporins (including one patient with additional positivity to vancomycin). Considering the eight patients who were negative to antibiotics in PT, seven were exposed to a fluoroquinolone. Four cases were patch tested again and three remained positive to antibiotics 2 to 5 years thereafter. Two patients with positive PT results had an accidental re-exposure to antibiotics and developed a maculopapular exanthema without systemic symptoms. CONCLUSION: Exposure to antibiotics during DRESS or its prodromal phase could enhance sensitization to antibiotics, as confirmed by a positive PT. Reproducibility of positive PTs to antibiotics after several years and reactivation after re-exposure support that T-cell-mediated hypersensitivity to antibiotics in the setting of DRESS is a specific reaction.

Erythema nodosum and sarcoid granulomas - letting the cat out of the bag.

A 41-year-old woman presented with a violaceous papule on the dorsum of the hand, large ipsilateral axillary lymphadenopathy, and tender, erythematous, subcutaneous nodules on the legs. Accompanying signs included fever, ankle swelling, and bilateral red eye. She recalled having a previous exposure to kittens one month before and had a positive family history for sarcoidosis. Histological examination of the hand lesion showed sarcoidal granulomas with positive Bartonella henselae DNA, whereas a biopsy done on the leg nodules was compatible with erythema nodosum. Cat scratch disease (CSD) typically presents as a tender regional lymphadenopathy preceded by an inoculation papule with spontaneous resolution occurring between 8-16 weeks. Cutaneous manifestations of CSD are rare, with erythema nodosum accompanying only 0.6% of cases. Although speculative, the background of a positive family history for sarcoidosis may explain the atypical presentation of this case, with red eye, persistent arthralgia, and associated sarcoidal granulomas.