Round fingerpad sign: an early sign of scleroderma

We describe a clinical skin sign in scleroderma termed round fingerpad sign. This term refers to disappearance of the peaked contour on fingerpads and replacement with a hemisphere-like fingertip contour; this change is especially apparent on the ring fingers. A positive round fingerpad sign was found in 72 of 72 ring fingers in 36 patients with progressive systemic sclerosis in 69 of 72 ring fingers in patients with mixed connective tissue disease, and in 24 of 24 ring fingers of patients with Raynaud's phenomenon and sclerodactyly. In contrast, a negative round fingerpad sign was seen in 240 of 240 fingers in normal women (controls). The high sensitivity of this sign is noteworthy. A positive round fingerpad was seen in sign not only typical scleroderma patients but also in groups with less severe skin sclerosis (i.e., in those patients with mixed connective tissue disease or those with Raynaud's phenomenon and sclerodactyly). The sign is a new and useful clinical marker for the early diagnosis of scleroderma.     

Blau syndrome: a new kindred

Blau syndrome is a rare condition typically defined by granulomatous arthritis, skin eruption, and uveitis occurring in the absence of lung or other visceral involvement. Other characteristic physical findings include synovial cysts and camptodactyly. We describe a new kindred demonstrating autosomal dominant inheritance and anticipation.     

Wells' syndrome. Recurrent granulomatous dermatitis with eosinophilia.

Two cases of granulomatous dermatitis with eosinophilla (Wells' syndrome) are reported. With Wells' original four cases, these two cases define a distinctive dermatosis with onset as cellulitis and formation of solid edema and either final spontaneous resolution or resolution with steroid therapy. Microscopic study showed diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. Biopsy of muscle and fascia showed comparable fasciitis and eosinophilic myositis. Immunofluorescence in one case disclosed fibrin in the dermis and lgM, lgA, and C3 in the blood vessels of the muscle. Recurrences of the lesions often appeared to be related to drug administration or surgery.     

间质肉芽肿性皮炎

报告1例间质肉芽肿性皮炎。患者女,17岁。因左胸部和左上肢出现呈带状分布、无自觉症状的丘疹和结节半年就诊。免疫学检查示类风湿因子阳性。组织病理检查显示真皮间质和血管周围弥漫而致密的以组织细胞为主的炎性细胞浸润,部分组织细胞核大,有异形：炎性浸润细胞中还有中性粒细胞和少量淋巴细胞、浆细胞、多核巨细胞,局部可见灶性胶原纤维变性、坏死,未见嗜酸性粒细胞浸润和血管炎改变。阿新蓝染色阴性。免疫组化染色示CD68强阳性,S-100蛋白、AE1／AE3、SMA、结蛋白、LCA、CD45RO、CD20、CD21、CD23、CD31、CD34、CD1a和Ki-67均阴性。符合间质肉芽肿性皮炎的诊断。     

Non-infectious granulomatous dermatitis: a clinicopathological study

BACKGROUND: Granulomatous dermatitis frequently presents a diagnostic challenge to dermatopathologists because an identical histologic picture is produced by several causes, and conversely, a single cause may produce varied histologic patterns. METHODS: A retrospective analysis of skin biopsies received over a period of 7 years was performed, and cases of non-infectious granulomatous dermatitis diagnosed on histopathological examination were retrieved. RESULTS: Out of a total of 586 cases of granulomatous dermatitis, 71 cases (12.11%) were categorized as non-infectious granulomatous dermatitis on the basis of clinicopathological findings. Further subcategorization was done based on morphology of granulomas as epithelioid granulomas; 15 cases of sarcoidosis, 21.1%, one case of Crohn's vulvitis, 1.4%, necrobiotic granulomas; 11 cases of granuloma annulare, 15.4%, two cases of rheumatoid nodule, 2.8%, 10 cases of foreign body granulomas, 14.0%; 32 cases of miscellaneous group, 45%. CONCLUSIONS: Morphology alone is seldom specific and cannot be used as a diagnostic tool for identification of specific diseases. Adequate clinical data and work up in combination of pathological resources can help in elucidation of specific etiology of granulomatous dermatitis. Mohan H, Bal A, Dhami GP. Non-infectious granulomatous dermatitis: a clinicopathological study.     

Persistent pigmented purpuric dermatitis: granulomatous variant

The persistent pigmented purpuric dermatitides (PPPD) are a spectrum of dermatologic disorders characterized by petechial and pigmented macules usually confined to the lower limbs. Their etiology is unknown and several clinical variants are recognized. At the microscopic level they are characterized by angiocentric lymphocytic inflammation, red blood cell extravasation and hemosiderin deposition. A granulomatous variant of the PPPD has recently been described and to date eleven cases have been reported in the literature. In contrast to the conventional type, this variant is characterized histopathologically by ill-defined, non-necrotizing granulomata admixed with the lymphocytic inflammatory background. Although initially the granulomatous variant of the PPPD was thought to occur only in Asian patients, this sole racial predilection has not been substantiated. A tenuous association with hyperlipidemia has been noted but this requires further study. The principal importance of recognizing this entity lies in the need to include it in the histopathological differential diagnosis of granulomatous dermal infiltrates. We report here an additional patient with the granulomatous variant of PPPD and elaborate on this entity in the context of existing information in the literature.     

Tooth pits: an early sign of tuberous sclerosis

The pit-shaped enamel defects seem to be pathognomonic of tuberous sclerosis and its detection may be an important help in the early diagnosis of this disease, especially in oligosymptomatic cases and when the dermatologic signs are still absent or only slightly evident.     

Sporadic Blau Syndrome With Onset of Widespread Granulomatous Dermatitis in the Newborn Period

Abstract:  Blau syndrome is a dominantly inherited, chronic autoinflammatory disorder characterized by the clinical triad of granulomatous dermatitis, symmetric arthritis, and recurrent uveitis with onset below 4 years of age. It is caused by activating mutations in the nucleotide-binding oligomerization domain 2 ( NOD2 ) gene, previously referred to as CARD15 gene. Noncaseating granulomas in affected tissues are the pathologic hallmark of the condition. We report the lifelong severe disease course in a 14-year-old Caucasian boy with sporadic Blau syndrome. Unusually, granulomatous dermatitis started in the first week of life. Whereas skin involvement faded away spontaneously in subsequent years, polyarthritis and anterior uveitis appeared in the second and third year of life respectively. Mutational analysis of the NOD2 gene revealed a missense mutation (R334W) previously detected in other Blau syndrome pedigrees. With this report, we would like to stress the rare possibility of Blau syndrome in generalized papular rashes of infancy and the importance of histopathologic study for clarification. The finding of early-onset widespread granulomatous dermatitis should prompt eye and joint examination in regular intervals and entail mutational analysis of the NOD2 gene.     

Childhood granulomatous periorificial dermatitis: a controversial disease

The etiology, diagnosis and treatment of childhood granulomatous periorificial dermatitis (GPD) are highly controversial. Some authors underline the similarities between GPD and perioral dermatitis and consider both as part of a spectrum while other authors regard GPD as a distinctive condition. Clinically GPD is a papulo‐pustular periorificial disease of the face which histopathologically shows a granulomatous perifollicular infiltrate. Because of its granulomatous pattern, GPD also has been related to cutaneous sarcoidosis. The clinical course is benign and self‐limited. Topical steroids are regarded as either the main cause or a worsening factor. While topical treatment is occasionally effective, systemic antibiotic therapy of GPD is usually recommended. We report a “typical” case of GPD and review the literature to discuss the difficulties in its diagnosis and treatment.