Distinctive lupus panniculitis of scalp with linear alopecia along Blaschko's lines: a review of the literature

Lupus panniculitis of the scalp (LPS) is a rare and distinctive clinical feature of lupus erythematosus panniculitis (LEP) with linear alopecia along Blaschko's lines. In this study, we investigated clinical features and treatments of LPS by literature review of articles in the English language from PubMed and SCOPUS databases up to April 2018. The following key words, “lupus panniculitis, “lupus erythematosus panniculitis”, “lupus profundus”, “head”, and “scalp”, were used. Twenty cases of LPS were identified (mean age = 26.4 [10–53] years, female: male ratio = 1:1, mean disease duration = 86.89 [8–336] weeks). The most commonly affected areas of scalp included parietal (70%), frontal (45%), temporal (40%), occipital (30%), and vertex (10%), along 70% of Blaschko's lines with morphologic lesions linear, annular, arch-shaped, and ulcer. Besides, ANA (60%) was in particular noted. Hydroxychloroquine, oral prednisolone, intralesional corticosteroid, and methotrexate were the most common treatments, with complete response after an average period of 8.08 (2–12) weeks. Systemic lupus erythematosus (SLE) was developed in four cases (20%) during follow-up, with high recurrence of 35%. We reported distinctive and rare cases of LPS.The continuing follow-up was highly recommended to avoid probable recurrence and SLE development.     

Cervical cancer metastasis to the scalp presenting as alopecia neoplastica

A 45-year-old Japanese woman was diagnosed in 1996 with squamous cell cancer of the cervix following an abnormal Papanicolaou smear and subsequent diagnostic conization. She underwent total abdominal hysterectomy with pelvic lymphadenectomy and was found to have poorly differentiated squamous cell carcinoma, International Federation of Gynecology and Obstetrics (FIGO) stage IB1. She remained asymptomatic until 2003 when she presented with obstructive uropathy with acute renal failure and hydronephrosis, suspected to be from the recurrence of cervical cancer. This was confirmed when computerized tomography (CT)-guided lymph node biopsy showed squamous cell carcinoma of the para-aortic lymph nodes histologically consistent with the cervical primary. In addition, there was evidence of lumbar spine metastasis by positron emission tomography (PET) and bone scans. She received several courses of chemotherapy with cisplatin and 5-fluorouracil (5FU), as well as radiation therapy. In July 2004, she was hospitalized for acute renal failure, nausea, vomiting, and anorexia. CT of the abdomen identified widespread metastases in the liver, pancreatic head, and lumbar spine. During this hospitalization, she complained of severe scalp tenderness and patchy hair loss first noticed 3 days prior to presentation. On examination of her scalp, two patches of alopecia were observed (Fig. 1). In the largest patch, there was a 5 x 2.5-cm, tender, erythematous plaque with atrophy. In the smaller patch, there was a 2 x 1.5-cm, erythematous, scaly plaque. A punch biopsy of the larger patch revealed atypical epithelial cells in nests with intravascular involvement and diminished numbers of focally miniaturized hair follicles (Fig. 2a). The scalp specimen was histologically identical with biopsy specimens of the cervical primary (Fig. 2b). There was also seborrheic dermatitis, with thick greasy scale, noted on the scalp, which resolved with fluocinolone solution. The patient decided against further treatment for her advanced cervical cancer but did accept hydromorphone for pain. She died 3 months after admission.     

Cutaneous angiosarcoma of the face and scalp presenting as alopecia

An 83-year-old woman presented with a 6-month history of hair loss and painless bruising involving her forehead and scalp. She was otherwise well. Skin biopsy of her scalp confirmed angiosarcoma with a significant increase in miniaturized and telogen hair follicles and some tumour-associated scarring hair loss. She was commenced on the chemotherapeutic agent paclitaxel and then subsequently the semi-synthetic taxane docetaxol. Treatment was terminated because of lack of response and adverse effects. Alopecia is an uncommon presentation in angiosarcoma and in this case there was a mixed pattern of focal scarring and follicular miniaturization. The latter was present only in areas of tumour involvement and not in a typical pattern distribution for androgenetic alopecia. The direct role of tumour in follicular miniaturization and alopecia is speculated and the implications of this for novel future treatment strategies is discussed.     

Linear lupus erythematosus profundus on the scalp following the lines of Blaschko

We describe a 10-year-old Japanese girl presenting linear alopecia on the scalp and forehead. Histological examination showed fat degeneration with mucin deposit and periappendageal infiltrate of mononuclear cells. We diagnosed her as having linear lupus erythematodes profundus with a linear configuration following the lines of Blaschko.     

Centrifugal lipodystrophy of the scalp presenting with an arch-form alopecia: A 10-year follow-up observation

We describe a 10-year follow-up observation of progressive arch-form alopecia caused by centrifugal lipodystrophy (CLD) in a Japanese boy. A 2.5-year-old boy developed a slightly depressed lesion demarcated by a horseshoe-shaped erythematous border on his right neck, which then extended to the scalp. Four years later, arch-form alopecia became apparent in the right temporal region along with an erythematous border. The arch-form alopecia gradually expanded centrifugally, leaving a slight residual depression, but hair regrowth was seen within the area of alopecia. Histological examination of the erythematous border revealed non-specific inflammatory changes in the subcutaneous fat. Magnetic resonance imaging findings revealed a loss of subcutaneous fat inside the lesion. The alopecia continuously extended until he was 12 years old, but, thereafter, expansion ceased and hair regrowth gradually occurred in the arch-form alopecia. A linear non-hairy lesion 5 cm in length still remained when he was 13 years old. CLD might involve the scalp and cause linear, arch-form alopecia.     

Reflectance confocal microscopy-assisted diagnosis of lichen planus pigmentosus distributed along multiple unilateral Blaschko's lines in a child: A case report

A boy with a 2-year history of asymptomatic, linear pigmented macules involving the right side of the trunk and right upper limb. RCM revealed the dermal papillary rings were destroyed, and numerous irregular particulate structures with high refractive values were distributed in the superficial dermis. The RCM features implied the possibility of interface dermatitis. RCM was a complementary diagnostic tool for linear pigmented macules.     

Linear arrangement of neutrophils along the Basal layer in bullous pemphigoid: a unique histological finding

Bullous pemphigoid is an inflammatory autoimmune subepidermal bullous disease with distinct immunohistological features. We report an unusual case of a 59-year-old woman with a bullous eruption whose lesional skin biopsy showed a subepidermal blister with a linear arrangement of neutrophils, mimicking linear IgA bullous dermatosis. However, direct immunofluorescence studies demonstrated IgG and C3 linear deposition along the basement membrane zone, compatible with bullous pemphigoid. We suggest that bullous pemphigoid should therefore be considered in the differential diagnosis of neutrophil-rich subepidermal bullous diseases along with dermatitis herpetiformis and linear IgA.     

Moniletrix of the scalp from almost normal aspect to total alopecia: variable intrafamilial expressiveness

Monilethrix is a rare defect of the hair shaft, with most cases showing an autosomal dominant pattern of inheritance and variable clinical expression. It is characterized by hypotrichosis secondary to hair fragility. The diagnosis is made through trichoscopy, detecting typical findings such as periodic narrowing at regular intervals, giving the hair the appearance of beads in a rosary. This article reports the case of six members of a family diagnosed with monilethrix with alopecia of varying degrees.     

Lichen planopilaris presenting as truncal alopecia: a case presentation and review of the literature

Lichen planopilaris is an inflammatory condition of unknown etiology, characterized by violaceous folliculocentric papules and hair loss. Some clinicians consider lichen planopilaris to be a variant of lichen planus, but others believe it to be a separate disease entity. Many treatment modalities have been utilized, with varying degrees of success. We describe the case of a 63-year-old man who presented with widespread alopecia of the trunk and extremities and was subsequently diagnosed with lichen planopilaris.     

A clinicopathologic study of scarring alopecia due to lichen planus: comparison with scarring alopecia in discoid lupus erythematosus and pseudopelade.

The clinicohistologic findings in 68 patients with lichen planus scarring alopecia (LP) were compared with those of 25 patients with discoid lupus erythematosus of the scalp (DLE) and 25 with pseudopelade (PP). The combination of diffuse scaling, erythema, telangiectases, and mottled hyperpigmentation within areas of scarring alopecia was a distinctive feature of DLE, whereas the clinical picture of PP was indistinguishable from that seen in 29 patients with LP. In most patients with LP, the histologic changes involved only the follicles and the perifollicular dermis. Less frequently, the inflammatory process extended to the epidermis and the papillary dermis. In all cases, histopathologic features allowed LP to be distinguished from DLE regardless of the stage of the disease. The finding of a bandlike fibrotic thickening of the papillary dermis accompanied by fibrotic tracts at sites of destroyed follicles appeared to be a hallmark of "burnt out" lesions of LP. This histologic clue may be helpful in achieving a specific diagnosis of LP in cases that fulfill the clinical criteria for PP.     

Follicular microhemorrhage: A unique dermoscopic sign for the detection of coexisting trichotillomania in alopecia areata

The diagnosis of trichotillomania (TT) is often difficult as it presents similar clinical manifestations with other hair loss diseases, especially alopecia areata (AA). As TT often coexists with AA, the methodology enabling reliable detection of TT in AA needs to be developed. Recently, characteristic dermoscopic findings of TT have been reported, yet, they were most clearly detectable by conventional immersion dermoscopy, not by dry dermoscopy, a technique more easily adoptable in daily practice. In addition, the usefulness of those signs for differentiating TT from AA has not been sufficiently assessed. Through intensive scanning of hair loss lesions by dry dermoscopy in AA patients with TT, we found a sign potentially useful for detecting hidden TT. The sign we named “follicular microhemorrhage” (FMH) represents a red dot corresponding to a follicular ostia capped or stuffed with blood clot and suggests a history of traumatic forced plucking. So far, we have detected FMH in four TT patients with moderate to severe AA. Although further accumulation of cases is necessary, FMH would be beneficial to dissect complicated pathophysiology of hair loss in AA patients with TT.     

The PUVA-turban as a new option of applying a dilute psoralen solution selectively to the scalp of patients with alopecia areata

BACKGROUND: Alopecia areata is a burden for many patients and often resistant, even to extensive therapy. Orally administered PUVA therapy has been shown among numerous systemic and topical treatment modalities to be a therapeutic alternative. However, the clinical use of oral PUVA is often limited by systemic side effects. Bath-PUVA therapy offers an alternative solution because of the negligible systemic absorption of psoralen with this technique. Through use of a "PUVA-turban" it is now possible to administer a dilute bathwater solution containing 8-methoxypsoralen (8-MOP) to the scalp. OBJECTIVE: The purpose of this study was to determine whether PUVA turban therapy is effective in treating alopecia areata in different clinical stages. METHODS: We treated 9 patients with severe, rapidly progressing, treatment-resistant alopecia areata with PUVA-turban treatment as a modification of bath-PUVA therapy. At each treatment session a cotton towel was soaked with a 0.0001% 8-MOP solution (1 mg/L) at 37 degrees C, wrung gently to remove excess water, and wrapped around the patient's head in a turban fashion for 20 minutes. This was directly followed by UVA radiation. Treatment sessions were initially performed 3 to 4 times per week. RESULTS: The cumulative UVA doses given over treatment periods of up to 24 weeks were 60.9 to 178.2 J/cm(2), with single doses ranging from 0.3 to 8.0 J/cm(2). After up to 10 weeks of treatment, hair regrowth could be noticed in 6 of 9 patients. Two patients did not respond to the treatment, and one patient showed only vellus hair regrowth. CONCLUSION: PUVA-turban therapy can be considered a useful method of administering a dilute psoralen solution selectively to the scalp of patients. It has been shown to be a well-tolerated and, in some patients, efficient therapeutic alternative in the treatment of alopecia areata.     

Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature

We present a remarkable case of subcutaneous panniculitic T-cell lymphoma (SPTL) that eluded diagnosis for 14 years and illustrates the importance of continued follow-up with repeat biopsy when SPTL is suspected. This case is unusual in that multiple biopsies demonstrated either a nonspecific panniculitis or lipomembranous panniculitis with calcified lipomembranes. A clinicopathologic review of 72 cases of SPTL from the English language literature is also presented, and approaches to diagnosis and treatment are reviewed.