Dermatitis Herpetiformis (DH) is a rare, blistering skin disease described by Duhring in 1884.[1] DH is strongly associated with the HLA-DQ2 phenotype, a gateway in which dietary gluten reaches inflammatory cells and stimulates an autoimmune process. The etiopathogenesis involves IgA anti-endomysial antibodies directed against tissue transglutaminase (TG); the presumed skin autoantigen is epidermal TG. IgA/TG immune complexes form locally within the papillary dermis leading to neutrophil chemotaxis and degranulation (which forms neutrophilic abscesses), proteolytic cleavage disrupting the lamina lucida, and blister subepidermal blister formation.[2] Current standard-of-care for DH is oral dapsone and a gluten-free diet. We describe a teenage patient in whom resolution of lesions was achieved with adjuvant use of topical dapsone 5% gel (aczone), the first case in the literature.A 14-year-old male had been suffering recurrent eruptions of blisters on his chest and arms for 16 months. He had been diagnosed with direct immunofluorescent proven DH based on granular IgA deposition in the upper papillary dermis. Though distinguishing LABD (linear IgA bullous dermatosis) from DH is often clinically impossible, the finding of IgA in a granular pattern at the dermoepidermal junction with accentuation in the dermal papillae was specific for DH in our patient. Upon an exacerbated eruption of blisters on the chest and shoulders, the patient presented to our clinic.On physical examination he presented with multiple, welldefined, pink keratotic papules, plaques and diffuse, hypopigmented macules and patches on the chest and shoulders, equally distributed on the left and right side. Primary lesions were counted before treatment and numbered 33 on the left side and 34 on the right side of the chest, respectively.The patient did not maintain a gluten-free diet and was receiving daily oral dapsone (25 mg). Twice daily application of topical dapsone (Aczone gel, 5%) was initiated on the right side of the patient’s chest and Aquaphor ointment to the left. Two physicians were blinded to which side received the medication. The patient was asked to follow up in four weeks.Over the following three days the patient’s skin lesions did not improve on either side of the chest. The oral dosage of dapsone was modified to 25 mg and 50 mg on alternating days. The patient continued to apply the topical products unilaterally as originally prescribed. On a follow-up visit four weeks later, the blinded physicians observed improvement of the lesions on the right side of his chest in comparison to the left. Physical exam demonstrated two remaining ulcerated vesicles on the right chest and five on the left without signs of erosion. It was also noted that relative to the left side, the skin on the right side was significantly smoother. The patient was then allowed to expand usage of topical dapsone to all affected areas and had similar improvements.There have been no reports on treating dermatitis herpetiformis using topical dapsone and the current standard-ofcare remains oral dapsone. Most clinical remissions are related to dietary gluten restriction, however, the gluten-free diet is inconvenient and unacceptable to some patients. Oral dapsone (diaminodiphenylsulfone) is the current treatment and the most effective sulfone but many physicians would prefer a non-systemic treatment choice. Though patients intolerant to dapsone may consider therapy with sulfapyridines, there is significant risk for nephrolithiasis and some patients may not respond at any dose. Although this patient had received oral and topical dapsone simultaneously, he only applied topical dapsone 5% gel to the right side, the same side which demonstrated significant improvement of the lesions. The left side where aquaphor was applied did not show the same level of improvement at four-week follow-up.High doses of oral dapsone simply increase toxicity while providing minimal benefit. The main side effects associated with oral dapsone may be classified as toxic/pharmacologic or idiosyncratic/allergic; these range from hemolytic anemia, the most common complication within 2 weeks after starting therapy, to nephritis and renal failure, precluding the need for strict monitoring of renal function tests.[3] Hemolytic anemia occurs in virtually every patient on oral therapy and may even occur in breastfed infants since dapsone is secreted in breast milk. A notable complication, especially in patients with glucose-6-phophate dehydrogenase (G6PD) deficiency, is hemolysis and methemoglobinemia due to oxidative stress from the hydroxylamine metabolite.[4] Adverse effects of dapsone are generally dose-dependent and are more commonly observed in patients with comorbid conditions such as anemia and cardiopulmonary disease.[3] Other rare complications include agranulocytosis early on, as well as a systemic drug hypersensitivity syndrome, a serious complication that requires medication withdrawal and systemic corticosteroid administration.[5] Regular follow-up visits and routine laboratory monitoring of blood counts are needed for patients receiving treatment with oral dapsone, especially during the first 3 months.[5]Dapsone is clinically useful in diseases containing neutrophilic infiltrates. Dapsone inhibits neutrophil myeloperoxidase, decreasing the damage from the neutrophil respiratory burst pathway mediated by this enzyme. The anti-inflammatory properties of topical dapsone benefit patients with acne and could also hinder the immunologic cascade and accompanying inflammatory process that occurs in DH. We attribute the efficacy of topical dapsone to a local inhibition of neutrophil chemotaxis to N-formyl-methionyl-leucyl-phenylalanine and interference of the CD11b/CD18-mediated neutrophil binding that induces chemoattractant signal transduction and inhibits leukotrienes. IgA adherence may also be inhibited though it remains to be proven.[5]Concerning our review of the literature, we conclude that there is therapeutic explanation for the moderate therapeutic effects seen from use of topical dapsone in DH. Using topical dapsone alone or in combination with a lower dose of oral dapsone is preferable to high doses of oral dapsone. Additionally, facial disease may prove refractory to oral dapsone therapy; topical dapsone gel may provide a more tolerable alternative than breaking facial vesicles and applying a potent corticosteroid gel. In order to further study the utility of topical dapsone in DH, a more extensive trial of topical dapsone is warranted. In summary, aczone appears to be a promising therapeutic agent for patients with dermatitis herpetiformis who are refractory to oral therapy and noncompliant with a gluten-free diet.

Benign fibrous histiocytomas of the skin sometimes extend into the deeper dermis with higher cellularity and show more aggressive clinical courses in comparison with typical dermatofibromas. They may occur either as a true neoplasm or in a reactive process. We describe a case of fibrous histiocytoma which was triggered by tuberculin skin test.

Proliferating pilomatricoma is a proliferative variant of pilomatricoma. Although it is considered as a benign tumor, local recurrence may occur. We report a case of a 49-year-old Japanese man with 3-year history of an asymptomatic subcutaneous tumor in the nuchal area. Histological evaluation demonstrated a cystic lesion lined by a basaloid epithelium at the periphery and filled with eosinophilic cornified material containing shadow cells in upper part of the tumor, and multilobular proliferation of basaloid cells in association with small foci of shadow cells in the remaining part. Based on these findings, the diagnosis of proliferating pilomatricoma was made. No recurrence has been observed during a 3-year follow-up.

inear atrophoderma of Moulin is a rare acquired disorder arising most commonly during childhood or adolescence, occurring equally in both sexes and characterized by hyperpigmented atrophoderma in a unilateral bandlike distribution along the lines of Blaschko. Since Moulin et al described the condition in 1992, only a few dozen cases have been reported. It has been postulated that linear atrophoderma of Moulin may be due to mosaicism.A 66-year-old man pre­sen­ted with a 46-year his­to­ry of evol­ving tan soft atro­phic con­fluent plaques in a striking­ly Blasch­koid dis­tri­bu­tion, in­vol­ving the left up­per back, shoulder, up­per arm, chest and flank. Ini­tial on­set, at age 20, con­sis­ted of a single mildly pru­ritic pink patch on the left back that was un­res­pon­sive to topical anti­fun­gals. Each new le­sion arose simi­larly as a pink pru­ritic patch, sub­se­quent­ly be­co­ming de­pressed, hy­per­pig­men­ted, and asym­pto­ma­tic over se­ve­ral years. Le­sions were never scaly, firm, or indu­rated. Punch biopsy speci­mens were obtained. The clinical and histo­patho­lo­gi­cal features con­firmed the diagnosis of linear atrophoderma of moulin.Our present case has the characteristic clinical and histopathological features of linear atrophoderma of Moulin, but is the first reported case with mild pruritus at the onset of each new lesion and progressing slowly over 46 years. The lack of any systemic symptoms or other complications in our patient reaffirms the benign nature of this skin disease.

Background:Alopecia areata is an autoimmune disease that is affecting anagen hair follicles. The triggers of autoimmunity in patients with alopecia areata remain unknown.Main observation:A 13-year-old boy developed multiple hairless patches of focal hair loss with typical clinical and trichoscopy features of alopecia areata. Mycology examination of the scalp hair and epidermal scrapings reveled massive growth of Alternaria chlamydospora.Conclusion:We hypothesize that fungal antigens (e.g. antigens involved in fungal melanin synthesis) may be possible triggers, contributing to autoimmune reactions in patients with alopecia areata. We discuss research data, which may indirectly support this hypothesis, however the concept has yet to be verified.

Background:Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare autoimmune blistering disorder. The disease may be either idiopathic or druginduced. Over the past 30 years, approximately one hundred LABD cases have been described as induced by a wide range of drugs, chiefly antibiotics.Main observations: We report the case of 37-year-old woman who developed pruritic bullous lesions spread all over the body three weeks after her last dose of cefuroxime axetil. Antibiotic therapy was started due to rhino-sinusitis.Conclusions:In most reported cases of drug-induced LABD, skin lesions occur within the time of drug administration. However, the onset of disease may be even after discontinuation of treatment. It seems that in such cases, other clinical conditions (like infection) act, as cofactors of immunologic response.

Background:Geographic stomatitis is an uncommon oral lesion that presents similar clinical, histopathological and genetic features as those of psoriasis. These findings suggest that this lesion may actually represent an oral manifestation of psoriasis. We report one case of geographic stomatitis and discuss a possible connection between this condition and psoriasis.Main observations:A 37-year-old woman presented with red patches, surrounded by a white border on the labial mucosa and a positive family history of psoriasis. Histopathological examination, immunohistochemical analysis with antibodies against CD4, CD8, CD20, CD68, CD31, and Ki-67 and HLA-A*, -B*, -C*, -DRB1*, -DQA1* and -DQB1* genotyping were performed. Histopathological examination revealed parakeratosis, marked elongation of rete ridges with acanthosis and clubbing, exocytosis, Munro microabscesses, pustule of Kogoj, dilated tortuous vessels at the tip of dermal papillae, and predominant superficial and perivascular lymphocytic chronic inflammatory cell infiltrate. Immunohistochemistry analysis revealed a predominant T-cell subepithelial infiltrate. Based on the referred clinicopathological findings and in the absence of cutaneous lesions, the diagnosis of geographic stomatitiswas confirmed.Conclusions:This case and theoretical data indicate that geographic stomatitis may be an oral manifestation of psoriasis. Moreover, to improve our understanding, psoriatic patients should routinely undergo a detailed oral examination and patients with geographic stomatitis should routinely be submitted to a cutaneous routine examination.

Background:Granulomatous slack skin is a very rare subtype of T-cell cutaneous lymphoma, characterized by the slow development of cutaneous sagging, especially on flexural areas. Its behavior is indolent and the treatment, in the majority of cases, disappointing.Main observation:We report a 54-year-old black patient with granulomatous slack skin, who at the beginning of the investigation showed intense xeroderma and generalized lymph node enlargement. The diagnosis was established based on histopathologic findings long before the disease’s characteristic clinical presentation appeared.Conclusions:During the twelve years of follow-up, the clinical manifestation evolved to marked skin looseness, most predominant in flexural regions, illustrating the clinical hallmark of granulomatous slack skin, long after first histological abnormalities were observed.

Psoriasis affects a considerable proportion of women in their reproductive years. Limited published data exist about the possible negative impact of the disease itself in the prognosis of pregnancy. On this background, the emergence of newer biologic agents for psoriasis treatment - such as ustekinumab - raises safety issues concerning the exposure to the drug during pregnancy. To our knowledge this is the first report in the literature describing a pregnancy outcome under ustekinumab treatment.Observation: We report a 35-year-old female psoriasis patient who was under treatment with ustekinumab for a year when she inadvertedly became pregnant. The drug was discontinued immediately and the patient did not opt for termination. During the 12th week of gestation she experienced a spontaneous abortion.Conclusion:Although the patient’s profile fulfilled 2 general risk factors for spontaneous abortion - she was a smoker and this was her third pregnancy - one could not underestimate the possible role of the drug and of psoriasis per se in this adverse pregnancy outcome. Pregnancy registries and large prospective studies are needed in order to determine whether poorer pregnancies outcomes in psoriatic women are due to the disease itself, associated comorbidities or side-effects of new therapies such as ustekinumab.

Background:The clinical and dermoscopic diagnosis of cutaneous melanoma metastases may be challenging especially in patients with unknown primary melanoma.Main observations:We observed repetitive dermoscopic patterns of peripheral stellate telangiectasias in cutaneous melanoma metastases from 3 patients, of whom 2 had an unknown primary melanoma.Conclusions:Stellate telangiectasias surrounding bluish to purple or red nodules with recent onset may represent a clue for cutaneous melanoma metastases.