Mona Abdel-Halim

ABSTRACT: Crystalglobulinemia (CG) is a rare disorder characterized by crystallization of monoclonal immunoglobulins in the microcirculation leading to multiorgan vascular thrombosis and ischemic injury. The main cause of CG is multiple myeloma. We report a case of a 52-year-old man who presented with widespread necrotizing plaques and ulcerations. A skin biopsy revealed eosinophilic rectangular-shaped crystals occluding the lumina of blood vessels with no associated features of vasculitis. The crystals were Periodic acid-Schiff stain positive. The findings were diagnostic of CG. Extensive work up lead to the discovery of multiple myeloma. Awareness of CG is important because it may be the first presenting manifestation of an underlying serious hematological malignancy.

CLINICAL QUESTION: A 26-year-old woman presented with asymptomatic skin coloured to whitish keratotic papules on the vulva, inguinal folds and inner thighs of 6 years duration. She had no similar lesions elsewhere and no family history. A biopsy was taken from one of the papules.Review the high-quality, interactive digital Aperio slide at http://virtualacp.com/JCPCases/jclinpath-2018-205434/ and consider your diagnosis. WHAT IS YOUR DIAGNOSIS?: Darrier's diseaseHailey-Hailey diseaseGenital wartsPapular acantholytic dyskeratosis of genitocrural areaPemphigus vegetansThe correct answer is after the discussion.

DISCUSSION: Papular acantholytic dyskeratosis (PAD) of the genitocrural area is a peculiar acantholytic dermatosis with dyskeratosis localised to the vulva and inguinal folds with possible extension to the thighs and perineum.1 It mainly involves young or middle-aged females.1 Cases affecting males and involving genitalia, thighs and perianal region have been reported.2 It represents an allelic variant of Hailey-Hailey disease as heterozygous mutations in gene (intron 5 and exon 24) have been reported in some cases.3 Clinically, PAD of the genitocrural area is typically characterised by solitary or grouped keratotic flesh coloured or white papules.1 Other clinical presentations include: vesicles, bullae, patches and plaques.4 Although the lesions are mainly asymptomatic, patients may experience variable degrees of itching or burning sensation.Histopathologically, the lesions show hyperkeratosis, hypergranulosis, acanthosis and acantholytic dyskeratosis in the spinous layer (figure 1). Typically, direct immunoflourescence (DIF) is negative.5 jclinpath;jclinpath-2018-205434v1/F1F1F1Figure 1Photomicrograph representing histopathological findings: hyperkeratosis, hypergranulosis, acanthosis and acanthloytic dyskeratosis involving the spinous layer; 254×143 mm.The most important differential diagnoses of this sporadic condition include: Darier's and Hailey-Hailey, both of which are familial. Hailey-Hailey involves also other flexural areas and presents with eroded crusted lesions with foul odour. Pathologically, the acantholytic process in PAD of the genitocrural area is rather focal compared with the broad zone of acantholysis in Hailey-Hailey, and columns of parakeratosis overlying suprabasilar acantholysis are characteristic of Darier's disease. Although, some cases of PAD of the genitocrural area can present with vesicles and bullae, the presence of dyskeratotic cells and the negative DIF speaks against pemphigus.Treatment options for this condition include topical and systemic retinoids. Cryotherapy and laser have been also reported to be effective. Topical steroids might be effective in reducing pruritus.

A 77-year-old female patient presented with bilateral tender, swollen, erythematous, focally crusted cheeks with a discharge of pus and necrotic material, which had developed one month after autologous fat transfer and a corrective injection procedure conducted to correct an overdone fat transfer. Histopathological examination of the discharged material using routine hematoxylin and eosin staining revealed degenerated collagen admixed with three different filler materials. Scattered all throughout the specimen were numerous thick-walled, basophilic, nonbudding spherical bodies of variable sizes. The bodies stained positively with Periodic acid-Schiff and Alcian blue and showed internal septation and sporangia with a morula-like appearance. The morphology of these bodies was characteristic of a infection. The patient was treated by surgical drainage accompanied by itraconazole 200mg daily for six months, ultimately showing marked improvement.

Mycosis fungoides is the most common type of primary cutaneous T cell lymphomas. Doxycycline promoted apoptosis in different human malignant cell lines and in vivo models. To test for the therapeutic efficacy of doxycycline in comparison to PUVA in early stages of classic MF and its effect on T cell apoptosis. Thirty-six patients were randomized into either: doxycycline 200 mg daily ( = 18) or PUVA (3 weekly sessions) ( = 18) for 12 weeks. The primary outcome (therapeutic efficacy) was defined in terms of objective response rate (ORR) which was measured according to changes in the modified severity weighted assessment tool (mSWAT). Doxycycline achieved significantly less ORR (partial response) in comparison to PUVA (11.1%, 50%, respectively,  = .016). The percent reduction in mSWAT, CAILS, histopathology score and CD3 expression was significantly less in the doxycycline group ( = .001,  = .001,  ˂ .001, and  = .004, respectively). Within the doxycycline group, changes in mSWAT and CAILS showed no correlation with changes in the CD3 or Bcl-2 expression. Gastric upset was significantly more encountered in the doxycycline group ( = .001). Doxycycline is not suitable as a sole agent in the treatment of early stages of classic MF, acting mainly by anti-inflammatory rather apoptotic function. NCT03454945 ( www.clinicaltrials.gov ).

Mycosis fungoides (MF) is the most common form of cutaneous T cell lymphoma (CTCL) with many clinical variants including papular and pityriasis lichenoides chronica (PLC)-like variants. During psoralen and ultraviolet A (PUVA) treatment of MF, PLC-like papular lesions were observed to appear. The exact nature of these lesions is not fully understood. This work aimed to study PLC-like papular lesions arising in MF patients receiving PUVA therapy clinically, histopathologically and immunohistochemically (using monoclonal antibodies against CD4 and CD8) and to compare them with lesions in classic PLC patients. Fifteen MF patients with PLC-like papular lesions arising during PUVA treatment were included and 15 patients with classic PLC served as controls. While the extent of these lesions significantly correlated with their duration (p < 0.05), it showed no significant correlation with the TNMB stage of MF, number of phototherapy sessions or cumulative UVA dose at which they started to appear. The response status of MF to PUVA did not affect their development. Compared to classic PLC, these lesions showed significantly more acute onset (p = 0.003). None of these lesions showed histopathological features essential to diagnose papular/PLC-like MF and no significant difference existed with regard to their histopathological and CD4/CD8 phenotypic features compared to classic PLC. Papular lesions mimicking PLC in MF patients receiving PUVA mostly represent an upgrading reaction with possible good prognostic implication.

The purpose of this study was to evaluate the efficacy of fractional carbon dioxide laser use in the treatment of mature burn scars. This was an uncontrolled, open-label clinical trial. The setting for this study was Dermatology Department at Cairo University in Cairo, Egypt. Twenty patients with mature burn scars were included in the study. Three fractional carbon dioxide laser sessions were given, 4 to 8 weeks apart. Primary outcome was measured using two scar scales, the Vancouver Scar Scale and the Patient and Observer Scar Assessment Scale. Secondary outcomes included evaluation of collagen and elastic fibers using routine hematoxylin and eosin, Masson's trichrome, and orcein stains. Outcomes were measured two months after the last laser session. Both Vancouver Scar Scale and Patient and Observer Scar Assessment Scale showed significant reduction following treatment (<0.001). Scar relief and pliability improved most followed by vascularity. Pigmentation improved the least. Percent improvement in Patient and Observer Scar Assessment Scale patients' overall assessment was 44.44 percent. The pattern and arrangement of collagen and elastic fibers showed significant improvement (<0.001, =0.001, respectively), together with significant improvement in their amounts (=0.020, <0.001, respectively). No significant correlation existed between clinical and histopathological/histochemical scores. Side effects and complications were mild and tolerable. Fractional carbon dioxide laser use is an effective and safe method for treating burn scars with a significant change in the opinion of the patients about their scar appearance.

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