Franco Rongioletti

Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review

Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud’s phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct diagnosis and appropriate management.

Successful Secukinumab treatment of generalized pustular psoriasis and erythrodermic psoriasis

with intravenous acyclovir. Unfortunately, his clinical condition rapidly worsened and the patient died a few days later on the ICU. Autopsy evaluation showed a centrilobular and confluent haemorrhagic necrosis of the liver. Culture results were positive for VZV. Test results for HIV, HSV and CMV were negative. The conclusion of the autopsy was fatal haemorrhagic liver necrosis due to a primary VZV infection. Our patient had initially been admitted for an ulcerative Erythema Nodosum Leprosum (ENL). Precipitating factors for ENL include hormonal changes, anaemia, diabetes, infections, vaccinations and psychological stress. In our patient, the dental infection may have been the primary trigger for the development of ulcerative ENL. Dermatological manifestations of ENL generally range from erythematous papules, plaques, nodules and bullae to painful subcutaneous nodules often in the face or extensor surfaces of the limbs. Characteristically, the bullae are large and haemorrhagic and tend to ulcerate rapidly (Figs 1 and 2). Tragically, after his initial improvement this patient contracted a VZV infection. Varicella is usually a benign infection in childhood but in adults, immigrants from tropical countries and immunocompromised patients it is known to be associated with serious complications such as is the case in a fulminant hepatitis (Fig. 3). In this patient, the lack of immunoprotection against the VZV combined with immunosuppressive medication for his ENL proved to be a fatal combination for this patient. Due to his immunosuppressed status, the infection could rapidly spread and lead to a massive haemorrhagic necrosis of the liver. We therefore advise that appropriate measures in terms of strict isolation should be considered when dealing with immigrant patients who have no prior childhood history of chickenpox. These patients must be considered to have an increased susceptibility for complications due to VZV infections.

Self-healing juvenile cutaneous mucinosis: Clinical and histopathologic findings of 9 patients: The relevance of long-term follow-up

Background: Self‐healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder, and its pathogenesis and long‐term prognosis are unknown. Objective: To elucidate the clinical and histopathologic characteristics, pathogenesis, and outcome in patients with SHJCM. Methods: Retrospective study of 9 patients with SHCJM. To complement initial findings, data collection forms were sent to the referring physicians. Results: All patients had an acute onset of firm nodules. Of the 9 patients, 6 presented initially with waxy papules on the dorsum of the hands; 5 suffered from periorbital edema, and 6 had a febrile prodrome. Histopathologic assessment of the papules revealed dermal mucin deposition, whereas the nodules showed proliferative fasciitis‐like features or nonspecific chronic lobular panniculitis. Laboratory studies elicited evidence of active viral infection in 2 patients (human herpes virus 6 and rotavirus). Seven cases had spontaneous resolution within 6 months, and 2 patients with incomplete resolution showed subsequent transition to fibroblastic rheumatism and an autoinflammatory rheumatologic disease, respectively. Limitations: This was a retrospective study with incomplete data from referring physicians. Conclusions: Although spontaneous complete regression is expected, patients with SHJCM need long‐term follow‐up because of the possible development of dematorheumatolgic conditions. The pathogenetic role of microbial agents deserves further investigation.

Successful ustekinumab treatment of noninfectious uveitis and concomitant severe psoriatic arthritis and plaque psoriasis

We report the first successful treatment of noninfectious uveitis with ustekinumab in a patient with severe concomitant psoriasis and psoriatic arthritis who failed to respond to conventional immune suppressants and with contraindications to tumor necrosis factor alpha inhibitors.

Skin manifestations of POEMS and AESOP syndrome in the same patient revealing plasma cell dyscrasia

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin signs) and AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndromes are rare paraneoplastic conditions due to an underlying plasma cell dyscrasia. We report a 70‐year‐old patient with the rare coexistence of POEMS and AESOP syndromes and in whom skin signs, that differ both clinically and histologically, were the clues to the diagnosis of a plasma cell disorder. Vascular endothelial growth factor‐A overexpression seems to be the common pathogenetic link of the different clinicopathological presentations of the skin lesions.

Methimazole-induced chronic cutaneous lupus erythematosus.

T. Dainichi, T. Tanaka, K. Izawa, R. Nishikomori, K. Kabashima* Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan, Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan, Singapore Immunology Network (SIgN) and Institute of Medical Biology, Agency for Science, Technology and Research (A*STAR), Biopolis, Singapore *Correspondence: S. Nakamizo and K. Kabashima. E-mails: s.nakami@ kuhp.kyoto-u.ac.jp; kaba@kuhp.kyoto-u.ac.jp

High‐dose intravenous immunoglobulin therapy for scleromyxoedema: a prospective open‐label clinical trial using an objective score of clinical evaluation system

Scleromyxoedema is a primary fibro‐mucinosis whose therapy is still challenging.

Diagnostic challenges in the mature patient: Growing old gracefully

Skin diseases in the elderly are frequent and sometimes misleading, due to age-related changes that alter the clinical presentation. Clinicopathologic correlation is often the clue for distinguishing among various pathologies for which clinical signs are more striking than the histopathologic findings. Pitfalls are always lying in wait to ambush inexperienced dermatologists, but usually the microscope provides subtle hints to solve the quandary. In an attempt to familiarize physicians with emerging skin care demands of older patients, we have reviewed major diagnostic challenges in this group of patients: age-related changes, more common skin pathologies in mature patients, manifestations of comorbidity, skin tumors, and paraneoplastic syndromes.

Pigmented fungiform papillae of the tongue: the first case in an Italian woman

nervous system (confusion, delirium, seizures) disturbances, hyperventilation, respiratory alkalosis, coma and death. Topical salicylic acid applied to intact skin in low to moderate doses normally causes minimal systemic effects, but if there is a break in the stratum corneum, as in warts, even application of low concentration preparations has been reported to result in measurable levels (8 mg/dL) of plasma salicylate. We hypothesize that the epidermal disruption caused by the cryotherapy permitted significant systemic absorption of salicylic acid. In a literature review of topical salicylic acid toxicity from 1966 to 2014, toxicity was linked to application in 13 cases of psoriasis, 8 cases of ichthyosis, 2 cases of tinea imbricata, 1 case of erythroderma and 1 case of seborrhoeic dermatitis. Salicylism from topical application of salicylic acid to viral warts has not been reported previously. In all of the previous cases, the salicylic acid preparation was applied over a large body surface area (BSA), often 50–80%. The lowest concentration and smallest BSA producing salicylism that we found reported was a 6% preparation used over 40% BSA. Lowerconcentration (1–2%) preparations have been found to cause salicylism in neonates when used over the whole body. Similar to our case, three previous cases reported symptoms of salicylism on the first day of treatment. In conclusion, we report a patient developing signs of salicylism within the first few hours of treatment with 26% salicylic acid preparation applied under occlusion to around 1% of her BSA. We believe this is the first reported case of salicylism from topical proprietary salicylic acid paint used in the treatment of viral warts on a small BSA.

Necrobiotic granuloma annulare of the penis: a case responding to anti‐tuberculosis therapy with a review of the literature

None declared. ? Funding sources The study was financially supported in part by the National Natural Science Foundation of China (Grant No. 81173400). References 1 Corazza M, Zauli S, Ricci M et al. Shiitake dermatitis: toxic or allergic reaction? J Eur Acad Dermatol Venereol 2014. [Epub ahead of print]. 2 Macfarlane AW, Curley RK, Graham RM, Lewis-Jones MS, King CM. Delayed patch test reactions at days 7 and 9. Contact Derm. 1989; 20: 127–132. 3 Li Baoqing MY. The clinical uses of Lentinan. Hebei Med J 2006; 03: 221–222. 4 Diaz-Corpas T, Mateu-Puchades A, Coll-Puigserver MN, Marquina-Vila A. Flagellate dermatitis after eating shiitake mushrooms. Actas Dermosifiliogr 2011; 102: 830–832. 5 Adler MJ, Larsen WG. Clinical variability of shiitake dermatitis. J Am Acad Dermatol 2012; 67: e140–e141. 6 Ricar J, Pizinger K, Cetkovska P. Shiitake dermatitis: a distinctive clinical entity. Int J Dermatol 2013; 52: 1620–1621. 7 Lippert U, Martin V, Schwertfeger C, Junghans V, Ellinghaus B, Fuchs T. Shiitake dermatitis. Br J Dermatol 2003; 148: 178–179. 8 Department of Commerce of PRC. China Standardization Manual of The Export of Shiitake Mushrooms.2009. URL http://www.21food.cn/ html/news/36/1405833.htm (last accessed 22 June 2014) 9 Fei G. The study of changes in the market of Japanese imports of fresh mushrooms. China Sci Technol Inf 2012; 23: 136–137. 10 Hanada K, Hashimoto I. Flagellate mushroom (Shiitake) dermatitis and photosensitivity. Dermatology 1998; 197: 255–257. 11 Nakamura T. Shiitake (Lentinus edodes) dermatitis. Contact dermatitis 1992; 27: 65–70.