A. Utani

Intralymphatic histiocytosis associated with rheumatoid arthritis

vaccination sites. On clinical examination, there was a red, crusted plaque, 30 · 15 mm in size, on the deltoid region of the right arm and a similar lesion on the left upper arm with an atrophic centre. Histological examination of both of these lesions showed superficial BCC, with the lesion on the left arm showing an early nodular component. Both lesions were subsequently fully excised. No further BCCs have developed elsewhere. Non-melanoma skin cancer is well known to develop within chronic inflammation or scars. There have been several reports of BCCs developing in smallpox vaccination sites and an isolated case within a chronic leishmanial scar. To date, there have been three reports of BCCs within BCG vaccination scars. To our knowledge, no cases have been reported in association with travel vaccine, specifically hepatitis A and typhoid. The aetiology of malignant transformation of scars is yet to be elucidated. Chronic irritation is proposed to be a predisposing factor, as is trauma. Tight, thickened or ulcerated scars and those with slow healing initially are considered to be the most at risk. There is no evidence to suggest that vaccination causes localized immunosuppression. With foreign travel being commonplace, particularly to tropical countries, which require more immunizations and allow greater sun exposure, we feel it is important to highlight this rare presentation of BCC.

Malignant melanoma derived from cerebriform intradermal naevus.

We report a case of malignant melanoma (MM) derived from cerebriform intradermal naevus (CIN) in a 66‐year‐old Japanese man. The patient had cutis verticis gyrata (CVG) on the posterior area of the scalp at birth. He noticed a dome‐shaped nodule at the centre of the CVG at 66 years of age. Histopathological examination found a nodule of MM arising within an extensive area of intradermal naevus. There was no metastasis to lymph nodes or other organs. To our knowledge, only two cases of CIN in which MM had later developed have been reported. We estimated that the incidence of melanoma from CIN including our case is 4.5% (3 of 67 reported cases), which seems to be comparable to the frequency of malignant alteration of giant pigmented naevi. This suggests that pathological examination is recommended for CVG, and once pathological diagnosis of CIN is confirmed, long clinical follow‐ups are necessary for detecting development of MM.

Occupation-related pigmented macules on the sole with parallel-ridge pattern on dermatoscopy.

A 27‐year‐old man presented with pigmented macules on the right sole, which showed a parallel‐ridge pattern on dermatoscopy. His work for a chemical company involved handling para‐phenylenediamine. Histological examination of a biopsy from a lesion did not find any proliferation of atypical melanocytes. Shaving the cornified layer of the lesions with a surgical knife resulted in the disappearance of macules. We speculate that para‐phenylenediamine on the sole of the patient’s work boot might have become blotted to the cornified layer of the cutis. This report adds a new occupation‐related differential diagnosis for skin diseases showing a parallel‐ridge pattern on dermatoscopy.

Familial case of piebaldism with regression of white forelock

1 Scott CM, Flint SR. Oral syphilis – re-emergence of an old disease with oral manifestations. Int J Oral Maxillofac Surg 2005; 34: 58–63. 2 Dalmau J, Alegre M, Sambeat MA et al. Syphilitic nodules on the tongue. J Am Acad Dermatol 2006; 54: S59–60. 3 Duarte EC, da Silva LM, Naves MD et al. Primary syphilis of oral mucosa: case report of an unusual manifestation. Quintessence Int 2004; 35: 728–30. 4 Kegg S, Pittrof R, Lau R. Homosexual men, HIV, and sexual risk in 2001. Sex Transm Infect 2001; 77: 325–6. 5 Hoang MP, High WA, Molberg KH. Secondary syphilis: a histologic and immunohistochemical evaluation. J Cutan Pathol 2004; 31: 595–9.

Pemphigoid without mucosal involvement showing autoantibodies against laminin-332 γ2 subunit

receiving monoclonal antibodies such as natalizumab, rituximab or more recently efalizumab in psoriasis. In our case, it is noticeable that the patient did not receive any immunosuppressive therapy prior to the onset of neurological disease, as he received only interferon alfa, a drug which has been reported to show partial efficacy in the treatment of PML. Currently, the treatment strategy in PML is based mainly on immune reconstitution, i.e. highly active antiretroviral therapy in AIDS and immunosuppression reduction whenever possible in cases related to malignancies and immunosuppressive treatments; no currently available treatment has clearly shown efficacy in JCV infection to date. Some recent in vitro results suggest that mefloquine could be an effective therapy. To our knowledge, this is the first reported case of PML in a patient with Sézary syndrome, which raises the need to detect JCV invasion of the central nervous system in patients with CTCL showing unexplained neurological symptoms. It also emphasizes the role of CD4 T-cell responses in the control of intracerebral JCV invasion of the central nervous system, and in the prevention of PML.

Two cases of dyshidrosiform pemphigoid with different presentations.

We report two cases of dyshidrosiform pemphigoid (DP) with different presentations. One patient was a 65‐year‐old Japanese man, who had been diagnosed with dyshidrosis and had been treated before visiting our hospital. When we stopped all treatments, the vesicles increased and spread to the trunk and limbs. We made a diagnosis of vesicular pemphigoid (VP) that was concomitant with or transformed from DP. Using Western blotting, the sera reacted with antigens with molecular weights of 60 and 180 kDa. The 60‐kDa antigen has not been found previously in the sera of patients with VP. The other patient was a 94‐year‐old Japanese woman, who presented with redness and swelling with bullae on the palmoplantar areas. Five days later, areas of oedematous erythema, as seen in prototypical bullous pemphigoid (BP), developed on the limbs. Study of the distribution of the BP antigen may elucidate the mechanisms involved in localized forms of BP such as DP.

Multiple dermatomal daughter lesions of postzoster granuloma

1 Onodera S, Ukai K, Suzuki Y et al. Changes in the prevalence of HCV infection during 10 years in an HCV epidemic area of Japan. Nippon Rinsho 2004; 62 (Suppl. 7):264–6. 2 Quesada JR, Gutterman JU. Psoriasis and alpha-interferon. Lancet 1986; i:1466–8. 3 Conlon KC, Urba WJ, Smith JW 2nd et al. Exacerbation of symptoms of autoimmune disease in patients receiving alpha-interferon therapy. Cancer 1990; 65:2237–42. 4 Georgetson MJ, Yarze JC, Lalos AT et al. Exacerbation of psoriasis due to interferon-alpha treatment of chronic active hepatitis. Am J Gastroenterol 1993; 88:1756–8. 5 Watashi K, Hijikata M, Hosaka M et al. Cyclosporin A suppresses replication of hepatitis C virus genome in cultured hepatocytes. Hepatology 2003; 38:1282–8. 6 Watashi K, Ishii N, Hijikata M et al. Cyclophilin B is a functional regulator of hepatitis C virus RNA polymerase. Mol Cell 2005; 19:111–22. 7 Inoue K, Sekiyama K, Yamada M et al. Combined interferon alpha2b and cyclosporin A in the treatment of chronic hepatitis C: controlled trial. J Gastroenterol 2003; 38:567–72. 8 Hayashi J, Nakashima K, Yoshimura E et al. Detection of HCV RNA in subjects with antibody to hepatitis C virus among the general population of Fukuoka, Japan. J Gastroenterol 1994; 29:147–51.

Severe muscle weakness to necessitate rehabilitation in a case of trichorhinophalangeal syndrome type II

© 2008 The Authors JEADV 2009, 23, 702–738 Journal compilation

Symmetrical pigmented sclerosis enclosed by pruritic erythema: a new variant of morphoea?

of this regimen showed no change in metastases. After 5 months of weekly cetuximab, lung lesions were stable but the general status of the patient rapidly declined and he finally died of sepsis. A nodular and infiltrative BCC of the right axillary fold had been initially removed in a 58-year-old white man. During the following years, he experienced a local recurrence treated by surgery and irradiation followed by the further development of deep axillary specific lesions and metastases in the regional lymph nodes, bones and finally in the lungs despite systemic chemotherapy with cisplatin. Because of overexpression of EGFR by the primary tumour, treatment with cetuximab was implemented with stabilization of distant metastases and axillary lesions while additional lesion occurred after eight weekly courses with 250 mg m. Side-effects were limited to mild head and neck folliculitis. Cetuximab was then interrupted on the patient’s request, but liver metastasis developed and the patient died 3 months after interruption of the cetuximab. mBCC occurs in 0Æ0028–0Æ5% of all cases of BCC and a number of clinical and histological risk factors have been defined, some of which were present in our patients. Furthermore, HIV infection might have played a role in our first patient. The prognosis remains poor with a mean survival ranging from 8 months to 3Æ6 years and a median overall survival of approximately 11 months. Cetuximab is a recombinant human and mouse chimeric monoclonal antibody which competitively inhibits EGFR, and is mainly used in recurrent, locally advanced or metastatic head and neck squamous cell carcinoma (SCC). Recently, individual reports of cetuximab use in locally advanced BCC or cutaneous SCC and in metastatic SCC have been published with encouraging results, including a complete response in two patients with extensive, in-transit SCC recurrences and a partial response in a patient with xeroderma pigmentosum with lymph node metastasis of SCC. In our patients, the treatment was well tolerated with a mild acneiform rash in one patient that did not require dose reduction. A stabilization of secondary lesions was obtained in both patients, and, although that effect was limited to the therapeutic period, the benefit ⁄risk ratio of cetuximab seemed favourable especially for our first patient. Accordingly, antiEGFR-based treatments are likely to find a significant place in future therapeutic strategies in advanced, locally uncontrolled or metastatic BCC.

Scleredema with diffuse pigmentation

disease. It significantly reduced the incidence of AIDS-associated events and deaths and even changed treatment regimens of AIDSassociated cancers. With the immune restoration afforded by HAART, patients better responded to cancer treatment. There are data demonstrating that HAART regimens alone lead to remission of KS. In conclusion, our case wants to outline the high risk for development of KS in HIV therapy–naı̈ve patients and the fact that the initial site of KS often is the oral cavity. The dominant role of HHV-8 in the pathogenesis of all epidemiologic forms of KS is undisputed; nevertheless, many other cofactors seem to be involved. In closing, the impressive response of HAART in our patient leads to a nearly complete remission of KS. That emphasizes the fundamental impact of HAART in HIV patients.