Hypereosinophilic syndrome (HES) is a rare haematologic disorder characterised by unexplained, persistent eosinophilia with organ involvement. We report the case of a 74-year-old patient who presented with a pemphigoid-like bullous rash as the only manifestation of an HES. This report highlights the importance of the dermatological manifestations of this potentially life-threating condition that may initially present as a skin-limited disease. Bullous pemphigoid (BP) is a very common autoimmune blistering dermatosis that may share common clinical and histological features or associate with an HES. HES should be considered when patients diagnosed with BP demonstrate atypical course under corticosteroids and/or recalcitrant blood eosinophilia.

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Follicular psoriasis is an uncommon diagnosis and probably the least well-known subtype of psoriasis. Hence, we report the clinical and histological findings of follicular psoriasis in one patient to raise awareness of this rare entity.

Sir, Methotrexate is an antimetabolite commonly used in dermatology for inflammatory and autoimmune diseases, including psoriasis. Malabsorption secondary to treatment with methotrexate after its use in high doses and/or long-term has been described in the literature. However, only several cases of a sprue-like disease secondary to a low dose of methotrexate have been described. Herein, we report an exceptional case of a sprue-like disease after a single dose of methotrexate. A 43-year-old female was admitted to our department with psoriatic erythroderma (Figs. 1a and 1b). Treatment with MTX was administered at a dose of 12.5 mg/week as well as folic acid supplements. Three days later, the patient developed bilateral leg edema, associated with biological malabsorption syndrome (hypoalbuminemia at 19 g/L, hypocholesterolemia at 0.98 g/L, prothrombin time at 45%, blood glucose at the lower limit of 0.7 g/L, and normocytic normochromic anemia at 9.4 g/dL). A duodenal biopsy performed three weeks later revealed no villous atrophy (Fig. 2). The discontinuation of methotrexate led to the disappearance of the leg edema and the progressive correction of biological parameters.

<p class="abstract">Epithelioid hemangioendothelioma (EHE) is a rare low-grade vascular tumour mainly affecting bones, deep soft tissues and exceptionally the skin. Multifocal cutaneous involvement is uncommon and should raise suspicion for metastasizing extracutaneous epithelioid hemangiodothelioma. Hereby we report the case of a young patient with multifocal cutaneous EHE involving the same lower limb. We emphasize the necessity for dermatologists to evoke the diagnosis of EHE in patients presenting with nonspecific tumours of the extremities. Prompt management and diagnosis are essential in this potential aggressive tumour.</p>

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Sir, Dermatomyositis (DM) is a systemic autoimmune disease affecting characteristically the skin and muscles. The typical cutaneous features include Gottron’s papules, a heliotrope rash, and edema of the eyelids. Spinulosis is an unusual sign, not raising suspicion for a DM diagnosis. Herein, we report a case of DM relapsing as Wong’s dermatomyositis (WDM) as an isolated spinulosic eruption. A 42-year-old female presented with a pruritic papular eruption involving both arms and the back persistent for the last two months. An anamnesis included a history of DM diagnosed five years earlier, in complete remission under the administration of corticosteroids for three years. A physical examination revealed erythematous and violaceous scaly plaques resulting from confluent follicular papules, resembling pityriasis rubra pilaris (PRP) on the lateral sides of the arms (Fig. 1) and the upper back (Fig. 2). Muscular testing was subnormal and the general status was conserved. Laboratory findings revealed a high level of blood CPK (3×N), LDH (4×N), and ASAT (3.5×N), while electromyography displayed myogenic changes. A muscular biopsy revealed necrotic muscular cells with perifascicular atrophy and lymphocytic inflammatory infiltrate. A histological skin examination found follicular hyperkeratosis with perifollicular interface dermatitis and interstitial mucin deposits in the dermis (Fig. 3). Based on these findings, the diagnosis of WDM was retained. A malignancy workup was negative. Corticosteroid therapy was initiated with prednisone 1 mg/kg/day in combination with hydroxychloroquine 200 mg twice a day, resulting in a remarkable clinical and biological improvement.WDM is characterized by the presence of PRP-like follicular papules in a patient with dermatomyositis (DM) [1]. Some authors suggest that this exceptional presentation should be considered a true association of DM with PRP rather than a variant of DM [2]. In this rare form, spinulosic lesions are mainly located on the trunk or limbs with occasional palmoplantar keratoderma [2]. Follicular lesions are usually associated with the typical signs of DM but some cases of isolated PRP features related to WDM have been reported [3]. To our knowledge, none has been reported as a relapse of a previous classic DM. Our patient relapsed showing spinulosic lesions absent in the prior episode five years earlier, not displaying at this time any classic cutaneous signs of DM. Such puzzling presentations are very unusual and may be confusing for physicians. In our patient, the diagnosis was retained on the basis of the histopathological findings of interface dermatitis and interstitial mucin deposits in the dermis, increased serum levels of muscle enzymes with a myogenic electromyographic pattern, the typical muscular histological changes, and a medical history of DM diagnosed five years earlier. In WDM, histology usually reveals follicular hyperkeratosis with keratotic plugs filling dilated follicular infundibula, which may raise several differential diagnoses, including PRP, discoid lupus erythematosus (DLE), and, to a lesser degree, follicular psoriasis and keratosis pilaris [4]. Interface dermatitis and mucin deposits, as in our patient, are the hallmarks of distinguishing DM from PRP, follicular psoriasis, and keratosis pilaris. Although these findings may also be observed in DLE [4], in our patient, there were no clinical or histological arguments supporting DLE rather than a diagnosis of DM. Myositis as, well as interstitial lung disease, appears to be comparable to the classic variant [5]. The risk of associated internal malignancy is unknown but could be lower than in the classic form [4]. Treatment and management do not differ from classic dermatomyositis.

Discoid lupus erythematosus (DLE) most commonly affects the face and scalp. Palpebral involvement is rare and not evocative when presenting as the prime manifestation of the disease. We report hereby the case of a young male patient with isolated palpebral and labial DLE. A 34-year-old patient with no medical history was referred from ophthalmology for an erythematous plaque of the eyelid resisting usual treatment of blepharitis. Skin examination revealed a congestive erythema on the right lower eyelid with eyelash fall (Fig. 1). There was also an atrophic cheilitis of the lower lip with slight erosions (Fig. 2). Scalp, oral mucosa and the rest of the integument were not affected. Skin biopsy of the eyelid revealed marked orthokeratosis with slight basal vacuolization and perivascular lymphoplasma cells infiltrate. Direct immunofluorescence displayed a positive lupus band (Fig. 3). Work-up for systemic involvement was negative. Ophthalmologic assessment found no intraocular involvement. Hydroxychloroquine 200mg twice a day with clothing and chemical photoprotection were implemented allowing significant improvement after 3 months (Fig. 4). Palpebral involvement of DLE is uncommon compared to the other suggestive locations including scalp, nose, cheekbones, ears, neckline and hands. An isolated involvement does not suggest DLE at first sight and often leads to delayed diagnosis while scarring and lid deformities might be expected if left untreated [1]. Most commonly it presents as erythematous telangiectasic scaly plaques on the external third of lower eyelid. Blepharitis-like, madarosis, periorbital edema or cellulitis presentations have also been reported [2]. Differential diagnosis may arise with several chronic palpebral dermatoses, as carcinomas, eczema, blepharoconjunctivitis, or seborheic dermatosis. Assessment for intraocular involvement such as keratitis should always be performed [3]. Early recognition and treatment are essential to avoid eyelid complications as ectropion, entropion, and trichiasis. Photoprotection and antimalarials are the mainstay treatment showing remarkable efficacy.