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Pityriasis Lichenoides Chronica in Black Patients

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Pityriasis Lichenoides Chronica in Black Patients PEDIATRIC DERMATOLOGY Series Editor: Camila K. Janniger, MD Pityriasis Lichenoides Chronica in Black Patients Tanda N. Lane, MD; Sareeta S. Parker, MD Pityriasis lichenoides chronica (PLC) is a cuta- uncommon for individuals to present with lesions char- neous disease of unknown etiology that most acteristic of both the acute and chronic forms.6-8 Indi- commonly affects children and young adults. vidual lesions occur in crops and last weeks to months, The highly variable presentation of this condition but the disease typically has an overall chronic clini- often poses a diagnostic challenge. The clini- cal course of several months to years.6-8 In this report cal presentation of PLC in black patients is not we describe a series of 5 black patients (4 children and well described. We report a series of 5 black 1 young adult) with PLC presenting as widespread patients (4 children and 1 young adult) with PLC dramatic and persistent hypopigmentation with who presented with extensive hypopigmentation prominent facial involvement. Additionally, one and prominent facial involvement. One patient patient had concomitant mycosis fungoides (MF). had concomitant mycosis fungoidesCUTIS (MF). The diagnosis of PLC should be included in the dif- Case Reports ferential diagnosis in dark-skinned patients who Patient 1—An 8-year-old girl presented with a present with widespread hypopigmented macules 3-month history of a minimally pruritic, generalized, and patches. The presenceDo of MF inNot one of our hypopigmented Copy rash. Review of the patient’s medical patients underscores the potential relationship record revealed that she had been treated with intra- between MF and PLC. muscular penicillin G benzathine for a scarlatiniform Cutis. 2010;85:125-129. rash 8 weeks prior to the onset of her skin hypopig- mentation. She had been subsequently diagnosed with tinea versicolor, atopic dermatitis, pityriasis ityriasis lichenoides is a T cell–mediated papular alba, and vitiligo. Prior treatments consisted of tri- dermatosis of unknown etiology that lies along a amcinolone acetonide ointment 0.1%, emollients, P continuum of acute and chronic eruption, both selenium sulfide shampoo 2.5%, and oral antihista- with similar but distinguishable clinicopathologic mines, all without benefit. On physical examination features. The chronic form of pityriasis lichenoides, the patient had extensive ill-defined hypopigmented pityriasis lichenoides chronica (PLC), most com- macules and patches with no infiltration, erythema, monly occurs during the early decades of life and or scale, and sparse red-brown minimally scaly papules classically presents with red-brown dusky papules (Figure 1). Facial involvement was prominent (Figure 2). with adherent micaceous scale often giving a frosted Histology of a thin papule from the shoulder revealed glass appearance.1-5 In contrast, the acute form of parakeratosis with focal interface alteration and lym- the disease, pityriasis lichenoides et varioliformis phocytic exocytosis, slight spongiosis, and sparse peri- acuta (PLEVA), has a tendency for a more papulo- vascular lymphocytic infiltrate consistent with PLC necrotic appearance with hemorrhagic crusts and (Figure 3). This patient was seen sporadically over the occasionally vesicopustules.4 However, it is not following 2 years, and despite treatment with topi- cal corticosteroids and oral erythromycin (30 mg/kg From the Department of Dermatology, Emory University School of daily), persistent pigmentary alteration was notable. Medicine, Atlanta, Georgia. She was subsequently lost to follow-up. The authors report no conflict of interest. Correspondence: Sareeta S. Parker, MD, Department of Dermatology, Patient 2—A 2-year-old girl presented with a sev- Emory University School of Medicine, 1365 Clifton Rd NE, Bldg A, eral month history of hypopigmented macular lesions 1st Floor, Atlanta, GA 30322 ([email protected]). primarily involving the face and extremities with VOLUME 85, MARCH 2010 125 Copyright Cutis 2010. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Pediatric Dermatology Figure 3. Parakeratosis with focal interface alteration, slight spongiosis with lymphocytic exocytosis, and sparse perivascular lymphocytic infiltrate (H&E, original magnification 320). spongiosis, focal vacuolar interface alteration, superficial perivascular lymphocytic infiltrate, and few scattered extravasated red blood cells in the papillary dermis consistent with pityriasis lichenoides. Her therapies included oral erythromycin (40 mg/kg daily), triam- Figure 1. Generalized ill-defined hypopigmented mac- cinolone acetonide ointment 0.1%, and tacrolimus CUTISointment 0.1% over 5 months. At follow-up 5 months ules and patches, and sparse red-brown minimally scaly papules. later, the hypopigmented macules were still present. She subsequently failed to return for reevaluation. Patient 3—A 27-year-old woman presented with Do Nota 4-yearCopy history of progressively worsening hypopig- mentation on her face and extremities. She denied associated symptoms, but upon further inquiry she reported that many of the hypopigmented mac- ules were preceded by small itchy scabs. She had no history of atopic dermatitis, and her medical history was unremarkable. Prior therapies, includ- ing griseofulvin, selenium sulfide shampoo 2.5%, lactic acid lotion 12%, and triamcinolone aceton- ide ointment 0.1%, were ineffective. Examination Figure 2. Scattered hypopigmented macules and thin revealed numerous ill-marginated, 2- to 4-mm, scaling papules with prominent facial involvement. hypopigmented macules distributed on the face (Figure 4), arms, and thighs. Amidst the hypopig- mented macules were rare 2-mm red papules, several relative sparing of the trunk. Upon further question- of them with an overlying adherent scale. Potassium ing, family members reported an antecedent pruritic hydroxide preparations were negative. Two skin biop- papulovesicular eruption of the involved areas as sies, each from papular lesions from her extremities, well as concomitant cough, rhinorrhea, diminished revealed parakeratosis, mild spongiosis, focal vacuolar appetite, and fever. On examination the patient had interface alteration, and a superficial and mid-dermal numerous hypopigmented ill-defined macules and perivascular lymphocytic infiltrate consistent with sparse scaling hyperpigmented thin papules. Tentative PLC. No therapy was instituted due to the patient’s diagnoses were Gianotti-Crosti syndrome versus PLC, newly noted pregnancy status; however, UV therapy and the patient was prescribed oral erythromycin was planned after delivery. (40 mg/kg daily) and triamcinolone acetonide oint- Patient 4—An 11-year-old boy presented with a ment 0.1%. Histology of a hyperpigmented scaly pap- several year history of asymptomatic, erythematous, ule from the anterior thigh revealed parakeratosis, scaly patches with hyperpigmented margins on the ® 126 CUTIS Copyright Cutis 2010. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Pediatric Dermatology Skin biopsies at that time and again at 4 years of age both revealed a superficial and mid-dermal perivascular lymphocytic infiltrate with mild spongiosis, focal vacu- olar interface alteration, and overlying parakeratosis. Rare extravasated red blood cells were present. A 6-month course of treatment with oral erythromycin was ineffective. Fluticasone propionate ointment 0.005% reportedly had minimal effect. A 6-month course of UVB therapy resulted in improvement but was discon- tinued when the patient moved. Currently he reports continued benefit, particularly with regard to repig- mentation of the facial lesions, with frequent regular Figure 4. Hypopigmented macules on the face with focal thin scaling papules. intervals of natural sunlight exposure. Comment trunk. Lesional skin biopsies from an erythematous Pityriasis lichenoides is a benign T cell–mediated patch on the buttock revealed an epidermotropic dermatosis of unclear etiology that lies along a lymphocytic infiltrate with marked lymphocyte atypia disease continuum of acute (PLEVA) and chronic consistent with MF. T-cell receptor g gene rearrange- (PLC) eruptions, both with similar but distinguish- ment analysis on paraffin-embedded tissue was nega- able clinicopathologic features. Histologically, tive. On a subsequent visit months later he was noted PLEVA and PLC exhibit varying degrees of dermal to have ill-defined macular areas of hypopigmenta- inflammation, perivascular lymphocytic infiltrates, tion on his cheeks and proximal extremities with parakeratosis, focal vacuolar interface alteration, sparse, widely scattered, thin, red-brown papules with epidermal necrosis, and extravasated red blood cells.9 micaceous scale that were not present at the initial Both eruptions contain lesional T cell infiltrates, visit. The patient’s parents reported thatCUTIS the erythem- with PLEVA being primarily CD81 dominant and atous patches on the trunk were longstanding and PLC being principally a CD41 infiltrate.3 Because persistent but that the hypopigmented macules devel- T cell monoclonality has been demonstrated in oped subsequent to papular lesions that occurred in the infiltrate in some cases of pityriasis lichenoides crops. Based on his historyDo and clinical Not presentation, and Copyrare cases of association with lymphoprolif- dual diagnoses of MF as well as PLC were made. On erative disorders have
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